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ABSTRACT
Year : 2018  |  Volume : 14  |  Issue : 11  |  Page : 912-927

Case reports


Date of Web Publication29-Nov-2018

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How to cite this article:
. Case reports. J Can Res Ther 2018;14, Suppl S4:912-27

How to cite this URL:
. Case reports. J Can Res Ther [serial online] 2018 [cited 2019 Sep 18];14:912-27. Available from: http://www.cancerjournal.net/text.asp?2018/14/11/912/246374




 > Case Report: 01 Top


Metastatic spindle cell tumor pancreas

Parul Gupta, Anil Kumar Dhull, Vivek Kaushal

Department of Radiation Oncology, Regional Cancer Centre, Pt. B D Sharma PGIMS, Rohtak, Haryana, India

Aims and Objective: Sarcomatoid (spindle cell) tumor of the pancreas is a rare, high-grade epithelial malignancy composed predominantly or exclusively of spindle cells demonstrating evidence of epithelial derivation, but no features indicative of a specific line of mesenchymal differentiation. Sarcomatoid (spindle cell) tumor rarely presents in the pancreas, with, to the best of our knowledge, only infinitesimal cases reported in the literature. Materials and Methods: The current study presents the case of a 64-year old female with no known comorbidities reported with chief complaints of pain in epigastric region for 3-months, which was insidious in onset, gradually progressive, aggravated by food intake and relieved by oral analgesics. Pain was localized in epigastrium, radiated to the upper back and was associated with nausea. CECT abdomen revealed a solid lesion of 4.3×3.1×3.2 cm on retroperitoneal surface of body of pancreas abutting left renal vein with few lymph nodes in gastrohepatic recess. A 3×2.5cm lesion was present in lower pole of spleen and 6.2×6.9×5.7 cm mass lesion in lower lobe of left lung in posterior basal segment. Ultrasonography abdomen revealed a 3.4×3.2 cm mass lesion abutting posteriorly to body of pancreas. Results: Ultrasound guided endoscopic biopsy of pancreatic body mass revealed spindle cell tumor. The neoplastic cells illustrated wide spread positivity for vimentin and B cell lymphoma-2 (Bcl-2) with focal positivity for CD34 stain. Mitosis was found to be >10 per high power field and Ki 67 proliferation index was 17%. Whole body PETCT revealed a FDG avid 3.6×3.2 cm mass in body of pancreas with multiple abdominal, bilateral axillary, mediastinal lymph nodes and left lung lesion in posterior basal segment of lower lobe of the left lung, causing encasement of segmental bronchi with 2.7×2.1 cm size lesion in spleen along with multiple FDG avid nodules in enlarged thyroid gland. The histopathological appearance and immunohistochemical profile of the biopsy tissue and related scans confirmed it to be spindle cell sarcoma of the pancreatic body, intermediate grade with metastasis to abdominal, axillary and mediastinal lymph nodes, spleen, thyroid gland and lung. Patient is planned for chemotherapeutic trial of combination chemotherapy with doxorubicin 70mg and dacarbazine 500mg, 3-weekly for 6-cycles and further response assessment. Conclusions: In conclusion, the current case study documents that pancreatic sarcomas with data substantiating the epithelial derivation of the nondescript malignant spindle cell element are extremely rare entities. Our patient presented with metastatic disease with survival of less than 12-months as per literature review. Our patient is undergoing treatment and is having good palliation after 2-cycles of doxorubicin and dacarbazine chemotherapy.


 > Case Report: 02 Top


Metastatic implantation of esophageal cancer at peg tube site: Case report and review of literature

Jenny Joseph, Judith Aaron, Johny K. Joseph, Jose Tom, T. G. Bindu, Jino Thomas, Teena Nelson

Objectives: Percutaneous Endoscopic Gastrostomy (PEG) is a procedure done in patients with Carcinoma Esophagus and locally advanced head and neck cancers whose nutritional status is compromised. A rare and serious complication of PEG is the metastatic spread to the gastrostomy site. The primary objective was to study risk factors, early detection methods, treatment and possible preventive measures of this very rare complication. Methods: A Pubmed search for published cases of “PEG site metastasis from carcinoma esophagus” was done with respect to risk factors, early diagnosis and treatment. Results: The first case of gastric and abdominal wall metastasis at PEG site in a patient with Carcinoma Esophagus was reported in 1993 by Heinbokel. It occurs in about 0.5-1% of PEG procedures and has a grave prognosis. Interval time from insertion of the gastrostomy tube to development of metastasis ranged from 3-16 months. Only few cases have been reported in literature. Majority of them were stage 3 or 4 squamous cell carcinoma. Pull string method of insertion was found to be a therapeutic risk factor for stomal metastasis and PEG placement prior to definite chemotherapy/radiation increased its chance. While techniques of PEG insertion may contribute to this risk, hematogeneous spread and desquamation theories have also been postulated. Early detection and surgical resection of stomal site lesion when there is complete resection of primary has shown good curative result. Positron Emission Tomography-Computed Tomography (PET-CT) imaging was found to aid in early detection. De Monès et al pointed out that even a hypermetabolic uptake seen on PET-CT images near or around the gastrostomy site must be carefully monitored and histopathological evaluation be done. Palliative radiation can be an effective tool in patients with residual primary lesion. Our case describes a 70 year old lady with Stage IVA squamous cell carcinoma esophagus who underwent PEG insertion prior to radical radiation. 9 months later, she presented with a painful, ulceroproliferative growth at the stomal site. A whole body PET/CT scan showed a metabolically active residual primary in the lower esophagus and a soft tissue deposit in the anterior abdominal wall at the PEG site. It was histopathologically proven to be a metastasis from the primary. Palliative radiation with a dose of 30 Gy in 10 fractions by 3D conformal technique was delivered and good clinical response was achieved. Conclusions: Despite this potential risk, we should not defer PEG tube placement in nutritionally compromised cancer patients. High risk patients should be identified. To prevent direct implantation, contact of gastrostomy tube with the tumour must be avoided by using a sheath. PET-CT imaging can be an important investigational tool in detection of early stomal site metastases. Timing of PEG placement is important because the risk of tumor seeding seems to be higher in cases with active tumor load. Therefore, deferring PEG insertion till the initiation of radiation or chemotherapy will help to decrease this risk.


 > Case Report: 03 Top


Gall bladder carcinoma with extensive skeletal dissemination in a young female: A case report

Aiman Mohammed, Monica Malik Irukulla, S. Fayaz Ahmed, Deepthi Valiyaveettil

Introduction: Gall bladder carcinoma is an extremely rare cancer in young patients. There are very few cases of metastatic gall bladder cancer in very young patients reported in literature. To the best of our knowledge there is no reported case with extensive skeletal and liver metastases at presentation with a silent primary in a patient as young as 20 years. Case Summary: A 20-year-old female with no significant past medical history or family history, initially presented with low back ache and generalised body pains of 3 months' duration and was being evaluated under department of Rheumatology for suspected inflammatory arthritis. Extensive serological testing for inflammatory arthritis panel was negative and during further evaluation she developed paraperesis and her MRI dorso-lumbar spine showed evidence of T2/STIR hyper intense lesion noted at D2, D6, D9, D12, L1, L2 and L4 vertebral bodies with involvement of posterior elements with few lesions appearing expansile and with extradural cord compression at L4 and D12. Biopsy from the lumbar vertebral bodies was ordered which was reported as poorly differentiated malignancy. A panel of immuno-histochemistries was carried out. PanCK was diffuse and strongly positive in lesional cells. Few cells showed nuclear positivity with GATA3. Negative for mammoglobin, and positive for CD 99. Meanwhile her neurological status deteriorated to complete paraplegia and was referred to department of Radiation Oncology with a diagnosis of cord compression due to metastatic disease with an unknown primary. On examination she was ECOG Performance Status 4, with diffuse skeletal tenderness and complete loss of power in lower limbs. There was no pallor, icterus, palpable peripheral lymphadenopathy. Mild hepatomegaly was present. Routine blood counts and blood chemistries were within normal limits. FDG PET-CT showed increased uptake in enhancing soft tissue density lesion with calcification involving fundus of the gall bladder possibly the primary neoplastic lesion with multiple metastases in liver and multiple lytic lesions involving axial and proximal appendicular skeleton with multiple vertebral body collapse. There was increased FDG uptake in right supraclavicular and abdominal lymph nodes. She was treated with palliative radiation on linear accelerator by 3DCRT to D2 in single fraction of 8 Gy and to LS spine in 5 fractions; 4 Gy per fraction to a total of 20 Gy. After completing the RT, there was mild decrease in pain with no improvement in neurological status of the patient. Patient was referred to Medical Oncology for further treatment but expired before starting chemotherapy. Conclusion: A silent gall bladder carcinoma presenting with disseminated skeletal metastases is extremely rare in young patients. Due to the unusual presentation, delay in diagnosis, aggressive biology and advanced disease the prognosis is poor.


 > Case Report: 04 Top


Case report of gall bladder neuroendocrine carcinoma

Harsha Balachandran, A. Sajeed

Department of Radiation Oncology, Regional Cancer Centre Trivandrum, Kerala, India

Neuroendocrine tumors of the gallbladder (NET) account for 0.5% of all neuroendocrine tumors and 2% of the gallbladder (GB) cancers. GB-NETs are asymptomatic and the diagnosis is usually made after cholecystectomy for cholelithiasis. Surgery remains the mainstay of treatment. The role of adjuvant therapy after complete resection is undefined. In metastatic disease, chemotherapeutic drugs have shown some activity. We report the case of a 65 year old female who was evaluated for left neck swelling and back pain radiating to chest. Fine Needle Aspiration done from the neck nodal mass. Evaluation with CECT and PET imaging was suggestive of gall bladder malignancy along with extensive lymph node metastasis. Biopsy from left supraclavicular lymph node confirmed the diagnosis of metastatic poorly differentiated neuroendocrine carcinoma. The patient received 6 cycles of chemotherapy. After completion of 6 cycles, reassessment scan showed partial tumoral response. GB-NEC is a special type of gallbladder carcinoma with low incidence rate. This disease has no specific clinical presentation. Pathological and immunohistochemical examinations are needed for definite diagnosis. GB-NEC are generally locally aggressive and lymphatic metastasis can be found at early stage. The prognosis of GB-NEC is poorer than gallbladder adenocarcinoma. Combined modality treatment may help increase patient survival. Only few studies focused on this disease and no universally accepted treatment recommendation is available.


 > Case Report: 05 Top


Neuroendocrine neoplasms in anal canal

Jyothi K. Thomson, A. Sajeed

Department of Radiation Oncology, Regional Cancer Centre Trivandrum, Kerala, India

Introduction: Anal canal neoplasms are rare and constitute 2.4% of gastrointestinal malignancies. Prognosis of anal canal tumour depends on the histological type. Neuroendocrine tumour accounts to 1%of all anal cancer. Case Reports: We present two cases of neuroendocrine neoplasms in anal canal. CASE 1Forty year old female evaluated for occasional bleeding per rectum. Examination revealed a one centimeter nodular growth just inside the anal verge and excision biopsy reported as neuroendocrine tumour, WHO Grade II, MIB-1: 2-4%. Post excision Dotate PET CT revealed no somatostatin receptor uptake and she is kept under regular follow up. CASE 2 Thirty four year old male evaluated for bleeding per rectum, altered bowel habits, malena and inguinal lymphadenopathy and per rectal examination revealed ulceroproliferative growth 1 cm from the anal margin on left lateral wall and upper limit not palpable. Biopsy from the lesion suggestive of large cell neuroendocrine carcinoma, WHO Grade III, Ki 67-100%. Metastatic work up revealed anorectal lesionwith lung, liverand bone metastasis and pelvic and inguinal lymphadenopathy. Conclusion: Carcinoid tumors affect younger age group than others. As the anal canal tumors are rare, no single prospective trial has been compared the different treatment methods available and analyzed the survival rates. The two case scenarios have shown how different the neuroendocrine neoplasm can present and finally the prognosis may depend on the primary tumor size, histopathology, grade, proliferative index and presence of metastasis.


 > Case Report: 06 Top


Endometrial stromal sarcoma: A rare site presentation

Abraham Eapen John, Aravindh S. Anand, Sneha John, S. Sachin, Vivekanand.K

Department of Radiation Oncology, Government Medical College, Trivandrum, Kerala, India

Introduction: Primary extra uterineendometrial stromal sarcoma (EESS) is very rare but it has been reported to occur at various sites like ovary, abdomen, peritoneum and pelvis.[1] We are presenting a rare site presentation of primary extra uterine ESS. Methodology: 62 year old postmenopausal lady presented to our department with complaints of lower abdominal pain for the past 6 months. On examination she had an ill-defined intraabdominal mass, 10 x 10 cm, in the right iliac fossa and lumbar region extending to the midline. CT abdomen showed a right sided abdominopelvic mass lesion with irregular calcifications. She was taken up for exploratory laparotomy + TAH + BSO + extended R hemicolectomy with ileotransverse anastomosis. Para aortic, inter aortocaval and common iliac lymph nodes were removed. Macroscopic appearance showed a large irregular nodular mass adherent to large intestine, caecum and right adnexa measuring 15x16x9 cm. Cutting open the mass showed grey white c/s with areas of calcification and necrosis. Microscopy revealed neoplasm composed of spindle shaped cells in sheets and nests with extensive areas of necrosis. Individual cells are small round to spindle shaped with moderate eosinophilic cytoplasm, elongated nucleus and fine chromatin. IHC revealed CD 10 diffuse positivity with negative CD 117, desmin and CD 99. Uterus, ovaries, tubes, appendix and colon and ileum were normal. 9/9 lymph nodes showed reactive changes. Final diagnosis of extra uterine endometrial stromal sarcoma (high grade) was made. Discussion: Most of the cases of EESS reported in literature were located in the abdominal peritoneum.[1] Occurrence around the colonic wall is exceedingly rare, with only 10 reported cases in the literature. 8 out of 10 studies showed associated foci of endometriosis. However in our case no such foci were identified. Endometrial stromal sarcoma can be a diagnostic challenge when encountered in the abdomen. ESS should enter the differential diagnoses along with common spindle cell tumours of the abdominal peritoneum such as GIST andleiomyosarcoma. Immunohistochemistry is important in distinguishing these tumours. Complete surgical removal of EESS is the treatment of choice, as has been performed in this case. As the presentation of EESS is very rare the treatment is purely based on the available case reports and extrapolating the treatment for ESS. Hence we are planning the patient for adjuvant systemic chemotherapy with docetaxel gemcitabine combination. Conclusion: An unexpected location and an unusual presentation of an EESS may make the diagnosis challenging, despite classic histologic features.

