Multifocal epithelioid angiosarcoma of the small intestine

A 67-year-old man presented with weight loss, intermittent severe abdominal pain and melaena. Initial radiology (including abdominal ultrasonography), gastroscopy and colonoscopy did not demonstrate any lesions that could exp lain the complaints. Three weeks later, upper gastrointestinal and small-bo wel barium studies revealed two areas in the small intestine with an abnorm al mucosal pattern. Explorative laparotomy revealed three tumoral lesions. Three partial enterectomies were performed. Gross examination showed centra lly depressed dark reddish tumoral lesions extending from the mucosa throug hout the full thickness of the bowel wall (diameter varying between 1.6 cm and 2.2 cm). The tumours, composed of large, plump, polygonal cells showing little architectural differentiation, were mainly situated in submucosa an d muscularis propria. The growth pattern appeared rather solid. The epithel ioid cells showed pronounced nuclear pleomorphism and atypia with central l arge nucleoli. There were several small blood vessels with occasional anapl astic endothelial cells. Immunohistochemical staining demonstrated an inten se expression of CD 31, CD 34, factor VIII related antigen and keratin. Thi s supported the diagnosis of an epithelioid angiosarcoma. The patient died 3 months after diagnosis. Tumours of the small intestine are very rare, and angiosarcomas of the small intestine are even more rare. Epithelioid varia nts have only been described in two patients and only one of these had a mu ltifocal presentation. The prognosis is very poor. Because of the epithelio id growth pattern and the cytokeratin expression, these tumours may erroneo usly be diagnosed as a carcinoma.