Lymphoma of the Terminal Ileum Presenting as Intussusception and Hematochezia

Abstract

An 8 year old boy presented with a six day history of hematochezia, diarrhea, and sharp, non-radiating periumbilical pain with intermittent nausea and vomiting. He had a 22 pound weight loss over the past month and denied any travel history. Past medical history and family history were unremarkable. He had an uneventful full-term vaginal delivery with normal developmental milestones. Upon admission, he was afebrile, BP 90/60, P 100. Physical exam revealed pallor. Abdomen was soft, nondistended, mild tenderness in the RLQ, hyperactive bowel sounds. Hgb 9.0 g/dL (nml 14.3-16.0), MCV 76 (nml 80-99), all other labs were normal. EGD showed hiatal hernia and mild esophagitis. CTscan showed a large soft tissue density mass that appeared intraluminal in the ascending colon and hepatic flexure (Fig 1). Colonoscopy revealed a friable ulcerated polypoid lesion protuding out of the ileocecal orifice into the cecum (Fig 2). The patient was then taken to the operating room for exploratory laparotomy, and had an ileocecal resection with primary end to end anastomosis. Pathology revealed malignant lymphoma of the ileum (high-grade, noncleaved, diffuse, large-B cell type) invading through the muscularis propria into the subserosal fat with intussusception of the ileum into the colon (Fig 3). Subsequent work-up revealed a normal bone scan, CT chest, and normal bone marrow biopsy making this a Stage II malignant lymphoma of the ileum. The patient tolerated intrathecal Methotrexate and Cyclophosphamide plus prednisone, and has since been in complete clinical remission 10 years after treatment. Discussion: Gastrointestinal lymphoma accounts for 4-12% of all non-Hodgekin's lymphomas. Primary ileocecal lymphoma is uncommon and thus poorly studied. Patients may present with abdominal pain, altered bowel habits, weight loss, abdominal mass, and/or hematochezia. Approximately, 75% of all cases of intussusception occur in children under 1 year of age, and almost 90% of cases are idiopathic. This is in contrast to the adult population where 80% of intussusceptions are neoplastic. Malignant lymphoma in the terminal ileum tends to cause intussusception because of the polypoid type of lesion with little attachment to the surroundings, and minimal disruption of the muscularis propria. Our patient was unusual presenting in the pediatric age group with a neoplastic cause of intestinal intussusception, in this case a malignant lymphoma. It illustrates the importance of prompt recognition resulting in successful treatment and longterm remission.