References

  1. Masand RP, Euscher ED, Deavers MT, Malpica A. Endometrioid stromal sarcoma: A clinicopathologic study of 63 cases. Am J Surg Pathol 2013;37:1635-47.
  2. Ghosal T, Roy A, Kurian S. Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis. Indian J Pathol Microbiol 2014;57:447-9.



 > Case Report: 07 Top


Malignant melanoma of cervix in a young female: A rare case report

Purvi Rathod, Ashok Kumar Diwan, Subeera Khan

Malignant melanoma is a rare cancer, its incidence being 1% of all cancers. Malignant melanoma of the female genital tract is even rarer with incidence of 3% to 7% of all malignant melanomas. Malignant melanoma of cervix usually occurs in postmenopausal women, between 60 to 70 years of age. We present a case of malignant melanoma of the uterine cervix in a mutipara premenopausal 29 year old female. She presented with foul smelling blackish brown per vaginal discharge since 6 months. Clinical examination and MRI pelvis was s/o soft tissue lesion in the uterine cervix and anterior wall of vagina 3.5x2.7x2cms, there was no parametrial invasion. She underwent a modified radical hysterectomy with bilateral internal iliac and right external iliac lymph node dissection. Her postoperative histopathology was showing cells with melanocytic proliferation in the squamous epithelium. 3mm deep infiltration in stroma without vascular invasion. 1 left internal iliac lymph node positive for metastases. A metastatic work up with CECT thorax and abdomen was done to rule out distant metastasis. She was stage III (T1N1). She was treated with external beam radiotherapy to the pelvis 45Gy/25# followed by 21Gy/3# of intravaginal HDR brachytherapy. Since then the patient is on regular follow up from past 4 months. She has no signs of residual disease and distant metastasis. Malignant melanoma of the cervix being a rare case has very less data in literature. Through this case report we would like to add some more data to the literature and emphasize on the role of radiotherapy in malignant melanoma of uterine cervix.


 > Case Report: 08 Top


Primary orbital yolk sac tumor

Etisha Rajput, Vijay Mahobia, Ashok K. Diwan

Introduction: Yolk sac tumor (YST) (endodermal sinus tumor) is a common malignancy occurring in the gonads. Primordial germ cells migrate from yolk sac to genital ridge. Rarely due to aberrant migration, ectopic primitive germ cell or defect in embryogenesis can it occur in sites like uterus, vagina, liver, prostrate and head and neck. Extra gonadal germ cell tumor of the head and neck comprise only 5% of benign and malignant germ cell tumors, with yolk sac tumor being the most common histopathological subtype. Primary yolk sac tumor (YST) of orbit is rare and only few cases have been reported in the literature. Its clinical presentation may mimic many common pediatric orbital conditions, and delay in diagnosis affects ocular morbidity and mortality. Case Report: A 2 year old, male child presented to our OPD with complaints of complete loss of vision and progressively increasing gross proptosis of right eye since 2 months. Initial diagnosis of malignant round cell tumor of eye was made on biopsy. Magnetic resonance imaging (MRI) of the head showed lesion in extraconal compartment of right orbit with small intraconal component. However with further work up, diagnosis of primary orbital yolk sac was confirmed with immunohistochemistry (IHC) and raised alpha-feto protein levels (>1210 ng/ml). No other ‘primary tumor’ or metastasis was detected by imaging of brain, abdomen, and chest. A treatment plan comprising of 6 cycles neoadjuvant chemotherapy (NACT) followed by debulking surgery was made. Patient has received 4 cycles of NACT (comprising of etoposide and cisplatin) every 21 days and had an excellent response both radiologically and cosmetically. Discussion: Orbit is a rare primary site for yolk sac tumors. It more commonly affects females with a male: female ratio of 1:1.5. Diagnosis between rhabdomyosarcoma and yolk sac tumor is challenging in orbital tumors. For the diagnosis of latter, immunohistochemistry and alpha fetoprotein levels are helpful. Both computed tomography (CT) and MRI can be used to determine the size, location, and extent of the lesion. Due to its rarity no clear management guidelines exist. Multimodal therapy combining surgery, chemotherapy, and radiotherapy have been used in the past. Conclusion: Primary orbital yolk sac tumors although rare should be kept as differential diagnosis for aggressively behaving tumors of eye in younger children. Yolk sac tumors are extremely malignant and tend to recur locally. They may present with early metastases in 50% of the cases. Extra gonadal germ cell tumors have a worse prognosis than gonadal tumors. However, intra orbital yolk sac tumors have better prognosis due to their early recognition. Recognition and early diagnosis is essential for appropriate management and prognosis.


 > Case Report: 09 Top


Nonmetastatic lung opacity in early breast cancer

Jamuna Angel Joy, C. Jomon Raphael, K. Mathew Varghese, G. Paul Gopu, Shebin George

Department of Radiation Oncology, Amala Institute of Medical Sciences, Thrissur, Kerala, India

Introduction: Carcinoid tumor, a type of slow-growing cancer affecting several parts of the body, often does not cause signs and symptoms until late in the disease. 10% of the carcinoid tumors originate in the lung. Compared to the general population, those with Lung Carcinoid tumors have an increased risk for subsequent development of Breast and Prostate cancers. Management of a lung carcinoid is different from other lung malignancies, and has better prognosis. Hence identifying the disease is of utmost importance. Case Presentation: A 56 year old post-menopausal lady was evaluated for lump in the right breast for 4 months duration. FNAC, trucut biopsy showed invasive ductal carcinoma. Metastatic work up with Chest X-ray revealed presence of right lung lower lobe mass lesion suspicious for metastases. She was further investigated with PET CT scan which showed malignant right breast lesion (SUV max 8.0), soft tissue lesion in right lung lower lobe (SUV max 3.3) with differential diagnosis of a second primary or metastases. In view of suspicion of second primary, her case was discussed in MDT and she was planned for Right MRM with Right lower lobectomy. MRM histopathology reportwas Invasive Ductal Carcinoma Right breast, pT2N1aM0. Right lower lobectomy histopathology report, to our surprise, was suggestive of Right Lung Lower Lobe Carcinoid Tumor. She received 6 cycles of Adjuvant chemotherapy and Post Mastectomy Radiotherapy. This patient has been on regular follow-up, and has been clinically and radiologically disease free for last two years. Conclusion: A case which could have been easily misdiagnosed as metastases changed its course owing to high index of suspicion and timely action. The inconsistent clinical presentation of carcinoid tumors makes it difficult to make an accurate diagnosis at presentation. Surgical management remains the mainstay of therapy for lung carcinoid tumors and can be curative. Hence a multidisciplinary approach to work up and treatment should be utilized for each patient. Presenting Author-Dr. Jamuna Angel Joy.


 > Case Report: 10 Top


Upper limb monoparesis of unknown cause

Jomon Raphael, K. Mathew Varghese, G. Paul Gopu, Shebin George

Department of Radiation Oncology, Amala Institute of Medical Sciences, Thrissur, Kerala, India

Introduction: Dermatomyositis is a well-known but rare autoimmune idiopathic inflammatory disease whose association with different types of malignancies have been reported in several studies. Most common malignancies associated with dermatomyositis were carcinomas of nasopharynx (21%), breast (15%), lung (15%), ovary (9%), colon (5%), NHL, cervical, and bladder cancers. There have also been few reports on dermatomyositis in association with recurrence and/or progression of tumor disease, which have a poor outcome. Treatment of dermatomyositis in such patients constitute management of both cancer and dermatomyositis. Case Presentation: A 45 year old lady was evaluated for bleeding per vaginum, in 2009. She was diagnosed with Carcinoma Endometrium FIGO Stage II and underwent TAH + BSO. Post-surgical evaluation showed multiple bone metastases. In view of metastatic disease, she received 6 cycles of Palliative Chemotherapy and was on regular follow-up. She was asymptomatic for 8 years. In 2018, she presented with difficulty in combing hair, and getting up from lying down position, which was evaluated thoroughly under Department of Neurology. No neurologic cause was found, and her monoparesis slightly worsened. She also noticed to have rash over her face. She was referred to Dermatologist and on examination, heliotrope rash, V-sign, Gottron's papules were noted. Clinical examination and biopsy from skin were suggestive of Dermatomyositis. To rule out a paraneoplastic event, she was referred to Department of Oncology. There was no evidence of malignancy clinically. PET CT scan showed FDG avid enlarged left external iliac lymph node (21x20x30mm) (SUV max 11.2). Her case was discussed in MDT and was decided to undergo biopsy from the involved lymph node. But in view of vascular proximity of node, she underwent Exploratory Laparotomy-Bilateral Pelvic Lymph Node Dissection + Inferior Omentectomy. Histopathology examination was reported as High Grade Serous Carcinoma – Left Iliac Lymphnode and Right Iliac perilymphnode fat metastases. 2 weeks post-surgery, her cutaneous and musculoskeletal features of dermatomyositis showed very good response, irrespective of the course of malignancy. Conclusion: This case illustrates that underlying malignancy can be missed easily if not thought of in patients presenting with skin rash and muscle weakness. An early detection and an appropriate therapy is crucial for a favorable outcome.


 > Case Report: 11 Top


Marginal zone lymphoma of bilateral buccal mucosa: A case report

Kushboo Jain, Ankita Parikh, U. Suryanarayan

Aim: Non Hodgkin's Lymphoma (NHL) includes a spectrum of behaviour ranging from relatively indolent to highly aggressive and potentially fatal. Extranodal presentation in NHL is relatively common with usual sites being stomach, lung, orbits, sinuses, thyroid, tonsil, salivary glands, breast, and kidney. But primary lymphomas of oral cavity are uncommon consisting of approximately 2% of all extrannodal lymphomas. Wehearby present a case of marginal zone lymphoma in bilateral buccal mucosa (BM), a rare presentation. Materials and Methods: A 49 year male with no comorbidities presented in surgical OPD with complain of ulcer in right BM since 1 year and restricted mouth opening. He had addiction of tobacco and betel nut chewing, Beedi smoking for 20 years. On examinationbilateral BM showed leukoplakic patches and fibrosis. Small erythematous, indurated area was present over right BM. No clinically palpable cervical lymph nodes. Biopsy was suggestive of low grade NHL in right BM and in left BM. IHC was suggestive of NHL, low grade B cell marginal zone type. With CD5, CD20, CD43, CD79a, BCL2 positivity and MIB1 10%. CT scan of PNS and Neck was suggestive of heterogenously enhancing soft tissue thickening involving anterior aspect of both buccal space, maximum thickness of 9 mm on right side. Few subcentimetric nodes were present in b/l level 1B. Bone marrow was uninvolved by lymphoma on biopsy. PET – CT was also performed which revealed absence of hypermetabolic lymphadenopathy on both sides of diaphragm or any other extralymphatic site to suggest disease infilteration. Case was discussed in tumour board. In view of solitary lesion, patient was planned for involved field radiotherapy. 40 Gy in 20 fractions was delivered on linac with 6 with AP-PA fields. During treatment, patient developed RTOG grade 3 mucositis. He developed trismus, allowing 1 finger between the two incisors. Results: After the treatment, patient was followed up on 15th day, and then monthly. On 15 day post-RT follow up, patient had decreased mucositis. On 3rd month, patient had complete resolution of lesion, clinically and radiologically. Patches of leukoplakia also resolved. With continuous physiotherapy trismus also improved. Conclusion: The case described here falls into the European–American classification of a low-grade B-cell lymphoma of the MALT type, with intra-oral lesion. oral cavity lymphoma most commonly presents with a persisting ulcerated swelling but other symptoms may include pain, foetor, paraesthesia, or mucosal discolouration. An exhaustive systemic study should be made to discard its presence in other tissues. Factors known to increase NHL risk include Epstein-Barr virus and inmuno deficiency. There is a tendency for the disease to remain localized for a long time, local treatment, is often indicated. They respond well to most treatments (surgery, radiotherapy and or chemotherapy), for which reason the least aggressive methods tend to be used, at least at the outset. However local recurrence is frequent, including several years after the first diagnosis, and long term follow up is necessary.


