Introduction: Diffuse Large B cell lymphoma(DLBCL) is an aggressive subtype of Non Hodgkin's lymphomas and represent less than 0.5% of colorectal neoplasms. Extra nodal involvement of DLBCL in Gastrointestinal tract is common, but the primary DLBCL of Colon is a rare entity with only few cases reported in the literature. Here in, we present a rare case of Epstein Barr virus positive Primary DLBCL of ascending colon and cecum.
Case Description/Methods: A 53-year-old African American female with a past medical history of well controlled HIV presented to the emergency department with complaints of diffuse abdominal pain with bloody stools, fever , night sweats, and weight loss. Vitals were stable. Physical examination revealed right inguinal lymphadenopathy and diffuse abdominal tenderness. Laboratory studies showed elevated lactate dehydrogenase (1883). CT scan of the abdomen and pelvis showed diffuse retroperitoneal and pelvic lymphadenopathy and a large eccentric mass measuring 5.4 x 2.8 cm in the right hemicolon. Excisional biopsy of the right inguinal lymph node showed atypical clonal B-cell proliferation. Colonoscopy revealed multiple areas of villous and sessile non-obstructing polypoid mass in the ascending colon ( figure 1), which was resected and multiple areas of erythema in the cecum. Epstein Bar-virus encoded small RNA (EBER) stain was positive. Biopsy of the cecum and ascending colon showed atypical lymphocytic infiltrate. Immunohistochemical stains showed the atypical lymphocytes that were positive for CD20, PAX-5, CD10, BCL-6, C-MYC, and negative for BCL-2 with high proliferation of Ki-67. Based on these findings, a diagnosis of Stage IV, DLBCL was confirmed. She was initiated on Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, and Prednisone (R-CHOP) chemotherapy with good response.
Discussion: Management of DLBCL usually includes chemotherapy, Surgery, Radiotherapy or Combination therapy. However, the role of surgery is debatable. Cai etal reported the site dependent efficacy of surgery and improved overall survival with surgical intervention in right sided primary colonic lymphomas when compared to left side and rectum. The gold standard treatment of DLBCL has always been chemotherapy. R-CHOP regimen has showed efficacy in survival benefit. Several other combination therapies and targeted therapies are under investigation. Primary DLBCL of the colon can present with non-specific site dependent symptoms. Early diagnosis and immediate intervention can greatly improve the survival.
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