Kaposiform Hemangioendothelioma of the GI Tract: AnException to Occam’s Principle in an Adult with SBO

Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive vascular tumor with histological features resemblingKaposi sarcoma and capillary hemangioma mainly occurring in children and adolescents. Approximately 200 cases have beenreported since its original description in 1993, with the vast majority presenting at an early age as raised ill-defined lesions witha red-blue hue mainly involving the skin and soft tissues in the extremities. Cases in adults remain extremely rare. Herein, wedescribe the case of a 29 year-old man who presented with progressive abdominal pain for 4 months and signs of obstipationfound to be consistent with small bowel volvulus. The patient underwent exploratory laparotomy and resection of 55 cm ofnecrotic small bowel followed by enteroenterostomy and anastomosis. Microscopic examination revealed KHE involving smallintestinal mesentery, muscularis propria, and submucosa. His recovery was uneventful and he was discharged after stabilization,opting to manage him expectantly with abdominopelvic imaging and to monitor for development of Kasabach-Merrittphenomenon. To our knowledge, this represents thefirst reported case of this entity presenting as intestinal obstruction in anadult for which we also present a review of the existing literature and possible treatment options.