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A rare case report of mucinous carcinoma ovary and n acetyl cystine in its management

K. N. Pradeep, L. Ashwini, M. Ankur, S. Pragya

Srinivasam Cancer Center, Bengaluru, Karnataka, India

Introduction: Carcinoma ovary third common malignancy in females 6.2%. The Incidence as per the GLOBACON 2018 data is 3.44% and death rate 3.34%., The ovarian cancers are divided in to epithelial, germ cell, sex cord stromal, metastases miscellaneous. The epithelial are classified by cell type serous, mucinous endomatriod benign border line. Case Summary: We report a case of mucinous carcinoma which is chemotherapy resistant treated with Intraperitonial N acetyl cysteine. A 56 year patient presented with distension of abdomen with past history of Hysterectomy in the year 2014 on evaluation routine investigations were normal with raised CA125 CT Scan abdomen and pelvis showed a diffuse adnexal mass without free fluid patient subjected for lapratomy under general anesthesia on the table it was found a mucine containing adnexal mass total debunking done as it is a chemotherapy resistant tumor likely to recur the operating surgeon has read an article Intraperitonial N acetyl cysteine reduces the risk of recurrence hence intra peritoneal N acetyl cysteine therapy done post operative period was uneventful patient got discharged on day 10 patient given option of adjuvant chemotherapy patient refused to take chemotherapy she was kept on fallow up at the end of one year patient is DFS. Conclusions: N-Acetyl cysteine is a mucolytic agent it causes mucolysis, intraperitoneal administration caused mucolysis in this patient, no adverse events were observed, postoperative period uneventful. Hence it can be an option as it is safe cost effective.


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Radiation therapy with chemotherapy for patients of cervical cancer with isolated supraclavicular lymph node involvement

M. Mohamed Harris, R. Giridharan

Department of Radiation Oncology, Madras Medical College, Chennai, Tamil Nadu, India

Objective: To evaluate the outcomes of cervical cancer patients with isolated supraclavicular lymph node (SCLN) involvement who received radiation therapy (RT) combined with chemotherapy. Methods: From January 2010 to July 2015, 5 cervical cancer patients with SCLN involvement were treated by RT and cisplatin-based chemotherapy. The RT field was designed to include the whole pelvis and the SCLN area. Results: The median follow-up period was 3 years and the patients were found to have better outcomes with minimal post chemo radiotherapy complications. Conclusion: For patients with advanced cervix cancer with isolated SCLN involvement, Radiation with chemotherapy as active therapy can be expected to provide favourable results.


 > Case Report: 14 Top


A rare case of malignant carotid body paraganglioma

D. Piyushi, M. Bhadauria, R. Masodkar, V. P. Singh

Introduction: Among Head and neck paragangliomas which have an estimated incidence of 1-10 cases per million people per year, Carotid Body paragangliomas are most common. About 5% of these tumours may show malignant potential. Due to the rarity of this tumour, their management remains challenging and therefore the need for reporting of cases encountered in day to day practice by various centres. Case Summary: A 26 yr old lady presented with one and half year history of non-tender, non-progressive swelling over left side of upper neck, pain and tingling sensation over left arm and history of on and off headache. DOTA-NOC-PET-CT revealedapprox. 2.5 cm mass lesion in the left carotid sheath along with Left retropharyngeal and cervical level II lymph nodes. She underwent pre-op embolization followed by Surgical excision. HPE was suggestiveofMalignant paraganglioma of size 3cm, completely encasing carotid artery with positive margin, 2/30 lymph nodes positive withperinodal infiltration. IHC was strongly positive for synaptophysin. She received Adjuvant Radiotherapy 54 Gy to the post op tumor bed along with ipsilateral neck nodes and 46 Gy to contralateral nodes, using IMRT technique. Results: Patient has been on regular follow up with three monthly clinical examination and imaging. Six months post treatment, the DOTA-NOC-PET-CT revealed no evidence of SSTR expressing lesion in left carotid sheath region or rest of the body. She remains disease free one year post treatment. Conclusion: Very few reports ofMalignant Carotid body paraganglioma have been published. Locoregional control is best achieved with primary resection followed by adjuvant radiotherapy. Due to the documented late onset recurrence, long term follow up and surveillance becomes necessary to improve survival.


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Clinico-pathological features and outcomes of adreno-cortical carcinoma-a single institution experience

Lekha M. Nair1, K. M. Jagathnath Krishna2, Aswin Kumar1, Susan Mathews1, John Joseph1, Francis V. James1

1 Department of Radiation Oncology, Regional Cancer Centre Trivandrum,2 Biostatistics and Epidemiology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Background and Objectives: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with aggressive behaviour. Most of our knowledge about this rare tumor is based on retrospective case series. This study aimed at analysing the clinico-pathological features and outcomes of patients treated at a tertiary cancer centre in India. Materials and Methods: We retrospectively reviewed the data of patients with adrenocortical carcinoma registered from 1st January 2006 to 31st December 2015. Results: Thirty seven patients were included in the study, 20 males and 17 females. Median age was 49(18-78). Hormonal overproduction was noticed in 27% of patients. Median tumor size was 10cm(2-22). Seventeen patients had metastatic disease and 20 patients were non metastatic at diagnosis. Median follow up was 22 months and median overall survival (OS) was 23.46 months. OS at 2 years and 5 years were 46.1% and 21% respectively. The median Disease Free Survival (DFS) was 20 months. DFS at 2 years and 5 years were 45% and 24% respectively. Age, sex, tumor size, hormonal overproduction, tumor laterality and stage of the disease did not influence survival. But advanced stage was associated with higher risk for recurrence (p=0.03). Conclusion: ACC is a rare endocrine malignancy with very poor survival rates. Rate of recurrence is high even after complete surgery. Systemic treatment options are limited. Newer agents are needed to improve outcome.


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A rare case of duodenal adenocarcinoma of D3-D4 segment treated by neoadjuvant chemoradiotherapy followed by surgery

Sankalp Singh, Niharika Bisht, Samir Gupta, Amul Kapoor, Richa Joshi

Aim/Objective: The aim of our study is to present a rare disease of duodenal carcinoma and share our experience of successful implementation of a non-standard treatment protocol of neoadjuvant chemo-radiotherapy followed by surgery. Introduction: Adenocarcinoma of the small bowel accounts for only one per cent of all gastrointestinal malignancies. Duodenal adenocarcinoma accounts for half of all small bowel adenocarcinomas. The duodenum is divided into four segments: D1 (proximal horizontal 5 cm beginning with the 3-cm duodenal bulb), D2 (descending), D3 (distal horizontal), and D4 (ascending). The most common location of duodenal adenocarcinomas is the ampullary region of D2 with lesions in D3 and D4 being much rarer and lesions of D1 segment almost unheard of. Duodenal adenocarcinoma is a rare but aggressive malignancy. Complete surgical resection is recommended when technically feasible. Materials and Methods: We present a case of a 47 year old man who presented with vomiting and pain abdomen. Upper GI endoscopy revealed a growth in segment D3 and D4 of duodenum. Biopsy of this growth showed an adenocarcinoma. Positron Emission Tomography revealed a hypermetabolic mass in D3 segment of duodenum causing luminal obstruction along with few regional lymph nodes. An exploratory laparotomy revealed the tumour to be encasing the superior mesenteric vessels deeming it to be unresectable. The patient was treated with neoadjuvant radiotherapy on a telecobalt machine to a dose of 5040cGy in 28 fractions along with Inj 5-flurouracil on days 2 to 5 and 29 to 32 concurrently. A repeat PET CT scan showed significant reduction in the size and metabolic activity of the primary tumour and lymph nodes. He subsequently underwent a Whipple's pancreatico-duodenectomy and the tumour histopathology revealed all margins to be negative with partial response to chemoradiotherapy. The post-op convalescence was prolonged due to delayed wound healing but the patient has remained disease free at one year post surgery. Results: The serial post op PET CT scans have revealed no evidence of residual or recurrent disease at 12 months after surgery. Conclusion: Neoadjuvant treatment in the form of chemoradiotherapy may be a feasible option in unresectable cases of duodenal carcinomas in carefully selected cases. There is paucity of data on this subject due to extreme rarity of this tumour and further studies are certainly warranted.


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Gastric mucinous adenocarcinoma of uterine cervix: A rare case

Tejaswita Singh, Surabhi Gupta

Department of Radiation Oncology, Agra, Uttar Pradesh, India

Introduction: Endocervical adenocarcinoma represents approximately 20%– 25% of cervical cancers, with increasing incidence in recent years.[1],[2],[3],[4],[5] According to WHO various types of adeno carcinomas of cervix are-usual type adenocarcinoma, mucinous carcinoma (gastric, intestinal, signet-ring cell type), villoglandular carcinoma, endometrioid carcinoma, clear cell carcinoma and others. Above all gastric type, as a subtype of mucinous adenocarcinoma shows distinct morphologic features, gastric phenotype and an aggressive clinical course. We report a rare and interesting case of stage IB1 GAC with negative HPV DNA and p16. Summary: A 46 year old multiparous menopausal woman was referred to our out patient clinic with chief complaint of whitish discharge per vaginum with intermittent episodes of bleeding abnormal pap smear suggestive of squamous cell carcinoma. On further investigations, the ultrasound whole abdomen and transvaginal sonograph revealed bulky cervix showing ill defined soft tissue mass lesion measuring about 26*23 mm in size, fat planes with bladder and rectum being preserved. CECT scan of pelvis revealed bulky and heterodense soft tissue lesion measuring approximately 38mm*34mm in size and 31 mm craniocaudal in extent. No significant streaking of parametrial fat was seen and fat planes with adjoining fat planes were conserved. She was reffered for surgical opinion, following which she underwent panhysterectomy at our hospital. Thepost surgical specimen consisted of firm white growth measuring 2cm*2cm and histopathology confirmed gastric mucinous adenocarcinoma of cervix. She was planned for concurrent chemoradiation. She received 6 cycles of weekly cisplatin (40 mg/m2) with a total dose of 50.4 Gy pelvic irradiation for 6 weeks. Conclusion: GAC is a rare variant of mucinous adenocarcinoma not associated with HPV infection. And p16 immunohistochemistry is also a very useful marker to distinguish endocervical adenocarcinoma from the benign diseases, but it is typically negative or focal in GAC that is HPV-unassociated group. Not only HPV DNA but also p16 protein are usually positive in the usual type, but are almost negative in GAC with only rare exception. Our GAC case also shows negative HPV DNA and p16 immunohistochemical stain. In challenging cases, CEA may be a good marker to differentiate these tumors because CEA is usually negative in clear cell carcinoma but increase in GAC [. But in our case, CEA was within normal range.


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Isolated skeletal muscle metastasis in a case of adenocarcinoma lung

Angela Elizabeth Eapen, S. Roshni, A. L. Lijeesh, A. Sajeed, M. H. Geethi, Arun Sankar, C. D. Sivanandan

Department of Radiation Oncology, Regional Cancer Centre, Trivandrum, Kerala, India

Introduction: Lung cancer is one of the most common cancers and cause of cancer related death worldwide. Almost 50% are metastatic at the time of diagnosis with spread to the brain (10%), bone (7%), liver (5%), and adrenal gland (3%). [1,2] The skeletal muscle though, is a very rare site of metastasis. This case report is of a patient withnon-small cell carcinoma lungwho developed solitary metastasis to the gluteus muscle. Case Report: A 65 year old gentleman presented with complaints of dry cough, breathing difficulty and significant weight loss. He was evaluated with a CT scan of the chest at a local hospital which showed a discrete lesion in the upper lobe of the right lung with pretracheal and ipsilateral hilar lymphadenopathy. He was then referred to our institution where a guided trucut biopsywas done. It was reported as adenocarcinoma lung with cytokeratin 7 (CK7) and thyroid transcription factor-1(TTF-1) positivity. FDG-PET scanshowed significant uptakein the lung lesion and ipsilateral paratracheal node-stage IIIB. He was treated with definitive chemoradiation-60 Grey in 30 fractions over sixweeks by Intensity Modulated Radiation Therapy (IMRT) technique and concurrent chemotherapy with Carboplatin and Etoposide. He remained on follow up after treatment completion. After four months, he developed a swelling of size 10x6cms in the left gluteal region. FDG-PET showed a solitary lesion with no evidence of metastasis elsewhere. FNACdone wascompatible withadenocarcinoma lung. He was advised surgery as the gluteus muscle was the only site of metastasis, but he was unwilling for the same. As he was symptomatic with pain, he was given short course radiotherapy-30 Grey in 10 fractions (3 Grey/day x 5 days). The swellingmarkedly reducedin size and the pain alleviated. Molecular testing for epidermal growth factor receptor (EGFR), anaplastic lymphoma kinase (ALK) and a receptor tyrosine kinase (ROS-1)was done which showed EGFR positivity. He was planned to be started on Tyrosine kinase inibitors. Discussion: The skeletal muscleis a rare site of metastases in carcinoma lung. It is usually associated with metastasis at multiple sites. Di Giorgioet al. who reported a study of 3000 patients treated for lung cancer, described only three cases showing SMM.[3] This case is reported because of the rarity of the presentation of skeletal muscle as the only site of metastasis in lung cancer. Conclusion: Solitary skeletal muscle metastasis in adenocarcinoma lung is extremely rare. For symptomatic patients who are unwilling for surgery, palliative radiation could be a reasonable option.

References

  1. Patchell RA, Tibbs PA, Walsh JW, Dempsey RJ, Maruyama Y, Kryscio RJ, et al. A randomized trial of surgery in the treatment of single metastases to the brain. N Engl J Med 1990;322:494-500.
  2. Albain KS, Crowley JJ, LeBlanc M, Livingston RB. Survival determinants in extensive-stage non-small-cell lung cancer: The Southwest Oncology Group experience. J Clin Oncol 1991;9:1618-26.
  3. Di Giorgio A, Sammartino P, Cardini CL, Al Mansour M, Accarpio F, Sibio S, et al. Lung cancer and skeletal muscle metastases. Ann Thorac Surg 2004;78:709-11.



