|Year : 2020 | Volume
| Issue : 1 | Page : 66-70
A case series of unusual presentations of Burkitt's lymphoma
Sunita Dashottar1, BS Sunita2, RK Singh3, Vandana Rana4, Virender Suhag5, AK Singh6
1 Department of Radio-Diagnosis, Military Hospital (CTC), Pune, Maharashtra, India
2 Department of Pathology, Army Hospital R&R, New Delhi, India
3 Department of Anaesthesia, Pacific Medical College, Udaipur, Rajasthan, India
4 Department of Pathology, Command Hospital (SC), Pune, Maharashtra, India
5 Department of Radiation Oncology, Army Hospital R&R, New Delhi, India
6 Department of Radio-Diagnosis, Army Hospital R&R, New Delhi, India
|Date of Submission||18-Apr-2016|
|Date of Decision||18-Sep-2017|
|Date of Acceptance||06-Apr-2018|
|Date of Web Publication||24-Oct-2018|
B S Sunita
Department of Pathology, Army Hospital R&R, New Delhi - 110 010
Source of Support: None, Conflict of Interest: None
Context: Burkitt's lymphoma (BL) is one of the fastest growing malignancies. It is the most common subtype of Non-Hodgkin's lymphoma in childhood. It has three major subtypes – endemic, sporadic, and immunodeficiency-associated types.
Aims: This study aims to study the clinicomorphologic features of this disease entity and to find optimal imaging technique for such cases.
Setting and Design: A retrospective observational study in a tertiary care center of academic and research potential.
Subjects and Methods: We are presenting three unusual cases of sporadic type of BL who presented initially as localized right iliac fossa mass mimicking as acute appendicitis. Initially, localized presentation progressed to diffuse abdominal mass lesions causing intestinal obstruction.
Results: These cases had emphasized the importance of accurate diagnosis by the ultrasonography (USG) or computed tomography (CT) scan for early diagnosis so as to manage such cases simply by early appropriate medical treatment.
Conclusion: In this article, we will discuss the clinical and imaging features of BL with the role of USG, CT scan and positron emission tomography/CT in the abdominopelvic imaging of pediatric patients.
Keywords: Burkitt's Lymphoma, childhood, imaging
|How to cite this article:|
Dashottar S, Sunita B S, Singh R K, Rana V, Suhag V, Singh A K. A case series of unusual presentations of Burkitt's lymphoma. J Can Res Ther 2020;16:66-70
|How to cite this URL:|
Dashottar S, Sunita B S, Singh R K, Rana V, Suhag V, Singh A K. A case series of unusual presentations of Burkitt's lymphoma. J Can Res Ther [serial online] 2020 [cited 2020 Jun 6];16:66-70. Available from: http://www.cancerjournal.net/text.asp?2020/16/1/66/243488
| > Introduction|| |
Burkitt's lymphoma (BL) is one of the fastest growing malignancies which is the most common subtype of Non-Hodgkin's lymphoma (NHL) in childhood. It has three major subtypes – endemic, sporadic, and immunodeficiency-associated types. We are presenting three unusual cases of sporadic type of BL who presented initially as localized right iliac fossa mass mimicking as acute appendicitis. Initially, localized presentation progressed to diffuse abdominal mass lesions causing intestinal obstruction (IO). These cases had emphasized the importance of accurate diagnosis by the ultrasonography (USG) or computed tomography (CT) scan for early diagnosis so as to manage such cases simply by early appropriate medical treatment. In this article, we will discuss the clinical and imaging features of BL with the role of USG, CT scan, and positron emission tomography (PET)/CT in the abdominopelvic imaging of pediatric patients.
