Cutaneous angiosarcoma usually occurs in the head and neck area of elderly patients and presents as an ill-defined and infiltrative ecchymotic patch or plaque. It may also develop in areas of chronic lymphedema or previous radiation therapy.

CHE has a tendency for local recurrence but low metastatic potential. There was no difference of biological behavior among cases with various combinations of histology. The presence of “angiosarcoma-like foci” and a prior history of long-standing lymphedema suggest that CHE may in fact be a low-grade angiosarcoma that behaves prognostically better than conventional angiosarcoma.

Total excision when feasible is considered the first-line treatment. Postoperative radiotherapy, preoperative or postoperative chemotherapy, and therapy with interferon alfa-2b have been used with variable results.