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Chondroid syringoma: Pattern of presentation

Bindiya Vijayan, Mahalaxmi Aal, Pradeep Kumar, Vijay Bhaskar

Aim: Sweat gland tumors are very rare among the malignant tumors of skin. Malignant Chondroid Syringoma (MCS) an eccrine sweat gland malignantneoplasm is one of the rarest subtypes and, unlike its benign variety usually presents in trunk and extremities. Here we present a case of a38-year-old lady who is a case of Metastatic Malignant Chondroid Syringoma, previously treated for the recurrences, status post adjuvant chemotherapy and radiation therapy. Introduction: A 38-year-old lady presented with recurrent MCS as complaints of swelling in the left breast. She underwent wide local excision + excision of nipple areolar complex. Post Op HPE revealed Metastatic Malignant Adnexal Tumor. She was alreadydiagnosed withand treated for MCS, at the age of 18 years, 13 years back in the 1st web space of left hand. In a span7 years she has underwent multiple surgical excisionsin this initial site due to the development of recurrences at the same site after 2 years, and she receivedExternal Beam Radiotherapy (EBRT) to a dose of 60 Gy in 30 fractions to the left hand. She was on follow up and after a disease free interval of 2 years she developed recurrence in the left axilla. She received 6 cycles of CT with Paclitaxel+Carboplatin and underwent Lumpectomy. This was followed by EBRT to dose of 60 Gy in 30 fractions to the left axilla + mediastinum. After 6 months she developed recurrence with 2 lesions in the left breast, with which she presented to us and after 1 month she developed recurrencein left thenar space, for which she underwent WLE + nipple areolar complex excision. And she was planned for adjuvant radiotherapy. Meanwhile, she was found to have distant metastases when she was evaluated for complaints of pain over right thigh on lateral aspect. She was evaluated with PET CT scan which revealed wide spread metastases to liver, left kidney, pancreas, lungs, head and shaft of right femur with extensive tumor thrombus in the above mentioned visceral organs. IHC was consistent with Metastatic Skin Adnexal tumor-ChondroidSyringoma to the liver. She was treated with palliative EBRT to the skeletal lesions in view of pain. Conclusion: MCS behave more aggressively than other skin carcinomas, with a series of local recurrences over a span of many years; in this case starting from web space of left hand to the left axilla followed by left breast and left thenar space with extensive local recurrencesandeventually to various visceral organs, over a span of 13 years. The wide surgical excision, adjuvant radiation therapy, with or without chemotherapy as well as patient education regarding regular follow-up for recurrences are critical in facilitating long term survival of the patients.


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Case report of primary breast rhabdomyosarcoma in a 17-year-old girl

Jayavignesh, N. V. Kalaiarasi, Giridharan, Sundaresan, Sanjalkumar, Poonkodi, Vijeykarthik, Senthilkumar

Department of Radiation Oncology, MMC, Chennai, Tamil Nadu, India

Objective: To report the occurrence of a rare case of primary rhabdomyosarcoma in breast in a 17 year old girl. Materials and Methods: A 17 year old girl developed a lump in left breast central quadrant during the last week of september 2016. The lump was gradually progressive associated with pain for which she underwent excision biopsy on 3.10.16 and the pathology report came as juvenile fibroadenoma. 3 weeks later the patient again developed left axillary lymphnodes, the fnac was positive for malignancy. patient underwent left MRM on 9.2.17. the HPE report came as small round bluecell tumour-rhabdomyosarcoma, with superior and inferior margins positive for tumour infiltration. 5/11 nodes were positive for metastasis. ihc report showed postive CD99 and diffuse strongly nuclear positive myogenin in both breast and nodal tissue. pathological staging was t4bn2mo. patient underwent 8 cycles of vac/ie regimen chemotherapy. CT chest showen chest wall recurrence with multifocal tiny centrilobar nodules in right lung lower lobe. patient was taken up for palliative radiotherapy to chest wall in 200CGY/#, 50 Gy in 25# for 5 weeks with medial and lateral tangenitial fields to treat the chest wall. completed rt on 23.9.17 and discharged. Results: Patient was on regular followup with both radiation oncolgy and medical oncology departments until march, 2018. patient expired in her home on may, 2018. Conculsion: Primary rhabdomyosarcoma of breast is a rare disease account for less than 0.2% of all breast cancer patients, and 5 year survival rate was 43 %. delay in diagnosis may lead to poor outcome in such cases.


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Intraabdominal desmoplastic small round cell tumor: A case report

C. S. Akhilesh, A. L. Lijeesh, S. Roshni, A. Sajeed, Arun Sankar, M. H. Geethi, C. D Sivanandan

Department of Radiation Oncology, Regional Cancer Centre, Trivandrum, Kerala, India

Introduction: Desmoplastic small round cell tumor is a rare malignancy with poor prognosis that predominantly affects young males. Its etiopathogenesis is still unknown and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. Due to limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and to avoid misdiagnosis. Case Presentation: We report a case of a 21-year-old male who presented with non-specific abdominal pain of 6 months duration. Physical examination revealed a palpable mass in his epigastric region. Computed Tomography scan showed exophytic cystic lesion in the abdominal cavity closely related to peritoneal surface of fundus of stomach and hilum of spleen. Similar lesions were noted in the caudate lobe, segment V, VI and VIII of liver, spleen and posterior-basal segment of lung. He underwent USG guided biopsy from the peri-portal node which was suggestive of desmoplastic small round cell neoplasm. Conclusion: Diagnosis of desmoplastic small round cell tumor can easily be missed because it presents with few early warning symptoms and signs, while the routine blood tests are within normal limits. A high degree of suspicion, a thorough physical examination, a full imaging check and an aggressive therapeutic approach are required in order to identify this disease and fight for a better quality of life for these patients.


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Role of radiotherapy in central neurocytoma: A single institutional experience

S. Arun Raj, B. Subathira, I. Bhargavi, Y. V. Lokesh, Prathap K. Reddy

Reddy Apollo Cancer Institute, Chennai, Tamil Nadu, India

Aims/Objectives: Neurocytomais a rare benign neuronal tumor which represents about 0.1-0.5% of all primary CNS tumors. Radical excision is the mainstay of treatment. The role of definitive or adjuvant radiation therapy is not well defined in neurocytomas. The aim of this case series is to evaluate the treatment strategy and the role of radiotherapy in Central Neurocytoma based on our institutional experience. Materials and Methods: We have done a retrospective review of 9patients with neurocytoma treated at our centre from 2012 to 2016. All the patients had a histological confirmation of diagnosis by stereotactic biopsy or surgical resection. We present the demographic details, clinical manifestations, treatment strategies and outcome of these patients. Results: There were 9 patients who were diagnosed with aNeurocytoma and who underwent Radiotherapy in our department. The median age of presentation was 26 years (20-56 years). 5 were male (55.5 %) and 4 were female (44.4 %). The commonest presenting symptom was headache associated with gait and visual disturbances (77.7%). All the tumors were intraventricular, with lateral ventricle being the commonest location. Surgery was excision (5/9) or stereotactic biopsy (4/9). The patients who underwent excision, had a post-operative MRI showing residual tumor. All the patients underwent Post-Operative/Definitive Radiotherapy (3DCRT, IMRT, IGRT, SRT). The median dose delivered was 50.6 Gy in conventional fractionation and 25Gy/5 # for the one patient treated with SRT. On follow up at 4 months post Radiotherapy, one patient had 50% regression, one had Progression and the rest were symptomatically better. Conclusions: Neurocytoma is a benign tumor of the Brain, commonly presenting with symptoms of raised ICT owing to its intraventricular location. Surgery forms the mainstay of treatment for these tumors. However in the presence of residual tumor or due to inoperability (comorbidities/eloquent location), Radiotherapy has a major role in the management of these tumors in the post-operative/Definitive setting, based on the type of surgery. Radiotherapy seems to be well tolerated and effective. However, our series requires a longer follow up for response assessment.


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Intramedullary spinal cord metastasis from carcinoma breast: Rare case

R. Dinesh Kumar, N. V. Kalaiyarasi

Background: Intramedullary spinal cord metastasis (ISCM) from breast cancer is relatively rare entity. Case Presentation: The patient was a 40 year old woman with lung metastasis appearing 2 years after breast conservative surgery. Complete remission was achieved for the metastatic lesion with chemotherapy. But multiple cerebral and cerebellar metastasis were found 4 months after completion of chemotherapy. Whole brain irradiation and steroid was administered, resulting in symptomatic improvement. Simultaneously MRI dorsolumbar spine taken for back pain, showed ill defined T2 Hyperintense intramedullary lesion in dorsal cord 17 x 10 mm at D4-D5 level and at conus 15 x 10 mm at D12-L1 level. In vertebra altered signal of D2, D3, L2, L5 vertebra noted and the patient is started on irradiation to the intramedullary spinal and vertebral metastasis. We herein report the case with a review of the relevant literature.


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A rare case report of porocarcinoma of abdominal wall

C. Saranya, Giridharan

Introduction: Eccrine porocarcinoma (EPC) is a rare malignancy of eccrine sweat glands. It is often seen during the sixth to eighth decades of life. EPC is a rare and aggressive tumor with an incidence of 0.005% to 0.01% of all epithelial cutaneous tumors. It carries high risk of local recurrence, regional lymph node invasion, and distant metastasis and most commonly affects the lower extremities. Herewith we report a case of eccrine porocarcinoma arising on the abdomen of a 55-year-old female. Case Presentation: A 55-Year-old female presented complaints of enlarging mass over left upper abdomen for 3 months. Abdominal examination revealed an ulcerated swelling in left hypochondriac region with slight serous discharge. CT scan of the abdomen and pelvis with contrast revealed a irregularly lobulated heterogeneously enhancing lesion involving skin and subcutaneous plane of left hypochondrial region. Edge wedge biopsy performed showed malignant adnexal tumour. Wide local excision with axillary node dissection was done. Histopathological examination revealed an eccrine porocarcinoma. Discussion: EPC is a rare and aggressive tumor. It may occur de novo or as a result of malignant transformation of an eccrine poroma. A long period of clinical history is often encountered. It usually occurs on the lower extremities followed by the, trunk, head and neck, and upper extremities. The clinical picture usually consists of a painless nodule or papule. Treatment is wide local excision. No strong evidence exists for adjuvant therapy. The risk of local recurrence is about 20%. Conclusion: High index of suspicion is required for diagnosis of EPC. Early diagnosis is achieved by histopathological examination and early definitive surgical excision leads to excellent results.


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Recurrent breast sarcoma in a young female

D. Niharika, R. Kavitha

Aims/Objectives: Rhabdomyosarcoma (RMS) is a common soft-tissue neoplasm in the pediatric age group. Common locations are head and neck, genitourinary areas, trunk, and extremities. The involvement of breast is rare with limited case reports in literature, little is known regarding the clinical behavior and treatment strategies. Diagnosis of RMS should be considered when we see a breast mass especially in young age groups for early management. Materials and Methods: Here we report a young girl with recurrent Rhabdomyosarcoma of breast treated with wide local excision followed by MRM at the time of first recurrence. Later she received 8weeks of chemotherapy and then adjuvant radiation to chest wall followed by continuation of chemotherapy. Results: Patient developed recurrence during chemotherapy and was managed accordingly. Conclusion: Rhabdomyosarcoma of breast should be kept as one of differential diagnosis in adolescent females, unless strong family history of invasive ductal carcinoma of breast is present. Diagnosis and treatment will be challenging. We believe that RMS of breast is an aggressive malignancy and need more studies to comment on prognosis and survival rates.


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A rare case of parapharyngeal spacesynovial sarcoma

Neelam Singh, Sapna Nangia, Robin Khosa, Jalaj Baxi, Maneesh Singh, Noopur Gupta

Introduction: Synovial Sarcoma is a rare malignancy of mesenchymal pluripotent cells. Synovial Sarcoma primarily occur in the periarticular region and are predominantly located in the extremities. They account for less than 10% of all body sarcomas and less than 3% occur in the neck. The first case of synovial sarcoma in neck was reported by Jernstorm in 1954. Diagnosis and management are challenging due to its low incidence. We hereby present an unusual case of left parapharyngealspacesynovial sarcoma in a 64-year-old ladywith comprehensive review of literature. Case Summary: A 64-year old lady presented with progressive painless swelling on left side of neck associated with foreign body sensation since 2-months. Ultrasonographic Examination revealed well defined hypoechoic lesion 3.7x2.8 cm in submandibular region with few subcentimetric left sided level II lymph nodes. CEMRI Neck revealed an oval shaped altered signal intensity space occupying lesion in the left parapharyngeal and carotid space extending into the retropharyngeal space and crossing the midline. The lesion measured 3.1x 4.6x5.3 cm and had well defined smooth margins, appearing hypointense on T1 and hyperintense on T2W images. The lesionshowed moderate post contrast enhancement and displaced the neck vessels posterolaterallyand was suggestive of Benign tumor like Schwannoma. She underwent wide local excision and submandibular gland excision. Histopathologic examination revealed solitary nodular tumor 5 x 4 x 3.5 cm, plump cells displayed vescicular oval nuclei and scattered mitotic figures. Stromal cells possessed spindle shaped nuclei with indistinct cellular boundaries, consistent with biphasic synovial sarcoma FNCLCC Grade II. On IHC plump cells expressed CK, EMA, bcl-2, Vimentinand CD99 with Ki67 labelling index 7%. Subsequently she underwent postoperative radiotherapy using Image Guided Radiotherapy with Rapid Arc on Novalis-Tx Linearaccelerator. A total dose of 70 Gy was administered to high risk and 56 Gy to low risk region, in 35 fractions. Results: Patient is on regular follow-up incorporating three monthly clinical examination and six monthly imaging. Post treatment PET-CT done after six months showed non-enhancing and non-FDG soft tissue thickening along left parapharyngeal space abutting and partially encasing ipsilateral External Carotid artery. Follow-up CECTdone after one and two years of treatment showed no definitive enhancing focal mass lesion. She continues to be on follow-up and is disease free two years post treatment. Brief Review of Literature: Vanacheswaran et al, John Mallen et al, French Sarcoma group reported cases of head and neck synovial sarcoma and concluded that well planned wide surgical excision with adjuvant radiotherapy remains cornerstone of treatment. Conclusion: Very few reports of primary parapharyngeal synovial sarcoma have been published. They have aggressive nature with poor prognosis. Anatomical limitations preclude margin negative surgery, hence a multimodal approach with a focus on wide local surgical resection and radiation treatment currently seems most effective to control local disease. With the historically high rates of late onset recurrence and metastasis, regular long term follow-up and surveillance is recommended to improve survival.


