Extranodal marginal zone lymphoma of mucosa associated lymphoid tissue: a rare cause of acute abdomen

Abstract

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a low-grade B-cell lymphoproliferative disorder that most commonly involves the stomach. Involvement of the small intestine is rare, accounting for a small proportion of gastrointestinal cases, and clinical presentation is often nonspecific. Acute abdomen resulting from intestinal perforation is an exceptional manifestation. Case presentation: We report the case of a 33-year-old male with no significant past medical history who presented with a two-week history of progressive diffuse abdominal pain, fever, and anorexia. Physical examination revealed signs of peritonitis and a palpable inflammatory mass in the right lower quadrant. Given the clinical diagnosis of acute abdomen, an emergency exploratory laparotomy was performed, revealing a small bowel inflammatory mass with intestinal perforation and purulent peritonitis, located approximately 50 cm from the ileocecal valve. Segmental small bowel resection with primary anastomosis and appendectomy were performed. Histopathological and immunohistochemical analysis of the resected specimen confirmed extranodal marginal zone lymphoma of MALT type. The postoperative course was uneventful, and the patient was discharged without complications. Small intestinal extranodal marginal zone lymphoma is a rare entity with an indolent course and nonspecific clinical manifestations, which may delay diagnosis. In exceptional cases, it may present as an acute abdomen due to complications such as intestinal perforation, requiring urgent surgical intervention. This case underscores the importance of maintaining a high index of suspicion and highlights the role of a multidisciplinary approach in diagnosis and management.