Cutaneous angiosarcoma metastatic to small bowel with nodal involvement

A 77-year-old male with a history of metastatic scalp angiosarcoma presented with intractable gastrointestinal bleeding from a jejunal mass detected on capsule endoscopy. He underwent laparoscopic-assisted resection of the mass. Intraoperatively, an isolated small bowel mass with bulky lymphadenopathy was seen and resected en bloc. Pathology showed a 6.8cm high-grade metastatic angiosarcoma with nodal involvement and negative margins. Angiosarcoma is a sarcoma with a grim prognosis. The incidence is 2% of all soft tissue sarcomas; cutaneous lesions comprise 27% of manifestations and usually appear on head and neck. Risk factors include lymphedema, neurofibromatosis, vinyl chloride, arsenic, and anabolic steroids. Overall 5-year survival is 30-35% and is higher in patients younger than 60, those without metastasis, tumors less than 5 cm, and favorable histology. Angiosarcoma metastasis to small bowel is rare but nodal involvement is even more unusual, reported only three times in the literature. This case is the first with nodal involvement to present at a resectable stage. To diagnose disease when still at a resectable stage, a high index of suspicion must be maintained with any gastrointestinal symptoms in a patient with a history of angiosarcoma. Laparoscopic-assisted resection is safe for the resection of small bowel angiosarcoma.