Kaposiform hemangioendothelioma: Five patients with cutaneous lesion and long follow-up

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infi ltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been report ed so far, and while many have died as a result of extensive disease and se vere coagulopathy, the long-term biologic behavior of this tumor remains un determined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report e mphasizes on the importance of cutaneous lesions being the most commonly af fected site, but also for its clinical diversity. Early diagnosis is possib le even for a small skin lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are wel l, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this seri es indicates that kaposiform hemangioendothelioma is incapable of metastasi s, despite a protracted course of many decades with no tendency for spontan eous regression.