|Year : 2015 | Volume
| Issue : 7 | Page : 104-105
|Date of Web Publication||24-Nov-2015|
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
. Paediatrics. J Can Res Ther 2015;11, Suppl S3:104-5
Case report of quadrilateral and trilateral retinoblastoma patients in retrospective analysis of 141 retinoblastoma patients (between 1983 and 2013) from GMCH, Nagpur
thR. Kabre , K. Kamble, A. Diwan, V. Mahobia
GMCH, Nagpur, Maharashtra, India, E-mail: [email protected]
Background: A presentation of intracranial tumor arising usually in the pineal region, rarely at the suprasellar or parasellar site in bilateral and unilateral retinoblastoma with or without family history is termed as trilateral retinoblastoma. Whereas trilateral retinoblastoma along with sellar and suprasellar intracranial neoplasm is termed as quadrilateral retinoblastoma. Trilateral and quadrilateral retinoblastomas are extremely rare. They occur in 1.5-5% of patients with unilateral or bilateral retinoblastoma and 2-11% in patients with bilateral retinoblastoma. The retrospective study is done by reviewing clinical records of 141 retinoblastoma patients (162 eyes) from 1983-2014. We report one case of quadrilateral retinoblastoma & two cases of trilateral retinoblastoma seen in this study. Case report- First case was variant of trilateral retinoblastoma with radiological features of left eye lesion with supra sellar mass at diagnosis who showed partial response to chemotherapy. Second case was deemed as quadrilateral retinoblastoma based on MRI findings of left intraocular retinoblastoma without intracaranial extention with suprasellar lesion showing calcification along with enlarged pineal gland with homogenous enhancement & intensity similar to suprasellar lesion. Third case was trilateral retinoblastoma with multiple extra axial and leptomeningeal metastasis along with pineoblastoma. Discussion: Quadrilateral & Trilateral retinoblastoma is rarely present at diagnosis of ocular disease. Commonly these entities are associated with bilateral retinoblastoma. In our series all patients presented with unilateral disease with intracranial lesion at diagnosis. Screening neuroimaging is a routine practice nowadays for early detection of asymptomatic intracranial tumors. The standard treatment for intracranial tumors in patients with retinoblastoma is surgical resection followed by chemotherapy and cranial or cranio-spinal radiation; but advanced stage disease with poor performance status precluded these protocols from being followed in our patients. Conclusion: These entities are reported in view of rarity and poor prognosis. Because of the lethality of the intracranial lesion in these variants of retinoblastoma, Brain screening by MR imaging with an emphasis on the pineal and suprasellar regions is imperative for early diagnosis even in unilateral disease as all our cases had unilateral eye disease.
Retinoblastoma: A retrospective analysis pertaining to clinical characteristics and amp; changing trend in the management of 141 patients (between 1983 and 2013) from GMCH, Nagpur
thR. Kabre , K. Kamble, A. Diwan
GMCH, Nagpur, Maharashtra, India, E-mail: [email protected]
Background: Retinoblastoma is most common intraocular tumour of childhood. It is a malignant tumor that originates from developing retina. Diagnosis is based on clinical signs and symptoms and is usually made in children under the age of five years. Nagpur cancer registry has shown incidence of Retinoblastoma as 0.1/100,000 population. A Retrospective analysis from 1983 to 2013 was done where total 173 patients of Retinoblastoma were registered but the records of 141 patients (162 eyes) were available for analysis. Aim: To evaluate Clinical profile, Management, Treatment & Follow up for Retinoblastoma patients at GMCH, Nagpur. Materials and Methods: The retrospective study is done by reviewing clinical records of 141 retinoblastoma patients (162 eyes). Personal, clinical & disease profile, as well as treatments and their results along with complications & outcome is analysed and being presented. Results: Of 141 patients, 81 (57.45%) presented in age group of 1-3 years. 81 (57.45%) males and 60 (42.55%) were females. 114 patients (80.85%) had unilateral, while 24 (17.02%) had bilateral and 2 (1.42%) had trilateral retinoblastoma. One (0.71%) case of quadrilateral retinoblastoma was also noted most common presenting symptom was proptosis/swelling of eyes seen in 81 (57.45%) patients. Calcification as presenting sign was seen in 45 (31.92%) 139 eyes could be staged, as per International Classification of Retinoblastoma and Reese Ellsworth Classification, of which 85 (61.15%) were in group E, 24 (17.27%) in group D, 14 (10.07%) in group C, 13 (9.35%) in group B while 3 (2.15%) in groupa. Surgery was done in 75 (53.19%) patients in which 65 (86.67%) underwent enucleation. 103 (73.05%) were treated by EBRT. Adjuvant or neoadjuvant chemotherapy was received only by 46 (32.62%) patients, majority being in latter half. Mean follow up of all patients was 112 days. Majority of patients were lost for follow up, with disease 64 (45.39%). Conclusion: This study shows relevant data from major institute in India which caters to large population which is rural based; in which poverty, illiteracy & lack of access to healthcare resources is widespread leading to high rate of advanced disease at presentation. Conducting Retinoblastoma awareness programmes, development of screening strategies, equipping the primary health care system for early diagnosis, prompt referral to specialized centers, improvement of patient follow up will improve prognosis and quality of life in patients.
