Rare Case of Intestinal Lipomatosis Presenting With Hemorrhage

Abstract

Introduction Diffuse intestinal lipomatosis is a very rare disease characterized by multifocal infiltration of mature adipose tissue into the intestinal submucosal or subserosal layers. The disease is mostly asymptomatic but complications like intussusceptions, obstruction, bleeding or perforation have been reported. Gastrointestinal (GI) lipomas occur in colon, small bowel, stomach and esophagus in decreasing frequency. Lipomas >2cm size are more prone to having complications. We here in describe a very rare care of diffuse intestinal lipomatosis presenting with hemorrhage. Case Report A 59-year-old Caucasian woman with hypothyroidism presented with worsening shortness of breath and dark stools for few days. Physical exam was remarkable for mucosal pallor. Laboratory studies showed severe anemia with hemoglobin of 4.7 gm/dL. Diagnostic upper endoscopy showed a large friable mass with overlying small old clot in second portion of duodenum (D2) adjacent to the ampulla. There were multiple other submucosal mass lesions in the entire examined small bowel. Colonoscopy also showed multiple (˜20) submucosal lesions throughout the colon suggestive of likely diffuse intestinal lipomatosis. Endoscopic ultrasound (EUS) done for elucidation of nature of D2 lesion revealed an oval hyperechoic lesion measuring 39×24mm arising from the submucosal layer without involving muscularis propria. Fine needle biopsy of the lesion revealed adipocytes. Discussion Solitary lipomas of GI tract are common accounting up to 8% of all GI tumors. However GI lipomatosis is very rare with an incidence of 0.04%. It's etiopathogenesis is unclear. Anomalies in embryonic development, fat dysmetabolism, chronic inflammation, alcohol use and hamartomatous syndromes have been implicated. Ileum is the most common site of enteric lipomas (25% of all GI tract lipomas) with duodenum being least frequent (4%). Endoscopic signs suggestive of lipomas include: tenting sign, cushion sign and the ‘naked fat’ sign (fat protruding from the biopsy site). A lipoma rarely can present with overt GI bleeding from apical ulceration. Ulceration results from pressure atrophy of mucosa and necrosis of the overlying epithelial layers due to abnormal peristalsis. Ulceration can be extensive with profuse bleeding requiring aggressive endoscopic, radiological or surgical management. Endoscopic modalities of managment include snare / endoloop resection and submucosal dissection.1870_A Figure 1. Multiple rounded submucosal lesions in the small bowel with positive cushion sign consistent with lipomas (A); large submucosal mass lesion in D2 adjacent to the ampulla (B).1870_B Figure 2. EUS showing an oval hyperechoic, heterogenous lesion measuring arising from the submucosal layer1870_C Figure 3. Fine needle biopsy showing adipocytes and mucosal epithelial cells