A Puzzling Case of Pouch Pathology

Abstract

A 36-year-old man with familial adenomatous polyposis secondary to an adenomatous polyposis coli mutation status post proctocolectomy with ileal pouch-anal anastomosis presented with hematochezia. Pouchoscopy revealed a 4-cm indurated mass in the distal ileal pouch just 17 months after a normal pouchoscopy. Histopathology was diagnostic for Burkitt lymphoma, and the patient achieved complete remission with subsequent chemotherapy. Although there are reports of Burkitt lymphoma in patients with ileal pouch-anal anastomosis, to date, this is the first report in a patient with familial adenomatous polyposis. This case highlights the presentation of a rapidly enlarging tumor not commonly seen in the adult gastroenterology population.

Histopathological Evaluation of Pouch Neoplasia in Inflammatory Bowel Diseases and Familial Adenomatous Polyposis

“…45,46 There is no known established increased risk for lymphoma in patients with FAP; however, EBV-negative Burkitt lymphoma in the distal pouch was reported in a patient with a history of FAP status post-IPAA. 45,47 Patients with lymphoma in the pouch have reported weight loss, abdominal or anal pain, urgency, and loose, watery, or bloody stools. [45][46][47] On pouchoscopy, lymphoma typically appears as a mass lesion in the distal pouch.…”

“…45,47 Patients with lymphoma in the pouch have reported weight loss, abdominal or anal pain, urgency, and loose, watery, or bloody stools. [45][46][47] On pouchoscopy, lymphoma typically appears as a mass lesion in the distal pouch. A biopsy of the lymphoma shows a monotonous proliferation of atypical large B cells.…”

“…There is no known established increased risk for lymphoma in patients with FAP; however, EBV-negative Burkitt lymphoma in the distal pouch was reported in a patient with a history of FAP status post-IPAA. 45,47…”

“…45 Other cases of patients treated with chemotherapy alone have reported remission, allowing the pouch to remain intact. 46,47…”