Adaptive radiation in a case of intracranial localised germinoma
thG. Nethula , S. Dikkala, S. Surana, P. R. Thammineni, N. Rajamoni
Krishna Institute of Medical Sciences, Hyderabad, Telangana, India, E-mail: [email protected]
Background: Germinomas are the most common gcts of pineal region & are more radiosensitive. Radiation alone had given cure rate of >90%. There is a trend in clinical trials to use neoadjuvant chemotherapy [NACT] which allows reduced doses & volumes of RT thereby reducing long term effects of RT. However optimal target volumes after NACT remains to be defined & focal RT after NACT has resulted in local failures. This mandates whole ventricular radiation [WVRT] irrespective of response to NACT. Herein we present a case of intracranial localised germinoma, treated with RT alone using adaptive RT technique. Case Report: A 12yr old boy presented with headache of 15days duration. MRI brain showed 4.3 x 5.3 x 4.8 cm well defined mass lesion in pineal region with extension into 3rd & 4th ventricles. CSF was negative for tumor cells. MRI spine screening showed no tumor deposits. Biopsy done from primary lesion. HPE & IHC features were consistent with Germinoma. Serum HCG & AFP values were in favour of Germinoma. Patient was planned for radical RT with WVRT to a dose of 24 Gy at 150 cGy/fr followed by tumor boost upto 45 Gy using Rapid arc technique. CTV Phase I was created by expansion of gross tumor [GTV + 1 cm as ctvp] & whole ventricles [ventricles + 1 cm as ctvwv] with 3mm PTV margin. At the end of 1st week CBCT has shown significant reduction in GTV. Volume of whole ventricles was also reduced owing to improved outflow as tumor shrunk. Patient was planned for adaptive RT. Re-Planning CT scan was performed after 8 fractions. Keeping ctvp constant, new ctvwv was created & added to ctvp to create Re-plan CTV1, which showed reduction in target volume by 6%. Expecting more reduction another re-planning CT was done at the end of 16 fractions. Re-plan CTV2 was contoured similar to last time which showed reduction in Target volume by 20%. New plans were generated & delivered each time with new ctvs. Thereafter tumor [GTV + 1 cm as ctvp] was boosted by 21 Gy. Conclusion: As germinomas are radiosensitive & significant shrinkage of tumor is expected during RT, practice of adaptive RT can lead to incremental reduction in dose to normal brain tissue thereby preserving long term neuro cognition in young patients.