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Case report on primary plasmacytoma of lymph node

Daliya Thomas, Ciniraj R, Sneha John, Anjali Murali

Department of Radiation Oncology, MCH, Trivandrum, Kerala, India

Introduction: Extramedullary plasmacytoma is a rare form of plasma cell dyscrasia, and represents 1.6~4% of all plasma cell tumors.[1] Primary lymph node plasmacytoma is quite rare, and represents 2% of all extramedullary plasmacytomas, and only 0.08% of all plasma cell malignant neoplasms neoplasms.[1]Methodology: 56 yr old man presented to the department of surgery with complaint of varicose veins in right lower limb. on examination he was accidently found to have a single enlarged lymph node in right inguinal region. On physical examination no other palpable lymph nodes in any other area were noted. Routine blood investigations were within normal limits. He underwent excision of lymph node. Upon macroscopic examination showed greish white nodular tiisue measuring 2×1×1.5 cm. Cut section revealed grey white with foci of grey brown areas. Microscopic picture revealed reactive changes along with increased plasma cells proliferation, the cells are positive for kappa and lambda and IHC showed positivity for CD 138. Patient was further evaluated in our department. A full work-up was conducted in order to ascertain whether there was any other evidence of disease. Complete blood count, liver function test including serum albumin, S cacium [8.9 mg/dl], ESR[11], LDH[229], β2-microglobulin (1.6 mg/L) were found to be normal, and no osteolytic lesions were noted on the skeletal survey. Serum Electrophoresis was within normal limits. Bone marrow aspiration showed trilineage haematopoiesis with mild erytthroid hyperplasia and plasma cell <5%. Serum immunoglobulin quantitative assay and free light assay were within normal limits. As there was no evidence of multiple myeloma it was decided to keep patient on follow up. Patient is adviced for review in OPD monthly for first 6 months with CBC, RFT, S. calcium, ESR and to consider immunological test like Serum electrphoresis/IF if any suspicion or else after 6 months. Patient is on regular follow up in our department. Discussion: Most patients with plasma cell neoplasm exhibit a generalized disease upon diagnosis. However, a minority (<5%) of these present either with a single bone lesion and less commonly with extramedullary plasmacytoma.[2] Primary plasmacytoma of the lymph nodes is quite rare. In order to diagnose primary plasmacytoma of the lymph nodes, there must be no evidence of plasma cell proliferation elsewhere. The case reported here was a patient who exhibited a inguinal lymh node mass which revealed histologically plasma cells in the lymph nodes. However bone marrow aspiration and and skeletal survey and immunological tests revealed no evidence of plasma cell proliferation. Conclusion: We present this case of extramedullary plasmacytoma of lymph node due to rarety of its presentation.

References

  1. Woodruff RK, Whittle JM, Malpas JS. Solitary plasmacytoma. I: Extramedullary soft tissue plasmacytoma. Cancer 1979;43:2340-3.
  2. Soutar R, Lucraft H, Jackson G, Reece A, Bird J, Low E, et al. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 2004;124:717-26.
Figure 1

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Figure 2: (a) Preradiotherapy, postbiosy images on PET CT performed in March 2018. (b) Post radiotherapy images from PET CT performed in july, 2018 showing no enhansing lesion, suggestive of complete response

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Epithelloid haemangioendothelioma of maxillary sinus: A rare case report

Riddhijyoti Talukdar, Enam Murshid Khan, Tanweer Shahid, Punit Sharma, Shantanu Panja, Mukti Mukherjee, Jibak Bhattacharya, Pavitra Vijayaraghavan, Chandrika Saikia

Introduction: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with an epithelioid and histiocytoid appearance, originating from vascular endothelial or pre-endothelial cells. It represents less than 1% of all vascular tumorsand was described for the first time in 1975 by Dail and Liebow as pulmonary EHE (P-EHE). Due to its rarity, diagnosing and treating a case of EHE can be challenging. Case Report: In February, 2018, a 62 years old gentleman presented withleft sided facial pain with swelling and bleeding from left nostrilfor2 months. MRI of PNS showed a 4.4 x 5.1 x 3.7 cm mass in left maxillary sinus, extending into left masticator space, abutting left lateral pterygoid, temporalis, masseter muscle and left nasal cavity, eroding floor of left orbit. Biopsy came as Malignant neoplasm, features suggestive of poorly differentiated carcinoma. He underwent left medial maxillectomy and excisionof left maxillary tumor on 22nd February 2018. IHCwasnegative for Chromogranin, Cytokeratin, MELAN-1, S-100, and Synaptophysin and positive for EMA, CD-31, CD-34 and factor VIII, and histological features were suggestive ofEpithelloidHaemangioendothelioma. Patient was then referred for Radiotherapy. Post-operative PET CT done on 13th March 2018 showed FDG avid enhancing 48 x 29 x 34 mm residual lesion (SUV max 3.9) in the left maxillary antrum, eroding lateral maxillary wall, extending into retromaxillary space, left orbit and pterygopalatine fissure. Received adjuvant radiotherapy to a dose of 66 Gy/33 fractions (2 Gy per fraction) to the high risk volume and 60 Gy/33fractions (1.81Gyper fraction to intermediate risk volume, by IMRT with SIB), completed on 25th April, 2018. Post treatment PET CT done in July, 2018, showed complete metabolic response (CMR). Background and Discussion: EHE is a rare vascular tumor thataffects lung, liver and bones, although it has been reported to be arising from almost any part of the body. Only a few reports have appeared describing EHE in the head and neck. It was formerly regarded as having intermediate malignancy, however WHO has recently re-classified EHE as a fully malignant tumor. Avadhani et al reported a case of maxillary sinus EHE in 2015 which was only the second reported case of EHE arising in a paranasal sinus. The etiology of EHE is unknown. Chronic Bartonella infection may have a causal relationship. IHC commonlyshows positivity for VIII–related antigen, CD31 and CD34and occasionally express cytokeratin. Because of its rarity EHE has no standard treatment. Surgery is the main treatment. RT after surgical resection is given if there is residual disease, irrespective of the site of tumor. Chemotherapy is preferred in case of widespread disease. In this case, complete excision of the lesion was not posssible. However radiotherapy proved to quite effective in eradicating the macroscopic enhancing and FDG avid lesion completely. A longer follow up is however required to report the effectiveness of radiotherapy in inoperable Epithelloid Haemangiendothelioma.


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NUT midline carcinoma of nasal cavity: A case report

S. L. Aryakrishna, R. Rejnish Kumar, Anitha Mathews, G. Renuka

Department of Radiation Oncology, Pathology and Imageology, Regional Cancer Centre, Trivandrum, Kerala, India

Introduction: NUT Midline Carcinoma (NMC) is considered as the clinicallyaggressive type of squamous cell carcinoma and majority of the patient will succumb to rapid disease progression. The actual NMC incidence is unclear and is almost certainly under diagnosed. It is characterised by undifferentiated morphological features, immunoreactive to NUT and defined by NUT rearrangement. High clinical suspicion and diagnosis of this rare entity is essential in a midline undifferentiated carcinoma. Case Report: 36 Year old lady was evaluated for nasal obstruction, epistaxis and anosmia of one month duration. At presentation here, she had polypoidal mass protruding from the right nasal cavity with active bleeding, widening of nasal bridge, fullness in the right nasolabialfold, proptosis of right eye and restrictedelevation of right eyeand anosmia. She was evaluated with an MRI head and Neck which showed a lobulated mass in the rightnasal cavity expanding it involving the cribriform plate of ethmoid andadjacent frontal bone and extending into the olfactory fossa and basi frontal region, intoorbit with displacement of extra ocular muscles. Histopathological examination showed fragments of cellular neoplasmcomposed of cells arranged diffusely and in perivascularpattern having brisk mitotic activity (MIB 75-90 %). IHC showed positivity with cytokeratin, EMA, CD 138 and negative for LCA, MUM 1, CD56, CD5, CD20, CD30, CD31, Myogenin, suggestive of high grade malignant neoplasm-undifferentiated carcinoma. NUT immunostaining was performed which showed speckled nuclear positivity and diagnosis of midline NUT carcinoma was obtained. As surgical resection was not feasible, she was initiated on platinum based chemotherapyShe received first cycle of chemotherapy withCisplatin and 5 Flurouracil. She improved symptomatically, there was also visible reduction in the protruding nasal mass, proptosis of right eye and was discharged in a stable condition. She got admitted in another centre after 10th day of chemotherapy with grade IV neutropenia and she succumbed to death. Discussion: NUT midline carcinoma (NMC) is a rare and aggressive genetically defined subtype of squamous cell carcinoma characterized by chromosomal rearrangements of the gene NUT, at 15q14. NMC can equally affect individuals at any age. The majority of cases occur in the midline of the body, including the head, neck, and the mediastinum. Most common symptoms are local mass and pain caused by infiltrating tumor cells. NMC is morphologically categorized as a variant of squamous cell. Recently, a highly sensitive and specific monoclonal immunohistochemical test for NUT (C52 monoclonal antibody) was introduced, which greatly simplified the diagnosis. No specific treatment protocol exists for NMC. Multimodality approaches using surgery, radiotherapy, chemotherapywere tried with limited clinical benefits. Recently targeted agents like histone deacetylase inhibitors and BET inhibitors have shown promising results. Conclusion: Midline NUT carcinoma is a rare, genetically defined and aggressive neoplasm. It should be considered as a differential in midline poorly differentiated carcinoma Survival is very poor even with aggressive multimodality approach.


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Malignant renal epithelioid angiomyolipoma after nephrectomy: A case report and review of the literature

Sheena Joy, Jenny Joseph, Judith Aaron, Jose Tom, Johny K. Joseph, Unni S. Pillai

Aims: The primary objective of this study is to highlight the evidence available for diagnosis, treatment and outcome of this rare tumor based on literature review and to describe a case of a patient who was treated with everolimus for renal epithelioid angiomyolipoma (REAML) with local recurrence. Methods: A pubmed search for malignant REAML was performed for case reports and retrospectively analysed the clinical course of a patient who developed local recurrence following nephrectomy for REAML. Results: The pubmed search yielded 20 results for analysis in which 5 studies included treatment with everolimus. Renal epithelioid angiomyolipoma (EAML) is a rare tumor comprising only 1% of renal angiomyolipoma (AML) and is potentially malignant. It belongs to the family of PEComas and share a common immunophenotypic profile of muscle and melanocytic differentiation and is negative for epithelial markers. Malignant potential is more with tumors more than 7cm, with necrosis, carcinoma like growth pattern, extrarenal extension or renal vein involvement, and is associated with tuberous sclerosis. The size of tumor correlates with the risk of hemorrhage. These tumors are characteristically driven by deleterious mutations in the tumor suppressors TSC1or TSC2, whose gene products typically act to inhibit mTOR, a critical regulator of cell growth, thus forming the basis of use of everolimus in malignant EAML. It results in a decrease in the size and volume of AML and thereby may also reduce the risk of bleeding. Median time for response was seen around 3 months and cessation of treatment was found to cause progression of disease. Hence patients should be kept on mTOR inhibitors for life. However most malignant EAMLs acquire resistance after few months and they progress. Hence role of drugs like VEGF inhibitors or anti PD-1 immune checkpoint inhibitors are being investigated. We present here a case of 70 year old man diagnosed with malignant EAML in September 2014. He underwent left radical nephrectomy. It had characteristic pathological features of REAML (HMB45/Vimentin-positive, EMA/CK-negative). In July 2016 (20 months later) he presented with multiple peritoneal and retroperitoneal recurrence (largest being 12 x 8 cm). Chemotherapy with single agent Inj. Adriamycin was started and he had an episode of intrabdominal hemorrhage which needed critical care. Since there was tumour regression, two more cycles were given. Evaluation post three cycles of chemotherapy showed disease progression. Therefore, it was planned to change to Tab. Everolimus 10 mg once daily. He had good clinical response but developed stomatitis and hence dose was reduced to 5 mg once daily. He has been on the same till date with no serious adverse event. However, he needed multiple transfusions for anemia. Conclusion: Malignant EAML is a rare entity with minimal treatment options. A progression free survival of around 2 years was reported with mTOR inhibitor, Everolimus with relatively good safety profile.


