Abstract
A 30-year-old woman developed cutaneous proliferating angioendotheliomatosis without endocarditis. She was treated with local excision and radiotherapy 42 months ago. There is no recurrence eight years after clinical onset of the lesion, making this one of the longest documented cases of survival. A literature review of the disease, including its controversial histiogenesis, treatment, and survival, is presented. In view of the rarity of the disease, only by studying more patients can we better understand the disease. Since more than one disease process may be included under this diagnosis, clearer clinical and histogenetic separation is required, with immunohistochemical techniques potentially helping to provide a more precise diagnosis.