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

Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma that typically presents with abdominal masses; ampullary involvement with obstructive jaundice and gastrointestinal bleeding is exceptionally rare in children. Early biliary decompression can be crucial to permit timely chemotherapy.

A 14-year-old boy presented with 1 week of worsening abdominal pain, non-bilious vomiting, constipation, jaundice, and melena. Examination showed pallor, icterus, firm hepatomegaly, and a palpable epigastric mass. Laboratory testing revealed cholestatic liver function abnormalities and elevated pancreatic enzymes. Ultrasound and contrast computed tomography demonstrated a large retroperitoneal mass compressing the biliary tree. Upper gastrointestinal endoscopy identified a friable, ulcerated ampullary mass with active bleeding. Biopsy confirmed BL by morphology (starry-sky) and immunohistochemistry/fluorescence in situ hybridization (CD20+, CD10+, c-MYC+, Ki-67 ~95%). Main diagnosis made was pediatric BL presenting with ampullary involvement causing obstructive jaundice and upper gastrointestinal bleeding. Given persistent cholestasis and bleeding risk, the patient underwent Roux-en-Y choledochojejunostomy for biliary decompression, followed by initiation of rituximab-cyclophosphamide-vincristine-doxorubicin-high-dose methotrexate/rituximab-ifosfamide-etoposide-high-dose cytarabine (R-CODOX-M/R-IVAC) with central nervous system prophylaxis. Post-operative bilirubin improved, and early chemotherapy cycles were tolerated. During the 2-week hospitalization, the patient had symptomatic improvement; however, objective radiologic response could not be documented because care was transferred and post-transfer positron emission tomography-computed tomography was unavailable.

Ampullary BL should be considered in pediatric patients with obstructive jaundice and upper gastrointestinal bleeding. Surgical biliary decompression can stabilize cholestasis and facilitate timely multi-agent chemotherapy.