Successful endoscopic diagnosis of angiosarcoma of the small intestine: A case report.

A 51-year-old man had hematochezia, anemia, and an intraabdominal mass. Gastroscopy and colonoscopy showed no significant lesions with intraluminal bleeding, while radiological examinations showed bulky swelling of the lymph nodes around the abdominal aorta and a tumor in the left ischial bone. Small intestine endoscopy detected a dark purpuric protruding tumor of the jejunum and its biopsy specimen brought a definitive diagnosis of primary jejunal epithelioid angiosarcoma from positive staining of AE1/AE3, CD31, and erythroblast transformation specific related gene in immunohistochemical studies. The patient underwent surgical resection with adjuvant chemotherapy but died of progression of metastases 7 months after the diagnosis. Epithelioid angiosarcoma of the gastrointestinal tract is an extremely rare malignancy with poor prognosis and it is challenging to distinguish from undifferentiated carcinoma or melanoma. Immunohistochemistry is necessary for a definitive diagnosis. Sufficient biopsy specimen may aid a prompt diagnosis of this disease of the small intestine, which may present as obscure gastrointestinal bleeding.