| > Subjects and Methods|| |
A 5-year-old male presented with pain abdomen, vomiting, and progressive distension of abdomen. USG of the abdomen showed multiple enlarged soft-tissue mass lesions in the mesentery, retroperitoneum, right iliac fossa, and pelvis. Multiple dilated bowel loops were also seen. Radiograph of the abdomen erect showed multiple air-fluid levels in the abdomen suggestive of IO. Exploratory laparotomy (EL) was done to relive the obstruction which showed 10 cm × 15 cm size confluent enlarged lymph nodal mass lesion fixed to the terminal ileum. The lump was excised, and resection anastomosis of the small intestine was done. Contrast-enhanced CT scan (CECT) of the abdomen showed multiple enlarged confluent lymph nodes in the entire abdomen with mild ascites. Spleen and liver were normal. Histopathological examination (HPE) and immunohistochemistry (IHC) findings were consistent with BL. He was given prophase chemotherapy (chemo) and postchemo the abdominal mass lesions were significantly regressed which was later confirmed on CT scan [Figure 1].
|Figure 1: Contrast-enhanced computed tomography scan of the abdomen axial section. (a) Confl uent soft-tissue density mass lesions are seen in the prehepatic space indenting and displacing the liver posteriorly. Bilateral mild pleural effusion is also seen. (b) Confl uent soft-tissue density mass lesions are seen in the right lumbar region in the peritoneal and retroperitoneal spaces displacing the opacifi ed small bowel loops to the contralateral side|
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A 4.5-year-old male presented with pain abdomen and abdominal lump. Initially, he was treated in the peripheral hospital as a case of subacute intestinal obstruction with abdominal mass. He underwent EL in a peripheral hospital, which showed ileocaecal intussusception. Hence, fixation of ileal loop along with appendectomy was done. HPE of the specimen showed reactive lymphadenopathy with normal appendix. However, his symptoms did not improve. Therefore, after 6 days, he underwent right hemicolectomy. Even after the operation, he kept on complaining of intermittent abdominal pain off and on with firm to hard lumps were noted in the entire abdomen. Hence, he was referred to tertiary care hospital. CECT abdomen showed multiple confluent soft-tissue density mass lesion in the entire abdomen extending from the dome of diaphragm to pelvis involving the peritoneal and retroperitoneum spaces predominantly in the infrahepatic and right lumbar region. The enlarged lymph nodal mass lesions were closely abutting and compressing the bowel loops and encasing the vessels; however, free passage of contrast was noted through the bowel loops. Mild ascites and right pleural effusion was also seen. Imaging findings were suggestive of lymphoma with the possibility of BL was made. HPE and IHC of the lymph nodal mass were suggestive of Burkitt's lymphoma. The patient was started on antitumor lysis syndrome therapy, and prophase chemo, with this significant regression of the abdominal mass lesions, was seen [Figure 2].
|Figure 2: Contrast-enhanced computed tomography scan of the abdomen coronal and sagittal reformate images. (a and b) Confluent soft-tissue density mass lesions are seen around the liver, infrahepatic space, right lumbar, and lower abdomen displacing the bowel loops to the contralateral side|
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A 21-year-old male presented with inguinal swelling progressing over 2 months associated with weight loss, anorexia, and vomiting for 1 month. Radiograph of the chest showed right-sided pleural effusion. USG of the abdomen showed multiple well-defined enlarged lymph nodes seen in the mesentery, retroperitoneum, pelvis and right inguinal region largest measuring 7.1 cm × 4.7 cm × 6.2 cm in lower abdomen. CECT abdomen and pelvis confirmed the USG findings. Bilateral moderate pleural effusion was also seen. Excision biopsy from right inguinal lymph nodes showed high-grade large cell NHL [Figure 3]. PET scan showed extensive fluorodeoxyglucose (FDG) avid supra- and infra-diaphragmatic lymphadenopathy with pleural and peritoneal thickening. FDG avid skeletal deposits were also seen in the pelvic bones, entire spine, and bilateral scapula. No FDG lesion was seen in lung, liver, spleen, and adrenals [Figure 4].
