Rosai-Dorfman Disease of the Stomach

Purpose: Rosai-Dorfman disease (RDD) is a rare condition of unknown etiology that typically manifests as lymphadenopathy with potential extranodal involvement. Gastrointestinal disease is very uncommon, with only fifteen cases reported to date, of which eight cases involving the colon. We present the first case of extranodal RDD involving the stomach, in a 27-year-old African American female with known colonic disease. The patient was diagnosed with RDD of the colon and pelvis eight years prior to current presentation, and since then underwent multiple abdominal surgeries, including mass debulking, nephrectomy, small bowel resection with colostomy, and adhesiolysis. Her disease course has been highly debilitating and resulted in multiple ER visits and hospital admissions. The patient presented to the ER with diffuse abdominal pain and bleeding into her colostomy bag for four days. The blood was described as dark in color. She also complained of nausea and non-bloody emesis. Her vital signs were stable, and abdominal exam revealed diminished bowel sounds with diffuse tenderness but no peritoneal signs. Laboratory studies showed baseline anemia, and abdominal series demonstrated non-specific air-fluid levels in the colon. She received an enema through her colostomy for presumed fecal impaction which led to a resolution of her symptoms. Prior to discharge she underwent an esophagogastroduodenoscopy that showed a 2 cm mass in the proximal body of the stomach without evidence of bleeding. Biopsies were taken and demonstrated gastric mucosa with a patchy lymphohistiocytic infiltrate. Morphologically, the histiocytes were large and vacuolated with evidence of emperipolesis. Immunohistochemically, the cells were positive for S100 and CD68 stains, and negative for CD1a, supporting the diagnosis of extranodal RDD of the stomach. As the gastric mass was neither causing any symptoms nor was it bleeding, and in view of the patient's complicated past surgical history, she is currently under close observation with repeated upper endoscopies.Figure: Esophagogastroduodenoscopy demonstrating a submucosal mass in the corpus of the stomach.