A Rare and Aggressive Lymphoma; Burkitt Lymphoma in the Terminal Ileum

Abstract

Burkitt Lymphoma (BL) is an aggressive form of high-grade B-cell neoplasm that is rarely identified in the adult population. The World Health Organization (WHO) describes three clinical variants; endemic, sporadic, and immunodeficiency associated.The following case is a rare presentation of Primary small intestine Burkitt lymphoma in an adult male presenting with fatigue, dyspnea on exertion, and unexplained weight loss. We present a case of an 81-year-old male with a past medical history of stage I Hodgkin's lymphoma treated with chemotherapy(may be state chemo regime) approximately 8 years ago, non-insulin-dependent diabetes, hypertension, hyperlipidemia, chronic kidney disease who presented to our a tertiary center with the complaints of increasing weakness, fatigue, and unexplained weight loss. In the emergency department the patient was found to have acute kidney injury and hypercalcemia 14.1mg/dL (Ca 8.4 - 10.3 mg/dL). Due to concern for malignancy with hypercalcemia and the patient's history of Hodgkin's lymphoma a computed tomography (CT) chest, abdomen and pelvis was obtained. Imaging revealed concerning findings of peritoneal carcinomatosis with gastrointestinal malignancy as possible source. Gastroenterology was consulted and an EGD as well as colonoscopy were performed.Colonoscopy revealed diverticulosis in the sigmoid colon and in the descending colon, a malignant-appearing tumor in the distal ileum that was biopsied. Pathology revealed an aggressive B-cell lymphoma with Burkitt like morphology and a germinal center phenotype. The infiltrate was positive for CD 20, CD 10, BCL 2, BCL 6, and MUM1. The Ki 67 proliferation rate was greater than 90%. Overall, the morphology and high proliferation rate were consistent with Burkitt lymphoma. The patient chose to follow up at an alternative facility for his ongoing oncology care. Burkitt lymphoma is a very aggressive form of B-cell lymphoma the sporadic form occurring worldwide with a predilection for children. Clinical symptoms depend on the site of involvement. There is frequently extranodal involvement commonly occurring in the gastrointestinal tract. CT findings can include peritoneal thickening, as well as ascites, and omental involvement. Often times when there is diffuse peritoneal disease, it is usually associated with a focal gastrointestinal mass. Although rare, Burkitt lymphoma should be kept on the differential when investigating CT findings of what appears to be peritoneal carcinomatosis.2530_A Figure 1. peritoneal carcinomatosis2530_B Figure 2. Malignant appearing tumor in the distal ileum