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

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          Abstract

          Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of cutaneous lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible 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 well, 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 series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.

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          Author and article information

          Journal
          Mod. Pathol.
          Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
          Springer Nature
          0893-3952
          0893-3952
          Nov 2001
          : 14
          : 11
          Affiliations
          [1 ] Department of Anatomical & Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong, China SAR. fmlai@cuhk.edu.hk
          Article
          10.1038/modpathol.3880441
          11706068

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