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Intracranial rosai-dorfman disease: A case report

S. Zuzaki, K. R. Rajeev, Anitha Mathews1, H. V. Easwer2

Division of Radiation Oncology, Regional Cancer Centre,1 Division of Pathology, Regional Cancer Centre,2 Department of Neurosurgery, SreeChithraTirunal Institute of Higher Medical Sciences Trivandrum, Kerala

Background: Rosai-Dorfman Disease (RDD) is a rare, benign histiocytic proliferative disorder of unknown etiology, characterized by painless massive bilateral cervical lymphadenopathy, fever and leukocytosis. Extranodal involvement has been reported in various sites, but intracranial involvement, particularly without nodal disease is extremely rare. Though the disease mainly affects children and young adults, intracranial RDD presents in older age with a male predilection. Case Report: Forty Five year old gentleman evaluated for loss of consciousness and focal seizures of six months duration. MRI brain showed an extra-axial dural based lesion in the right parietal region extending into the leptomeninges, causing bone erosion and marrow infiltration of the adjacent parietal bone. The radiological diagnosis was meningioma and patient underwent right tempero parietal craniotomy and excision of the tumor on 2nd July 2018. Histopathology reported as Rosai-Dorfman Disease of right parietal region. Post op MRI showed residual disease along the surgical bed which was extending to dural sinuses. In view of residual disease patient currently undergoing adjuvant radiation. Discussion: Most of the intracranial RDD is benign. It usually occurs in children and young adults and the mean age of onset is 20 yearswith a male predilection. Extranodal RDD can occur in 43% of cases and only less than 5% of cases occur in central nervous system with or without nodal involvement. Radiologically, intracranial RDD can be mistaken as meningioma. Histopatholgy and IHC provide definitive diagnosis. Since this condition is very rare, its clinical course and treatment are not well established and prognosis varies depending on the involved site and treatment. Surgical resection is the primary treatment of choice and is curative. In some cases, subtotal resection followed by radiation has produced excellent results.


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Squamous cell carcinoma in pilonidal sinus

P. Krishna Priya, A. Sajeed, A. Priya, K. R. Anila

Department of Radiation Oncology, Imageology and Pathology, Regional Cancer Centre, Trivandrum, Kerala, India

Squamous cell carcinoma arising in longstanding pilonidal sinus is an extremely rare complication. It is postulated that chronic and recurrent inflammation leads to malignant transformation in about 0.1% of cases. The standard of treatment is en bloc resection with tumour free margins. Early local recurrence rate is high. Adjuvant chemotherapy and radiation is proposed to reduce the rate of local recurrence. We report a case of squamous cell carcinoma arising in a longstanding pilonidal sinus in a 69 years old lady. She underwent an unplanned excision for recurrent pilonidal sinus disease. Diagnosis of squamous cell carcinoma was made on histopathological examination. Radiological evaluation showed lesion infiltrating to gluteus maximus, lower sacrum and coccyx for which a re-wide excision was done. Within a periodof four months, she developed local recurrence with gluteal deposit and metastasis to bilateral inguinal nodes. The patient was treated with four cycles of chemotherapy with cisplatin and 5-fluorouracil and local radiotherapy to the pelvic and inguinal nodes and the gluteal deposit. Disease progressed even after completion of radiotherapy for which best supportive care was considered but she expired within one month. Symptomatic cases of chronic pilonidal sinus disease require radical excision of the sinus tract and the specimen must be subjected to histopathological evaluation to rule out the rare possibility of malignancy. Adjuvant radiotherapy after wide excision with tumour-free margins can reduce recurrence rates.


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False positive PET scan in Breast cancer –report of two cases

A. Anju Farsana, K. R. Rajeev, Sumod Mathew Koshy

Department of Radiation Oncology and Imageology, Regional Cancer Centre, Trivandrum, Kerala, India

Background: Positron emission tomography (PET) with18 F fluoro-deoxyglucose (FDG) is now an established functional imaging modality predominantly used in several neoplastic diseases including breast cancer. As compared to imaging with just CT or PET alone, PET/CT has been shown to be beneficial in altering the management of patients. Standardized Uptake Value (SUV) is the most commonly used semi-quantitative parameter utilized for analyzing FDG-PET images in routine clinical practice. However there is significant overlap in SUV between malignant and certain inflammatory lesions. Therefor care must be taken while interpreting PET CT findings in routine clinical practice. We present two false positive results of PET CT scans in patients with breast cancer. Case 1: Thirty six year old female, treated for papillary carcinoma thyroid in 2013. She presented with FNAC proven carcinoma of the right breast in March 2016. She underwent MRM and in view of early stage low risk disease, was on adjuvant endocrine therapy with tamoxifen. After a disease free interval of 5 years she presented with isolated contralateral axillary nodal metastasis detected in CT scan. PET CT revealed an uptake in axillary and sub pectoral nodes which was suggestive of metastasis. Upon biopsy the final diagnosis turned to be toxoplasmosis. Case 2: Forty five year old female underwent BCS in November 2014 for malignant phylloides tumor. In April 2015 she underwent MRM for ipsilateral breast tumor relapse. Ten months later she presented with local recurrence for which she underwent wide excision followed by radiation to the chest wall. After a disease free interval of two and half year she presented with fever and cough for which CT done outside showed liver lesion and cavitory lung lesion. PET CT showed multiple lesions in liver, bone and lung with varying SUV ranging from 3-8. Her sputum AFB was positive for tuberculosis and guided biopsy from lung lesion reported as necrosis. Discussion: PET/CT is a common imaging modality used in the evaluation of oncology patients. While being extremely sensitive to identifying sites of malignancy F18FDG is very non-specific. False positive results are commonly observed in areas of active inflammation or infection with a reported false positive rate of 13%. These areas of infection/inflammation will demonstrate intense uptake of FDG and unless correlated with the patient's clinical history and pathologic biopsy will result in an incorrect diagnosis. Due to the limited specificity of FDG-PET/CT in excluding malignancy, FDG positive lymph nodes require histologic assessment where ever feasible according to the clinical scenario for accurate diagnosis and management.


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Extra osseous Ewings sarcoma of oral cavity

Ganesh Kumar Nair, Aravindh S. Anand, Abul Hussain, P. Gopika

Department of Radiotherapy, Government Medical College, Trivandrum, Kerala, India

Introduction: Extraosseous Ewings sarcoma (EES) belongs to the Ewings sarcoma family of tumors with unique t(11;22)(q24;q12) translocation and expression of EWS-FLI1 protein. EES of head and neck is extremely rare.[1]Methodology: 15 year old boy presented to the oncology OPD of Trivandrum medical college with complaints of swelling in left side of the floor of mouth for about one year duration. Clinical examination revealed a 5x4 cm nontender firm swelling in the floor of mouth towards left side with ankyloglossia. He was evaluated with MRI of oral cavity which revealed a well defined soft tissue mass lesion, 4.2X3.2X4.8cm arising between genioglossus muscles, from lingual septum without mandibular involvement. Biopsy reported malignant round cell neoplasm. Immunohistochemistry showed diffuse strong membrane positivity for CD99(MIC2) with negative LCA, desmin and synaptophysin. The patient was started with Vincristine, Adriamycin and Cyclophosphamide alternating with Ifosfamide and Etoposide three weekly. He was reassessed with PET-CT after twelve weeks which showed no active disease. The chemotherapy was continued till forty weeks, after which an MRI scan was donewhich revealed complete response. The patient is on regular followup with a DFS of two years. Discussion: Presentation of Extraosseus Ewings Sarcoma in oral cavity is unusual. Among the oral cavity presentation, mandible is the most frequent location. In this case, the primary site is floor of the mouth and while going through the literature, there is no case reported with a similar presentation. Hence this case may be the first case to be reported. The published survival data on extraosseus ewings sarcoma vary considerably, though it is assured that tumours in head and neck region have better prognosis than other locations. Conclusion: The extreme rarity of tumor and complete clinical and radiological remission with systemic chemotherapy alone make this case unique. It seems necessary to be aware of this rare tumor at this uncommon site as early diagnosis and treatment can result in best chance of survival.

References

  1. Vikas Prasad B, Ahmed Mujib BR, Bastian TS, David Tauro P. Ewing's sarcoma of the maxilla. Indian J Dent Res 2008;19:66-9.



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Waldenstroms macroglobulinemia presenting as Hyperviscosity Syndrome

Ganesh Kumar Nair, Aravindh S. Anand, P. Gopika

Department of Radiotherapy, Government Medical College, Trivandrum, Kerala, India

Introduction: Waldenstroms Macroglobulinemia is a rare B cell neoplasm characterised by IgM monoclonal gammopathy and infiltration of bone marrow with malignant lymphoplasmacytic cells. It comprises about 1% of all cancers involving the lymphocytes.[1]Methodology: 66 year old gentleman presented to the Oncology OPD of MCH Trivandrum with complaints of visual disturbances, fatigue, head ache and facial puffiness. On physical examination, the patient had pallor and bilateral pitting pedal edema. We did further blood investigations which revealed anemia(Hb-8 gm%), ESR-150 mm/hr, B. Urea-30 mg/dl, S. Creatinine-0.8mg/dl, S. Ca2+: 8 mg/dl, β2 microglobulin- 8.27 mg/L, skeletal survey-no lytic lesions. Serum electrophoresis showing monoclonal gammopathy (M spike) seen in gamma globulin region, IFEP detected IgM band and kappa band and IgM quantitative assay- 514 mg/dl. A bone marrow examination was performed, which showed few plasma cells and CD 20, CD 38 and CD 138 positivity. A dilated fundus examination revealed features suggestive of hyperviscosity syndrome. Hence, a diagnosis of waldenstroms macroglobulinemia was made and the patient was started on weekly Bortezomib and Dexona combination chemotherapy and completed 68 cycles. The patient was evaluated regularly with serum IgM assay and fundus examination which were within normal limits. Discussion: Presentation of Waldenstroms macroglobulinemia as hyperviscosity syndrome is very rare as per the literature. Eventhough the treatment for hyperviscosity syndrome is mainly plasmapheresis, in our patient the signs and symptoms were reversible even without it. Management of hyperviscosity syndrome in Waldenstroms macroglobulinemia with proteasome inhibitor and dexamethasone without plasmapheresis is a good treatment option. But this being a case study more number of patients in a well designed prospective therapeutic trial can only address the scenario scientifically. Conclusion: Waldenstroms macroglobulinemia is usually a diagnosis made by exclusion of other plasma cell and haematological disorders. The chance for hyperviscosity in this set of patients is high in view of hypersecretion of IgM immunoglobulin. Hence it is always better to have a proper ophthalmologic evaluation once the diagnosis is made which will rule out hyperviscosity syndrome.

References

  1. Turgeon ML. Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001. Clinical Hematology: Theory and Procedures. Hagerstown, MD: Lippincott Williams & Wilkins; 2005. p. 283.



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Metastatic breast cancer, unusual sites of metastases to the tip of nose and the choroid of eye: Prognostic significance and progression-free survival

Karan Sood, Kislay Dimri, A. K. Pandey

Government Medical College and Hospital, Chandigarh, India

Aims: Metastatic breast cancer (MBC) is unlikely to be cured; meaningful improvements in survival have been seen, coincident with the introduction of newer systemic therapies. Patients should be selected based on selective prognostic markers to help in the identification of high risk for disease recurrence. We are presenting two cases of MBC: one patient with metastases to the tip of the nose and a second case presenting to the choroid of the eye to highlight the role of site of metastases as a prognostic indicator and progression-free survival in carcinoma breast. Discussion: MBC can involve any organ and the range of treatment options available frequently make its management challenging. The goal of any treatment is disease control, palliation of symptoms, and maintenance of the highest quality of life. About one-third of patients with MBC present with a local or regional recurrence involving lymph nodes and/or the chest wall. Over time, 75% of these patients develop metastases to other organs i.e. bone 40–75%, lung, 5–15%, pleura 5–15%, liver 3–10%, brain < 5%. The most useful prognostic factors are clinically based, namely: relapse-free interval, metastases involving the chest wall, bones, or lymph nodes. Patients with hormone receptor positivity generally have a more favorable prognosis. Other adverse prognostic features include weight loss, poor performance status, and elevated serum lactic dehydrogenase. Results: Both the patients were defaulters in their management of carcinoma breast. There was a mean time of 6.5 months between the 1st dose of AC and MRM. The mean time between MRM and Local RT was 3 months. The mean time for the patients to develop metastases to the unusual site post diagnosis and last dose of RT was 21 months and 10 months respectively. Conclusions: This calls for more strict patient compliance and better prognostication at the time of diagnosis to anticipate shorter progression-free survival. The role of node positivity, grade, DCIS component, site of metastases, the time between chemotherapy/surgery and radiation, time for follow-up post-treatment must be addressed in future. Hence these metastases to unusual sites namely tip of nose and choroid of eye tend to have a poor prognostic significance and indicate shorter progression-free survival in carcinoma breast.


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Rhabdomyosarcoma of maxilla: A case report

P. Gopika, Aravindh S. Anand, Teena Rajan

Department of Radiotherapy, Government Medical College, Trivandrum, Kerala, India

Introduction: Rhabdomyosarcoma is a malignant neoplasm of skeletal muscle origin. It is the most common soft tissue sarcoma seen in childhood and adolescence. It accounts for 6% of all malignancies in children under 15 years of age.[1] The most frequently affected areas are head and neck region, genitourinary tract, retroperitoneum and to a lesser extent the extremities.[2]Methodology: 16 years old girl presented to the Oncology OPD with complaints of bulging of left eye, diplopia and multiple episodes of watering from the eye. On clinical examination, she had proptosis of left eye and periorbital edema. She was evaluated with CT scan of orbits and paranasal sinuses which revealed a space occupying lesion in the maxillary sinus with local extension. A biopsy was taken from the maxillary sinus, which showed malignant round blue cell tumor compatible with Rhabdomyosarcoma(alveolar type). IHC showed Desmin positivity and NSE, CD99, LCA, chromogranin and synaptophysin negative. PET CT was done which showed high grade FDG uptake in left maxillary sinus with local extension. Bone marrow examination had no abnormal infiltrate and dilated fundus examination was within normal limits. The patient was taken up for chemotherapy with Vincristine, Adriamycin and Cyclophosphamide alternating with Ifosfamide and Etoposide regimen for 8 cycles followed by Image Guided Radiotherapy of 55.8 Gy in 31 fractions. Patient was kept on follow up. She was reevaluated with PET-CT after a follow up period of six months and it showed mild metabolically active thickening in left maxillary sinus. Now the patient is on regular followup with a DFS of 11 months. Discussion: Head and neck Rhabdomyosarcoma occurs frequently in children and adolescents, but paranasal sinus primary is very rare. Alveolar Rhabdomyosarcoma exhibits an aggressive biological behaviour and the response to chemotherapy is high. In this patient, at the time of presentation, she had a florid picture and immediately after the systemic chemotherapy she showed dramatic response. This response was consolidated with local radiation treatment. After a followup period of two years, the patient is disease free. Conclusion: Rhabdomyosarcoma being both a chemo and radiosensitive tumour, even in a locally advanced scenario the patient should be considered with a radical intent.

