Epithelioid angiosarcoma of the small bowel

Abstract

Background: Angiosarcoma is a rare soft-tissue neoplasm that occurs most often in the skin and the subcutaneous tissues but very rarely in the GI tract.

Design: We report a case of multifocal epithelioid angiosarcoma involving skin and bowel and review the 18 previously reported cases of epithelioid intestinal angiosarcoma.

Main outcome measurements: Our patient presented with weakness from anemia. Two small blue-black nodules were present on the skin. Fecal occult blood tests were positive, but gastroscopy and colonoscopy showed normal results. Enteroscopy revealed small ulcerated nodules in the distal duodenum and the proximal jejunum. Biopsy specimens of skin and duodenal nodules showed epithelioid angiosarcoma.

Results: The intestinal nodules were treated with argon plasma coagulation, but the patient died 6 weeks after diagnosis.

Conclusions: The survival rate of intestinal angiosarcoma is poor, and most cases progress rapidly, with a 2-month median survival after diagnosis. Because of the infiltrative and the multifocal nature of this malignancy, complete surgical excision is often not possible. Further studies are needed to establish the role of adjuvant radiation or chemotherapy in the treatment of angiosarcoma.