Unusual Ampullary Presentation of Pediatric Burkitt Lymphoma: Case Report and Literature Review

Abstract Background: Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma with a predilection for abdominal involvement. Ampullary localization is exceptionally rare and can mimic adenocarcinoma, posing major diagnostic challenges. Case Presentation: We report a 14-year-old boy presenting with abdominal pain, vomiting, jaundice, and melena. Examination revealed pallor, icterus, hepatomegaly, and a palpable epigastric mass. Laboratory evaluation showed anemia, direct hyperbilirubinemia, elevated transaminases, and high lactate dehydrogenase. Computed tomography demonstrated a large retroperitoneal mass encasing major vessels and compressing the biliary tree. Upper gastrointestinal endoscopy revealed an ulcerated ampullary lesion. Histopathology showed a “starry-sky” pattern; immunohistochemistry was positive for CD20, CD10, and c-MYC, with Ki-67 ~ 95%, confirming BL. A Roux-en-Y choledochojejunostomy was performed for biliary decompression, followed by intensive R-CODOX-M/IVAC chemotherapy with CNS prophylaxis. Discussion: Ampullary BL is exceedingly uncommon in children and may be mistaken for carcinoma. Multidisciplinary management with early biopsy, biliary decompression, and timely initiation of intensive chemo-immunotherapy is essential. Conclusion: This case highlights an unusual pediatric presentation of BL and underscores the importance of considering lymphoma in ampullary masses to enable prompt treatment and improved outcomes.