References

  1. Stuart A, Radhakrishnan J. Rhabdomyosarcoma. Indian J Pediatr 2004;71:331-7.
  2. Duraes GV, Jham BC, Mesquita AT, dos Santos CR, Miranda JL. Oral embryonal rhabdomyosarcoma in a child: A case report with immunohistochemical analysis. Oral Oncol Extra 2006;42:105-8.



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A rare case of nut midline carcinoma of maxilla

Sai Kumari, Sanjay Mishra, Channabasappa Kori

Aims and Objective: Since NUT Midline Carcinomas is an uncommon diagnosis, the purpose of presenting this report was to create more awareness about this rare, aggressive, rapidly progressive and most fatal variety of Carcinoma. Materials and Methods: 18 year old boy noticed drooling of blood stained secretion from his right nostril which was associated with local pain and cheek swelling in January 2016. On examination by a Dental Surgeon a growth was noticed in right upper gingiva. Biopsy reveals an undifferentiated malignant tumor with epitheliod morphology. The tumour cells expressed cytokeratin(focal) and NUT and were immuononegetive for EMA, p63, MUM-1, HMB-45, S100 protien, desmin, synaptophysin and MIC-2. In situ hybridisation for EBER is negetive. Nuclear INI-1 expression is intact. Impression was Right maxillary mass- NUT midline carcinoma. From 9-03-2016 to 20-04-2016 Definitive 66gy/30 # IMRT was delivered. Repeat PETCT reveled residual disease and he underwent Near total maxillectomy+Right SOND+right level Ia+buccal pad fat +orbital bone +posterior margin +superior bony margin+nasal mucosa+inferior margin +nasal margin+nasal bone +palatal bone+sinus mucosa in June 2016. In July he developed bilateral lower limb weakness and PET CT reveled Multiple bony metastasis along with lung and liver metastasis. He completed Palliative Radiotherapy to involved vertebra which was causing impending spinal cord compression in August 2016 and expired on 9 September 2016. Results: The index case mimicked typical characteristics of NMC that it was very aggressive, responded very well to Radiotherapy and rapidly lethal. Patient died within an year of diagnosis. Conclusion: The challenge is not the diagnosis of NMC, but when to perform the immunohistochemical test for NUT expression. It should be considered as differential diagnosis of any midline poorly differentiated carcinoma in a never-smoking patient, and in the absence of EBV or HPV within the tumor.


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Post cricoid and upper oesophagus malignancy with calcaneum metastases: Rare metastatic entity at rare site

ChinmayKumar Prajapati, Pooja Nandwani Patel

Introduction: Metastatic disease of the skeleton occurs in at least 20 to 30% of patients with a malignancy, but only a small fraction of these patients will have acrometastasis-metastasis to the hands or feet. Bone metastasis from post cricoid malignancy is a rare entity and infact metastases to the bone distal to elbow and knee especially to the calcaneum are even rarer. Here we report a case of post cricoid malignancy with calcaneal metastasis and review of literature. Materials and Methods: A 65 year old female presented at a private hospital with complain of difficulty in swallowing solid food for 2 months. On workup she was diagnosed as locally advanced metastatic carcinoma of post cricoid and upper oesophagus with mediastinal lymphadenopathy and metastases in bilateral lung fields. She was given six cycles of chemotherapy with combination of paclitaxel and carboplatin with good response at both primary and metastatic site but later she developed brain metastases for which she was treated with 30 Gray/10# (fractions) palliative whole brain radiotherapy. After one month, she developed severe pain over right heel. On examination, tenderness was present at right heel and Magnetic Resonance Imaging (MRI) of right foot showed well defined soft tissue intensity lesion on postero-inferior aspect of calcaneum. The histopathology showed nests of poorly differentiated squamous cell carcinoma. Palliative radiation of 30 Gy/10 # was given to the painful site. Results: Patient has good symptomatic relief after completion of radiotherapy. Patient is now called after a month for complete clinical and radiological assessment. We plan to do clinical examination and MRI to see the response with two weeks of palliative RT given. Conclusion: Osseous metastases are rarely seen arising from post cricoid that too in bones of foot like calcaneum. Palliative treatment in the form of radiation to the painful site can provide good symptomatic relief. However this presentation can be very slow going and usually patients die because of other non-osseous spread or local spread.


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Squamous cell carcinoma of Stomach –a rare case

D. Samuel Sandeep, C. Sanjeeva Kumari

Squamous cell carcinoma (SCC) of the stomach is a rare entity. There are several theories regarding the development of this tumor, but its pathogenesis remains obscure. Fewer than 100 cases of primary SCC of the stomach have been published in the literature. Due to advanced stage at the time of diagnosis in most of these cases, the prognosis is generally poor. In the case presented here, endoscopy revealed a tumor in the stomach described as SCC by biopsy and IHC. Following curative surgery, adjuvant chemotherapy, chemoradiotherapy followed by 1 more cycle of adjuvant chemotherapy was administered (ARTIST 1 trial) The young patient(26 year old) survives more than a year without a recurrence, after successful treatment.


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Sinonasal undifferentiated carcinoma: A rare case report

Salini, Lalitha Subramanian, U. Insuvai

Introduction: Sinonasalundifferentiated carcinoma (SNUC) is an aggressive neoplasm arising within the nasal cavity or paranasal sinuses. SNUC is a rare malignancy comprising of 3% of all head and neck malignancies with a median survival of 12 months to 40.5 months. It has a male preponderance. Given the undifferentiated nature of this malignancy, immunohistochemical analysis is extremely useful. Overall prognosis remains poor inspite of combined treatment modalities including surgery, chemotherapy and radiation. Case History: 40 years old female presented with complaints of persistent nasal obstruction for 2 months duration and mass protruding through left nasal cavity for 1 week duration. Patient had no history of headache, diplopia and epistaxis. Patient had no history of exposure to known carcinogens. On examination a growth was seen within the left nasal cavity with a bulge seen externally. No neck nodes were palpable. MRI paranasal sinuses showed a mass lesion of size 4.8 x2.2 cm arising from lateral wall of nasal cavity with its epicenter at middle turbinate and bony resorption of left maxillary sinus. Biopsy from nasal cavity mass showed features suggestive of neuroblastoma. Immunohistochemistry was done which showed features of sino-nasal undifferentiated carcinoma (S-100-Negative, Neuron specific enolase-positive in 25% of cells, Synaptophysin-focal weak positive, Pan cytokeratin-positive in 60% of cells). Patient underwent endoscopic removal of mass followed by chemoradiotherapy. Patient received 66 Gy to nasal cavity and is on follow up now. Conclusion: Immunohistochemistry is essential for differentiating SNUC from other types of non-epithelial tumours. Aggressive nature of the tumour warrants combined modality of treatment.


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Hemangiopericytoma of brain: A rare case report

Luri Borah, A. K. Kalita, M. Bhattacharyya, Partha P. Medhi

Aims and Objectives: To find out the role of Adjuvant radiotherapy after surgical resection in a case of Hemangiopericytoma. Materials and Methods: Hemangiopericytoma is a rare mesenchymal soft tissue tumourbut can also be found in viscera, bone, orbit, paranasal sinus, CNS, retroperitoneum, colon, thorax, and extremities Though surgery is the main stay of the treatment but postoperative radiation has shown good overall survival (OS) and recurrence free survival (RFS). A 36 year old male presented elsewhere with complaints of vertigo, vomiting, generalized weakness but no history of convulsion; where he underwent craniotomy and removal of the intracranial SOL after his CECT Brain showed large circumscribed lobulated mixed intensity lesion in right temporo-parietal region (6.7x5.3X5.5 cm) containing large cystic/necrotic component with thick irregular margins and solid component with marked heterogeneous ring enhancement and midline shift. Histopathology confirmed grade III astrocytoma. After 2 months, patient presented in another hospital with complains of visual blurring and headache over the parietal area. Repeat MRI Brain which showed a large recurrent/residual heterogenous enhancingSOL in the right temporal lobe with internal cystic and hemorrhagic changes causing mass effect on adjacent structures and midline shift. Patient was planned for re-surgery and Right parietal redo craniotomy and gross total excision was performed. Histopathologyreported anaplastichemangiopericytomaWHO grade III. Immunohistochemistry was positive for CD34 and vimentin but negative for synaptophysin, GFAP, EMA. Post-op MRI Brain showed a well defined (1.3X 1 cms) lesion noted in base of the middle cranial fossa with homogenous contrast enhancement. Post op site showed CSF collection. During hospital stay post-operatively he developed wound leakage and was managed symptomatically. Patient was then referred to our hospital for adjuvant treatment where he presented with wound leakage only. Due to two different HPE reports and dilemma of starting the adjuvant treatment in view of wound leakage the case was taken for discussion in disease site specific meeting where the second report was agreed upon and started on adjuvant treatment after a neurosurgery consultation. Since hemangiopericytoma is known for early distant metastasis so full metastatic workup was done which came normal. Patient is now on conformal radiotherapy treatment in our hospital. Results: Hemangiopericytoma is a rare tumor and no single institution has had a broad experience in treating it. Surgical resection remains the mainstay of treatment. There is little available information in the literature regarding the role of radiotherapy, which has been a point of debate. Some authors have previously regarded this to be a radioresistanttumor. In our case we found that adjuvant radiotherapy prevented recurrence of the tumor and alleviation of pain and other associated symptoms. Conclusion: we can say complete surgical resection is necessary for preventing tumour recurrence. Also adjuvant RT is equally recommended to prevent recurrence even in completely resected cases diagnosed as WHO grade II – III tumours.


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Metastatic paragangliomaof the neck in a young girl

Diptajit Paul, Anil Kumar Dhull, Vivek Kaushal

Department of Radiation Oncology, Regional Cancer Centre, Pt. B D Sharma PGIMS, Rohtak, Haryana, India.

Aims and Objective: Paragangliomas (PG) are rare tumors composed of chromaffin cells, arising from neural crest cells associated with autonomic ganglia along the sympathetic trunk, accounting for only about 0.3% of all neoplasm. Most PGs are defined as benign in nature, criteria for malignancy is mainly distant metastasis which is 10-20% of all PGs. Most common sites for metastasis are bone, lungs, liver and lymph nodes. The reported incidence of PGs located in the head and neck region is very less. We present an atypical case of aggressive type ofParaganglioma presenting in the neck region of a young girl. Materials and Methods: A 14-year young girl presented with history of painless swelling in left side of neck for four years which was insidious in onset and initially of the size of a small almond, gradually progressed to the size of a lemon. She also developed high grade fever, off and on for last one month, associated with rigor and chill and headache, and relieved by taking antipyretics. She had history of pain abdomen for one month which was insidious in onset, dull aching in nature, mild to moderate in intensity. She also had history of significant weight loss of more than 5Kg in last one month. On local examination, patient had multiple, hard, mobile, bilateral neck nodes maximum of size 6 × 5 cm. Right sided inguinal node of size 2 × 2 cm was also present. Results: The neoplastic cells illustrated wide spread positivity for neuron specific enolase and chromogranin immunocytochemical stain. The histopathological appearance and immunohistochemical profile of the biopsy tissue confirmed it to be Paraganglioma. CECT revealed heterogenous mass of size 3.7 × 2.2 cm in left upper aspect of neck which extends up to the submandibular region. Mildly enhancing lymph node of size 2 × 1 cm was seen lateral to this. Sub centimetric lymph nodes were seen in bilateral submandibular region. Multiple hypodensities were visualized in both lungs favouring lung metastasis. USG abdomen revealed multiple mesenteric lymph nodes in umbilical, para umbilical region and right iliac fossa, largest of size 2 cm. As patient initially was inoperable, 6-cycles of 3-weekly intravenous chemotherapy were givenwith CVD regimen (cyclophosphamide, vincristine and dacarbazine). Post treatment PET-CT revealed partial treatment response with left side neck node regression to size 2.6 × 1.5 cm and few sub centimetric sized lesions in upper lobe of bilateral lung. In view of partial response, patient was advised 2nd line chemotherapy but patient refused and accordingly, advised for ‘Drug Holiday’ and close clinical monitoring. After 6-months of regular follow-up patient is without any progression of disease. Conclusions: Paragangliomasare extremely rare before the age of 20 andhave a female predominance. Current studies have shown that a subset of patients diagnosed with metastatic PGs do not demonstrate favourable outcomes. Chemotherapy with CVD are most thoroughly evaluated regimen although symptomatic response is 40-50% in the literature. More studies should be tried to establish theeffective treatment modalities.