Primary intrarenal neuroblastoma mimicking wilms' tumor in a 7-year-old girl: A case report
thS. Samdariya , P. Bagri, P. Pareek, A. Sinha, P. Elhence,
All India Institute of Medical Sciences, Jodhpur, Rajasthan, India, E-mail: [email protected]
Background: Primary intrarenal neuroblastoma (NB) primarily arises from kidney. Owing to the similar locations, this tumor can mimic Wilms' tumor, a more common pediatric renal tumor at presentation. As both have different treatment and prognosis, it is crucial to distinguish them from each other. Due to its rarity with less than 100 cases described in English literatures, the characteristics of primary intrarenal NB have not been widely studied. Aim: The authors report this case of primary intrarenal NB, which occurred in right kidney of a 7-year-old girl in order to illustrate the characteristic features of this tumor. Materials and Methods: A 7 year old girl from Jodhpur presented with complaints of progressive abdominal distension since 3 months & pain in the left side of abdomen since 1.5 months. She had born to non-consanguineous marriage. On examination a huge lump was found occupying of abdomen. CECT revealed a large enhancing irregular/lobulated soft tissue density lesion measuring 12 x 10 x 14 cm with cystic/necrotic areas and focal dystrophic calcifications in left hypochondrium and left lumbar region involving left kidney with encasement of renal vessels. With the age at presentation and involvement of kidney, a presumptive diagnosis of wilms tumor was made and left sided radical nephrectomy and splenectomy was done. HPE revealed an irregular lobulated whitish red soft tissue mass with reniform appearance weighing 900 grams and measuring 30 x 12.5 x 6.5 cm. Mass infiltrated adherent segment of intestine. On sectioning, no cortico-medullary differentiation was made out. Ureter and pelvis could not be identified. Microscopy revealed poorly differentiated neuroblastoma (<5% differenatiation), unfavorable histology according to revised shimada classification with high mitosis-karryorhexis index (MKI >4%). On IHC tumor cells were positive for chromogranin, synaptophysin but negative for vimentin & desmin. Brisk mitotic activity+ (15/HPF) and large areas of necrosis were present. Splenic hilum and wall of intestine infiltrated by tumor. Results: Final diagnosis of poorly differentiated left sided intrarenal neuroblastoma INSS Stage III & INRG-intermediate risk was made. Currently she is on her 4th cycle of chemotherapy. Conclusion: Intrarenal neuroblastoma is a very rare tumor arising in kidney in children. A high index of suspicion is required to differentiate it from wilms tumor.
Conformal radiation therapy for paediatric orbital granulocytic sarcomas
thB. Prasad , S. Pathy, S. Mallick, A. Biswas, S. Chander
All India Institute of Medical Sciences, New Delhi, India, E-mail: [email protected]
Aim: Orbital Granulocytic sarcoma (OGS) is an uncommon manifestation associated with hematological malignancies. The role of radiation is not defined. Materials and Methods: We retrospectively retrieved radiotherapy treatment data of 11 consecutive pediatric patients of OGS. Results: The median age was 7 years (Range: 2-16 years). OGS was most commonly associated with Acute myeloid leukemia (AML) M2 in 6 (54.4%), AML M2 in 2 (18.18%), Primary orbital granulocytic sarcoma in 2 (18.18%), Bi-phenotypic leukemia in 1 (9%). Amongst imaging modalities most commonly employed were Contrast enhanced computed tomography of orbit (CECT) in 7 (63.63%), Magnetic resonance imaging (MRI) in 2 (18.18%), Positron emission tomography (PET) in 1 (9%) and in 1 patient imaging was not done. Bone marrow report was available in 10 patients. 8 patients with hematological malignancies had blasts ranging from 8% to 90% at baseline. Two patients with primary OGS had normal marrow aspirate. Cerebrospinal fluid analysis (CSF) was done in all patients with only one (9%) being positive. Timing of OGS development was documented with five (45.45%) had lesion in orbit at time of diagnosis of AML, three (27.27%) developed post completion of consolidation therapy, two (18.18%) had Primary OGS without any association of hematological malignancies, one (9%) developed during course of leukemia treatment. Patients received radiation therapy to involved orbit by 3DCRT. Radiation dose prescribed was varied with median of 24.5 Gray (range 15 to 45 Gray). Two fields were used for radiation planning with 8 (72.72%) planned with two oblique fields and 3 (27.7%) planned with one direct anterior and one lateral oblique field. The prescription iso-dose was varied ranging from 90% to 97% with 5 (45.45%) plans prescribed at 90% isodose, 4 (36.36%) prescribed at 95% isodose, 1 (9%) each prescribed at 91 and 97% isodose. Among eleven patients six (54.4%) had complete response, 3 (27.27%) partial response, 1 (9%) each had stable disease and progressive disease. Follow up was varied ranging from 2 to 7 years with five (45.45%) patients alive and 6 (54.54%) patients expired in view of disease progression. Conclusion: In patients with residual orbital disease low dose radiation can be used to improve local disease control and improve quality of life. Local conformal radiotherapy of 24-30 Gray appears optimal with excellent local control and minimal morbidity.
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