Objective- The aim of this case report of a patient presenting with diffuse abdominal pain, who was operated for ileocolic intussusception and then diagnosed as Burkitt’s lymphoma. 

Case Report-A 10 year old female presented in our emergency department with complaints of abdominal pain. Ultrasonography of the abdomen showed evidence of ring like bowel within bowel appearance, seen in the right hypochondrium suggestive of intussusception. Exploratory laprotomy was done, revealing short segment ileo-colic intussusception with large multiple mesenteric lymph nodes at root of mesentery, reaching a  maximum size of 3 x 3 cm. Reduction of ileocolic intussusception was done and mesenteric lymphnode biopsy taken. The histopathologic examination of the excised mesenteric lymphnode was suggestive of a high grade lymphoma, Burkitt lymphoma. The immunohistochemistry studies showed tumor cells were strongly positive for PAX5, BCL2, BCL6, CD10, CMYC and negative for CD3, Tdt, CD34. The Ki-67 was >90% which was suggestive of high grade mature B cell Non- Hodgkin lymphoma- Burkitt lymphoma. Burkitt lymphoma is an aggressive, highly malignant and rapidly growing B-cell neoplasm. Patients often originally present with nonspe¬cific symptoms, such as abdominal pain, gastrointestinal bleeding, anemia, or signs of acute abdomen. The lym¬phoma can be localized in mesenteric lymph nodes or extraperitoneal space. The most common extranodal location of Burkitt lymphoma is near the ileum or the cecum. High index of suspicision is necessary in children who are presenting with acute abdomen/ intussusception. 

Conclusion-Burkitt lymphoma is the most common pediatric gastrointestinal malignancy and pediatric surgeon should consider it when a child comes with unspecified clinical presentation with diffuse abdominal pain.

Burkitt lymphoma is an aggressive, highly malignant and rapidly growing B-cell neoplasm. Burkitt lymphomas represent 8-10% of all tumours in children under 15 years of age [1]. It was first described by Dennis Burkitt in 1958. Three clinical variants have been described – endemic, sporadic and immunodeficiency associated. The endemic form is more commonly seen in African region, and develops due to a chromosomal translocation between chromosome 8 and 14 [2]. It presents as tumour of jaw (maxilla & mandible) or other facial bones and may be associated with Epstein Barr virus (EBV) as well as frequent concomitant malaria infection. The sporadic form is less frequent but is seen worldwide mostly in United States. This results from a chromosome 8 translocation involving the c-myc oncogene and commonly presents as an abdominal mass involving the terminal ileum, caecum or mesentery, with ascites and atypical presentation mimicking acute appendicitis. The third form of Burkitt lymphoma is seen in immunocompromised patients, and typically presents with diffuse lymphadenopathy, bone marrow and central nervous system involvement. Intussusception caused by Burkitt lymphoma, with often misleading symptoms that make the diagnosis more difficult (3). It is the most common cause of intussuception in children above 4 years. Burkitt lymphoma has a high proliferation rate which gives the histological appearance of a “starry sky”, the blue lymphoid cells being the sky and numerous tingible body macrophages, due to the high proliferation, being the stars. (4). Burkitt lymphomas have an excellent prognosis, inspite of the stage of the disease due to the better response to the chemotherapeutic regimen. The aim of this case study is to highlight the rare presentation of Burkitts lymphoma with intusussception in paediatric age group. 

Case Presentation

A 10 year old female child presented in our emergency department with complaints of abdominal pain and backache for last 2 days. There was a history of similar episodes of abdominal pain for the last 2 months. On admission the child was pale and irritable, with a scaphoid abdomen, diffuse pain and tenderness in right upper quadrant and guarding. Bowel sounds were present. Laboratory investigations showed anaemia with a hemoglobin level of 6.8 g/dl and total leukocyte count of 13,900/cu mm with 75% neutrophils and 20%, lymphocytes,; erythrocyte sedimentation rate of 18 mm/hr and platelet count of 274,000/µl. Urine microscopy was normal. Her X- ray abdomen erect was unremarkable. Ultrasonography of the abdomen showed evidence of ring like bowel within bowel appearance seen in the right hypochondrium. Exploratory laparotomy was done under general anesthesia which revealed short segment ileo-colic intussusception with large multiple mesenteric lymph nodes at root of mesentery. The enlarged mesenteric lymph nodes had a maximum size of 3 x 3cm. Reduction of ileo-colic intussusception was done and mesenteric lymphnode biopsy taken.  Two units of blood were transfused. 

Postoperative recovery was uneventful. The histopathological examination of the excised mesenteric lymph node revealed atypical lymphoid infiltrate of sheets of monotonous population of small lymphoid cells with dispersed tingible body macrophages suggestive of a high grade Non-Hodgkin lymphoma possibly Burkitt lymphoma (Figure i). The immunohistochemistry studies showed tumor cells were positive for PAX5, BCL2, CD10, BCL6, C-MYC (Figure 2) and were negative for CD3, Tdt and CD34. This confirmed the diagnosis of a high grade mature B cell Non- Hodgkin lymphoma-Burkitt lymphoma. Burkitt lymphomas are usually BCL2 negative, or may show weak positivity. Our case showed a diffuse strong positivity for BCL2, BCL6 and MYC, (Fig.iv and v) which leads us to consider the newer entity of High grade B cell lymphomas (HGBL) with rearrangements of all three. These may be called as double hit or triple hit lymphomas. This diagnosis is made on FISH studies for the rearrangements. Our study is limited by the absence of FISH studies due to monetary restraints.

Figure (i): Sheets of small atypical lymphoid cells, with interspersed tangible body macrophages; classic of Burkitt lymphoma. Figure(ii) shows PAX5  positivity, Figure(iii) CD10 positivity. Figure (iv) c-myc positivity. Figure (v) shows diffuse strong positivity for BCL2.

Intussusception is the most common pediatric surgical emergency seen in the early age group with a clinical triad of abdominal colic, red currant jelly stool and palpable mass. The incidence of primary gastrointestinal lymphoma is around 1-4 % of all gastrointestinal malignancies with Burkitt lymphoma accounting for 0.3-1.3% of all Non Hodgkins lymphomas (5). The peak age of presentation of gastrointestinal NHL in children is 5-15 years with males being 1.8-2.5 times more affected as compared to females (6). Distal ileum or ileocecal region is the most commonly involved in children. It is difficult to suspect a lymphoma in pediatric patients presenting with acute abdomen. Intussusception is the first clinical sign in nearly 18% of case with primary abdominal Burkitt lymphoma. In 80