Intussussception and Ileal Ulceration: A Case of Recurrent Near-Tetraploid Diffuse Large B-Cell Lymphoma of the Small Intestine

Abstract

Diffuse Large B-Cell Lymphoma (DLBCL) presents extranodally in nearly 40% of cases. In patients with polyploidy, however, extranodal presentation is far less common. To date, no primary gastrointestinal cases of polyploid DLBCL have been reported. We present a case of DLBCL with polyploidy diagnosed as intussusception in the terminal ileum with recurrence as an ileal ulcer. A 71-year-old man with history of an inguinal hernia repair and empyema presented to our hospital with two weeks of back pain with periumbilical radiation, non-bilious emesis, inability to tolerate food by mouth, ten pound weight loss, and decreased stool caliber. His last colonoscopy over ten years ago was normal. CT imaging revealed a small bowel obstruction with circumferential wall thickening at the terminal ileum causing 4-cm of intussusception (Figure 1). He underwent exploratory laparotomy and right hemicolectomy with side-to-side ileocolic anastomosis. Pathology revealed a non-germinal center DLBCL with a near-tetraploid karyotype. Resection margins were negative. After positron-emission tomography CT (PET-CT) revealed no evidence of disease, he received six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. Following treatment, repeat PET-CT revealed normal bowel with adjacent newly avid peritoneal and mesenteric nodules (Figure 2). Given radiographic recurrence, he was treated on a clinical trial with a combination of ibrutinib, lenalidomide and rituximab. During his first cycle, he re-presented with anemia and dark, guaiac-positive stool. Although EGD was unremarkable, colonoscopy revealed a solitary, non-bleeding, 10-mm ulcerated polyp in the neo-terminal ileum with surrounding healthy mucosa proximal to the site of the prior end-to-end ileocolic anastomosis (Figure 3). Pathology revealed recurrent DLBCL and imaging confirmed progressive disease. Treatment with salvage chemotherapy was ineffective. He was transitioned to hospice and died two weeks later. To our knowledge, this is the first reported case of gastrointestinal DLBCL with polyploidy. In addition to reduced infiltration of extranodal sites, these karyotypes carry an unfavorable prognosis and poor overall survival. Early identification and attention to extranodal disease as well as prompt initiation of therapy are imperative to optimize any potential treatment response in these patients.2594_A Figure 1. Coronal CT of Intussusception.2594_B Figure 2. Coronal PET-CT of Recurrent Abdominal Lymphoma.2594_C Figure 3. Endoscopic View of Ileal Ulceration.