|Figure 3: Contrast-enhanced computed tomography scan abdomen coronal reformats of case-3, pre- and post-chemotherapy. (a) Confluent soft-tissue density mass lesions in infrahepatic, right lumbar, and mesentery compressing and displacing the bowel loops. (b) Postchemotherapy scan shows almost complete resolution of the mass lesion noted|
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|Figure 4: Emission computed tomography scan fluorodeoxyglucose avid lesions are noted in the abdomen as well as in the anterior and posterior mediastinum and the vertebral bodies|
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HPE showed total effacement of lymph nodal architecture by the atypical lymphoid cells. These atypical lymphoid cells were medium to large size with have high NC ratio, irregular nuclear chromatin with multiple small basophilic nucleoli. Many of these were squared off with retraction space around them. Scattered among them are tangible body macrophages ingesting apoptotic bodies giving a starry sky appearance. Mitosis was brisk with no necrosis [Figure 5].
|Figure 5: Histopathological low (a) and high-power microscopic image (b) shows: Atypical lymphoid cells of medium to large size with high NC ratio, irregular nuclear chromatin with multiple small basophilic nucleoli are seen. Many of these are squared off with retraction space around them. Scattered amongst them are tingible body macrophages ingesting apoptotic bodies giving a starry sky appearance|
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IHC showed tumor cells were strongly positive for LCA, CD20, CD10, BCI6, MIB L1-95%–100% but were negative for BCI2, TdT. CD3. CD5. HPE and IHC were consistent with BL (Classical type). He was started on Magrath regimen, and the patient showed significant improvement.
| > Discussion|| |
BL is an extremely aggressive B-cell Non-Hodgkin lymphoma characterized by translocation and deregulation of c-myc oncogene on chromosome 8 and the incidence is 34% of all NHL. It is the fastest growing tumor in humans with doubling time is as short as 24 h so early diagnosis and introduction of chemotherapy are essential components for complete cure. It commonly occurs in males with male to female ratio ranging from 1.3:1 to 8.8:1. It occurs in the age ranging from 0 to 20 years with median age 8 years and one third occurs in 5–9 years. The World health organization has described BL into three subtype-endemic, sporadic and immunodeficiency-associated types. Endemic (African) type involves mainly the maxilla and mandible while nonendemic (sporadic) type involves mainly the abdominal organs like distal ileum, cecum and mesentery. Ninety-five percent of endemic type is associated with Epstein-Barr virus while sporadic type in 15% of the cases. Immunodeficiency associated type mainly occurs in HIV patients but can also occur in allograft recipients and congenital immune deficiencies diseases. The endemic variety clinically presents by maxillary/mandibular swelling (26.3%), proptosis (5.3%), and frontal swelling (3.3%) while the sporadic variety presents with abdominal mass (73.7%). All three of our cases were of sporadic variety. BL can be localized or disseminated and can involve various sites. Sporadic type of BL usually develops in the abdomen and spreads to other organs, including the brain. In abdomen, it can involve gastrointestinal tract (GIT) and solid organs. The incidence of involvement of GIT is 22.5%, abdominal/pelvic masses in 45%, and hepatic lesions in 17% of cases. It can also involve the head and neck, the genitourinary tract, gonads, mesentery, peritoneum, and retroperitoneum. Approximately 90% of sporadic type of BL and 50% of endemic type presents with abdominal masses while jaw tumors are very rare in sporadic BL.
Radiology helps in early diagnosis which defines the treatment outcome. BL should be excluded by HPE in children presenting with intra-abdominal masses. Clinical manifestation in the abdomen is primarily due to compression, obstruction, and infiltration of the surrounding structures by the malignant cells. Children usually presents as nausea, vomiting, abdominal pain and distension due to abdominal masses, intestinal obstruction due to bowel compression or intussusceptions or acute appendicitis. The patient may develop fever with weight loss in disseminated type. Children may sometime present with obstructive jaundice due to compression of the biliary tree by the periportal lymphadenopathy.
Imaging techniques most commonly used were USG, CT, PET/CT, and bone scan. USG is the initial modality used in children who present with abdominopelvic masses. CT scan not only shows the visceral and bowel involvement but also helps in doing the staging of the disease. PET/CT is the most widely used functional imaging technique for the initial staging and further evaluation of the treatment response.