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Intracerebral malignant nerve sheath tumour-a rare case report and review of literature

Moumita Paul, M. Bhattacharyya, A. K. Kalita, P. P. Medhi, Gautam Sarma, Ghritashee Bora

Department of Radiation Oncology, Dr. Bhubaneswar Borooah Cancer Institute, Guwahati, Assam, India

Objective: Evaluation and review of literature in Intracerebral Malignant Nerve Sheath Tumour at our Institute. Materials and Methods: A 55 years old male, known case of systemic hypertension and diabetes mellitus presented with the symptoms of decreased hearing in left ear for 8 years, tinnitus for 2 years and imbalance while walking for last 5 months. MRI Brain was done which showed left CP angle space occupying lesion, causing compression over left cerebellum and brainstem with resulting hydrocephalus. Results: Patient underwent “Right (posterior parietal) ventriculo-peritoneal shunt” followed by Left retromastoid craniotomy and debulking of left CP angle space occupying lesion. HPE was reported as Spindle cell sarcoma suggestive of malignant peripheral nerve sheath tumour, high grade probably arising from pre-existing Schwannoma. Patient's higher mental functions, motor system, sensory system were all intact. Cranial nerve examination revealed mild UMN left facial weakness and left Rinne showed BC>AC while right Rinne showed AC>BC; Weber's test showed lateralisation to right ear. He received Adjuvant Radiotherapy (IMRT) to brain to a dose of 60Gy/30# and is on follow-up. Review of Literature: Malignant peripheral nerve sheath tumour is a relatively uncommon disease and intracerebral malignant nerve sheath tumour is even rare with an incidence of only 0.001%. It is highly aggressive with generally a poor prognosis. Intracerebral MPNST is presumed to arise from peripheral nerves which traverse the cerebral white matter, probably with blood vessels. It is known that Schwann cells accompany peripheral nerves in the dura and may penetrate the brain alongside the blood vessels they accompany. It is conceivable, therefore, that a Schwann cell tumour could originate from an intracerebral perivascular nerve. Conclusion: The treatment of choice is resection of the tumour followed by adjuvant radiotherapy although treatment is not standardized. Chemotherapy is used in patients with systemic recurrence. The addition of adjuvant therapies has improved survival, but even so, the 5-year survival rate is 52%, and the rate drops lower if good surgical margins cannot be achieved. For intracranial MPNST, the reported median overall survival is 9 months. Even with aggressive treatment regimens, the prognosis for intracranial nerve MPNSTs remains poor, being poorer than for MPNSTs in all other body sites.


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An unusual case of MALT lymphoma of base of tongue

B. M. Muhammed Ali Azher, Maitrik J Mehta, U. Suryanarayana

Aim and Objective: To report an unusual case of MALT Lymphoma of Base of Tongue. Introduction: It's a distinct type of non hodgkins lymphoma involving extralymphoidal lymph tissue. MALT lymphoma of head and neck is second most common after gastrointestinal tract; however, MALT lymphoma of base of tongue is an unusual entity and has only been reported in few cases. Materials and Methods: 50yr old female presented with dysphagia and nodular lesion in mouth. CT scan showed 20x24mm mass lesion involving base of tongue. Initial biopsy showed squamous mucosa with exuberant reactive lymphoid tissue and further immunohistochemistry consistent with MALT lymphoma (CD20+, CD2−, CD79a+, CD43+). PETCT revealed metabolically active lesion of 19x36mm in base of tongue (SUV max 7.2) and no evidence of active disease elsewhere in the body. Involved site radiotherapy is contemplated with IMRT modality upto 30.6 Gy. Results: Patient was advised for follow up after 4 weeks of treatment. We planned clinical evaluation and PET CT imaging. She is symptomatically better. Conclusion: There is no consensus on the treatment of MALT lymphoma especially on the tongue. To the best of our knowledge, ours is the seventh reported case.


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Galactomannan antigen as a marker of invasive aspergillosis in patients with malignancy: A case series

M. Mridula1, K. E. Vandana, Karthik Udupa1, Ananth Pai1, Kiran Chawla

Departments of Microbiology and1 Medical Oncology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India

Aims and Objectives: To assess the role of Galactomannan antigen (GMAg) detection as a marker of invasive Aspergillosis in patients with malignancy and to report the clinical course of patients who tested positive. Materials and Methods: Patients with malignancy suspected of invasive aspergillosis and testing positive for GMAg assay were identified from the laboratory records. The details of treatment, clinical response and re-testing for confirmation of diagnosis were collected retrospectively from the hospital records. The test details were collected and analysed with respect to various clinical parameters. Results: Between September 2017 to August 2018, nine out of ten patients with malignancy who were tested for GMAg showed positive result. The mean age was 45.5 years (range: 24-63 years), and 6 (54.5%) were women. Five (55,5%) had acute leukaemia; others had breast cancer (22.3%), osteosarcoma (11.1%) and DLBCL (11.1%). Though 4 (44.5%) had grade IV neutropenia (range: 10-187/μL), only 2 had concurrent leukocytopenia. Two patients tested positive on Bronchoalveolar lavage; the remaining had positive result in serum samples. The mean Galactomannan index (GMI) was 3.565 (range:1.731-5.721). All the six patients undergoing fungal culture of respiratory samples tested negative. As histopathological diagnosis of invasive Aspergillosis was not established in any of the cases, all fell into the category ‘probable aspergillosis’. Six patients received antifungals, five with amphotericin B and one with a combination of voriconazole and Amphotericin. Four patients were discharged against medical advice, one expired of progressive infection and four recovered. There was no association between GMI and age, gender, severity of leukocytopenia, neutropenia or type of cancer (haematological vs. non-haematological; 3.879 vs. 2.833, respectively). Conclusion: Leukaemia, bone marrow transplantation, cytotoxic chemotherapy, neutropenia, increase the pre-test probability of GMAg test. Though the assay does not help in proving invasive Aspergillosis, it is a useful test for its early identification. The test due to high negative predictive value will help in ruling out invasive Aspergillosis.


 > Case Report: 47 Top


Ewing's sarcoma of the left thumb: A rare site for Ewing's

Purvi Rathod, Ashok Kumar Diwan, Subeera Khan.

Ewing's sarcoma is a second most common bone tumor of childhood and adolescence and mainly affects pelvis, long bones and ribs. Involvement of the phalanx is very rare.1 This is a case report of Ewing's sarcoma of the left thumb in a tertiary care center. A 24 year male, right hand dominant, came with complaints of a swelling on left thumb since 2 years. Patient had undergone excision biopsy of the tumor 2 years back (histopathology s/o high grade malignant tumor). Patient had recurrence 2 months later and his MRI of left thumb was s/o large soft tissue lesion in left thumb distal to metacarpophalangeal joint 9x9x7 cms with cortical breech and marrow infiltration. Heunderwent left 1st carpometacarpal joint excision with left axillary lymph node dissection for same. A complete metastatic work up was done to rule out any distant metastasis. He is undergoing adjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide alternate with ifosfamide and etoposide regimen. In this case we emphasize on the importance of early initiation of diagnostic workup and treatment to prevent recurrence and metastasis in Ewing's sarcoma.


 > Case Report: 48 Top


Adenocarcinoma fistula in ano: A case report

Malini Mohan, C. Jomon Rapahel, K. Mathew Varghese, G. Paul Gopu

Adenocarcinoma of the anal canal accounts for 5 to 19% of all anal canal cancers. Adenocarcinoma associated with anorectal fistula is a rare entity which may be developmental or acquired secondary to underlying inflammatory bowel disease. The usual histologic appearance is of a well differentiated mucinous adenocarcinoma however tubular adenocarcinomas and squamous cell neoplasia can also be present. The cell of origin could be related to either rectal type glandular mucosa or anal glands or ducts. Case Report: 59 year old gentleman with a 4 month history of discharging sinus in perineal region. MRI Fistulogram showed multiple fistulous tracts with opening at 6' o clock position of natal cleft. An abscess in region of external sphincter with extension to intersphincteric space. Colonoscopy showed no suspicious lesions. Biopsy from lesion suggestive of mucinous adenocarcinoma with chondroid areas. He was treated with radical radiotherapy to a dose of 45Gy in 25 fractions to pelvis and bilateral inguinals followed by boost dose to perineal region along with concurrent chemotherapy (Capecitabine).


 > Case Report: 49 Top


Role of various fractionation schedules of radiation therapy in treatment of bone metastases

Kalyani Premchandra, A. G. Hasib

Objectives: To report the outcomes of various fractionation schedules of radiation therapy in terms of pain relief and improvement of quality of life in patients with painful bone metastases. Methods: A prospective randomised study performed on 80 patients were randomised to 4 treatment arms with different radiation therapy fractionation schedules at the Dept. ofRadiation Oncology, Father Muller Medical College Hospital. The fractionation radiationdoses used in the study were 8 Gy in 1 fraction, 20 Gy in 5 fractions, 24 Gy in 6fractions and 30 Gy in 10 fractions. Patients were assessed for pain by Visual AnalogScale (VAS) and Rupee scale, performance status, analgesic usage, quality of lifebefore initiating the treatment, on the day of completion of treatment, 1 week, 1month and 3 months post completion of treatment. Results: In our study majority of the metastases were from the breast of 33.75%, 25% from the lungs, 18.75% from the head and neck, and 12.5% from prostate. Cervix (2.5%), esophagus (1.25%), stomach (1.25%), liver (1.25%), ovary (1.25%) comprised the other primary sites. 2.5% of the metastases were from unknown primary. In total 27.5% of the patients had metastases to the thoracic vertebra, 26.25% to the lumbar vertebra, 22.5% to the pelvis, 8.75% to the sternum, 6.25% each to cervical vertebra and femur and 1.25% each to humerus and ribs. The pain reduction was significant in all the 4 arms. The mean VAS score was 5.23 prior to start of treatment. The score reduced to 2.88 at the completion of treatment and further dropped to 1.57, 0.79, and 0.81 at 1 week, 1 month and 3 months post completion of radiation therapy. Similar resultswere obtained using the Rupee scale for assessment of pain. The mean score prior totreatment was 54.23 in the entire study group. It is reduced to 33.81, 25.87, 12.78 and12.84 on the day of treatment completion, 1 week, 1 month and 3 months post completion ofradiation therapy. Complete pain relief occured in 40% of the patients. 25% of thepatients in 8 Gy in 1 fraction had complete relief of pain whereas the complete reliefin the remaining 3 arms was 45% each. Improvement in the quality of life wasobserved in all the arms, both in terms of symptoms and function. Conclusion: This prospective study demonstrated that various radiation fractionation schedules used in the treatment of painful bone metastases have been effective in reducing the pain. The pain reduction by various fractionation schedules are similar and no statistically significant difference was noted. Quality of life improved in all the four treatment arms post radiation therapy. 8 Gy in single fraction may be considered for treatment of painful bone metastases.


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Radiotherapy as a part of multi-modality treatment of ewings sarcoma: Experience of a tertiary cancer center

K. Ankita, C. Manish, B. Rajendra

Department of Radiation, Jupiter Hospital, Thane, Maharashtra, India

Aims and Objectives: Current standard treatment in Ewings Sarcoma (ES) is neoadjuvant chemotherapy followed by local treatment such as surgery, radiotherapy (RT), or a combination of both modalities. This is followed by further adjuvant chemotherapy. The aim of this study was to analyse our experience in RT in the management of ES. Materials and Methods: The medical records of ES patients who received RT in our department from Jan 2012 to Dec 2017 were reviewed. The patients received 3D conformal radiotherapy (CRT), Intensity Modulated Radiotherapy (IMRT) or Brachytherapy on Elekta or Siemens. The data were entered in excel sheet and was imported in SPSS, version 20 for statistical analysis. Results: Twenty patients with localized ES treated from 2012 to 2017 were included in this analysis. Two patients received extracorporeal radiotherapy. Primary tumor size was ≥ 8 cm in 60% patients. Median follow-up was 36 months. The 5-year overall survival (OS), progression-free survival, and local control (LC) were 60%, 50%, and 80%, respectively. According to treatment, 5-year LC was 60% with RT and 90% with combined surgery and RT (p=0.04). The important prognostic factors for OS were tumor size (≥ 8 cm, p < 0.001) and surgical resection (p < 0.001). Conclusion: RT is an important modality in the management of ES. Our patients had comparable outcomes to that of previously published literature.


 > Case Report: 51 Top


Post chemo radiotherapy feeding tube, tracheostomy dependence and mobility of larynx: A study of 100 patients

R. Anoop, K. U. Pushpaja, Subramania Iyer, Krishnakumar Thankappan, Deepak Balasubramanian, Khyati Vasavada, Arya S. Nair

Introduction:

  • Organ preservation regimens that combine chemotherapy and radiotherapy are increasingly used as the primary treatment of laryngeal and pharyngeal cancers.
  • Meta-analysis shows good survival rate in advanced laryngeal and hypopharyngeal cancers.
  • Swallowing difficulty, airway problems and hoarseness of voice is recognized as common and devastating late effects following chemoradiotherapy.
  • Drawback of regime is morbidity associated


Aims and Objectives: To evaluate feeding tube and tracheostomy dependency and the mobility of larynx in advanced laryngohypopharyngeal cancers treated with Chemoradiotherapy.

Materials and Methods:

  • Retrospective analysis of 100 patients.
  • Scopy-fixed vocal cord (T3 and T4 with out cartilage invasion).
  • Direct laryngoscopy and biopsy was done to confirm histopathology of tumor. Concurrent chemo radiotherapy.
  • Chemo radiotherapy is given concurrently in form of 70 Gy in 33 fractions and 3 weekly Cisplatin at 100 mg/m2 of body surface area.
  • At 6 months follow up parameters evaluated are: Feeding tube dependency, Tracheostomy dependency, Vocal Cord mobility.


Results:

  • Total no of patients – 100.
  • Male – 67 (67%).
  • Female – 33 (33 %).
  • Mean age – 51.
  • Age range – 40 – 78.


Conclusion:
  • Functional preservation is key focus of contemporary Head and Neck Cancer management.
  • Study shows 38 % feeding tube dependency at 6 months 14 % tracheostomy dependency.
  • Vocal cord mobility evaluated on scopy at 6 months shows improvement in 50 % of cases.
  • Results are comparable to other studies.





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