Primary Small Bowel Diffuse Large B-Cell Lymphoma: Excellent Endoscopic Appearance

Abstract

Primary gastrointestinal tract lymphoma is a rare entity and its occurrence primarily in the small intestine is even more uncommon. A 37-year-old female presented with abdominal pain ongoing for 3 months associated with nausea and vomiting. The pain was diffuse, radiating to back and it limited her oral intake. She endorsed night sweats and chills but did not report exposure to any sick contacts. She was HIV positive (CD4 count: 46 cells/mm3). CT abdomen and pelvis showed diffuse irregular small bowel wall thickening and submucosal edema, retroperitoneal and diffuse mesenteric lymphadenopathy. Endoscopy revealed multiple hard, friable nodules ranging from 5mm to 2 cm starting at the second portion of the duodenum and extending into the visualized jejunum (Image 1 and 2). Pathology confirmed the diagnosis of DLBCL (Image 3). Bone Marrow biopsy showed no evidence of lymphoma. The patient was started on chemotherapy with dose-adjusted rituximab with etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin. Primary gastrointestinal lymphomas are quite uncommon and constitute 5-10% of all gastrointestinal tumors. [1] In the small intestine, most commonly affected region is ileum followed by jejunum, duodenum (6-8%) and then other sites. [2] HIV, is a known risk factor for lymphoma [3]. With the involvement of small intestine, the symptoms are quite non-specific. [1] Diagnosis is made via biopsy. Endoscopy is used to identify suspicious lesions preceded by radiographical evaluation. In some rare cases, it can present as multiple lymphomatous polyposis (MLP). [3] No guidelines exist for treatment of DLBCL of the small intestine. Historically, in HIV patients chemotherapy combined with ART remains the first step in the management of aggressive lymphomas. [4] Radiotherapy, as a mono treatment modality, has not been shown to be beneficial in DLBCL of the small intestine. [1,2]2555_A Figure 1. Nodules seen endoscopically in the duodenum.2555_B Figure 2. Friable nodules seen in the small bowel2555_C Figure 3. Malignant lymphocytes with moderately abundant cytoplasm, and the nuclei are round to ovoid with prominent nucleoli and occasional mitoses. H&E Stain, 400x