Gastrointestinal tract involvement
BL most commonly involves distal ileal loops, caecum, and appendix due to the presence of prominent lymphatic tissue. It may lead to enlarged focal masses or diffuse wall thickening. Enlarged lymph nodes usually lead to intussusception. Diffuse bowel wall thickening may be due to tumor infiltration or lymphedema. The incidence of involvement of appendix is 1%–3% and presents with nonspecific findings of acute appendicitis due to diffuse wall thickening. BL may also involve the proximal bowel loops or stomach. The involvement of stomach is very rare and may show as diffuse or focal wall thickening.
Mesenteric and retroperitoneal disease
BL usually presents as confluent lymph nodal masses encasing the major abdominal vessels and displacing and compressing the bowel loops. The lymph nodal masses may show central necrosis or fluid levels. Calcification can also be seen as punctate or linear in the center or amorphous in the periphery of the large masses type.
Nodular lesion can be seen all along the peritoneum reflection or over the liver surface or may also cause thickening of the omentum. Ascites is seen in 25% of the cases. Solid organs – liver is involved in 17% of the BL cases. It can have solitary or multicentric lesions or periportal infiltrating mass lesion with or without biliary duct dilatation. Spleen can show diffuse enlargement or low attenuating mass lesions. Pancreas involved in 10% of the cases which may be seen as focal enlargement or focal masses leading to obstructive biliary dilatation. Gallbladder involvement may be seen as diffuse wall thickening or intraluminal filling defects. Kidneys may be involved due to direct infiltration by the tumor cells or obstruction or paraneoplastic effects such as glomerulonephritis, paraproteinemia, or cryoglobulinemia. Kidney involvement may be as diffuse involvement in 90% of the cases or focal renal masses in 30% or hydronephrosis due to ureteric obstruction in 50% of cases. Postchemotherapy renal calculi formation may be due to tumor lysis syndrome or renal failure due to obstruction may be seen in one-third of the patients. Gonads-testis may be involved in 25% of the cases which may be diffusely enlarged or intra-testicular lesions. The epididymis or spermatic cord may be involved with or without testicular involvement. Epididymis may show diffuse enlargement or focal lesions on imaging.
Microscopic appearance of BL shows uniform monoclonal proliferation of B-cell lymphocytes with scanty basophilic cytoplasm, numerous vacuoles, round nuclei with stippled chromatin and multiple nucleoli. The characteristic feature of BL is the presence of “starry sky appearance” due to the presence of tingible body macrophages. IHC shows B-cell lymphocytes with surface markers positive for CD19, CD20, CD22, IgM, and positive Ki67 staining.
Treatment of BL is by short- or long-term chemotherapy (Chemo) while surgery and radiation has no role. Short-term chemo is by Magrath regimen and CALGB 9251 protocol while long term is by hyper CVAD and CALGB 8811 protocol. Rituximab is the latest drug which has been added in the regime.
We had two cases of BL in the age group of 4 and 5 years and one adult of 21 years and all were males. They all presented with the clinical manifestation of pain abdomen and abdominal distension. Initial modality used was USG then further evaluated by CT and PET-CT. On image, all three had solid mass lesions all over the abdomen in the mesentery as well as in retroperitoneum. Mandible is not involved. Imaging helped us in the diagnosis and staging of the disease as well as in the assessment of treatment response. In our study, complete remission was seen in two of our patients while the adult patient developed some complications as abdominal wall and intra-abdominal abscess due to severe neutropenia. However, with the broad spectrum antibiotics, the patient showed complete recovery.
| > Conclusion|| |
BL is a treatable childhood malignancy with significant geographical variation in the incidence, clinical presentation, and response to treatment. Our cases achieved complete remission with chemotherapy. Although ultrasound is the initial imaging modality to study in a child with an abdominal mass; however, CT is often used because of the multifocality of presentation and a propensity to involve bowel and mesentery. PET/CT recently has become the preferable functional imaging technique in children because of the shorter interval between injection and imaging, completion of the studies in only a few hours, improved image quality, and better dosimetry.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The authors would like to thank the Oncology Division and Oncopathology department for their contribution and inputs.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]