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Genetic and Rare Diseases Information Center (GARD)


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Your Question

I have been told that surgery is really the only treatment for hemangioendothelioma; that chemo isn't used and that radiation only helps with the pain rather than shrink the tumors. Is this true? Also, what is the mortality rate or life expectancy for those with this type of cancer?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might hemangioendothelioma be treated?

Treatment for hemangioendothelioma may depend on the type of hemangioendothelioma present in the affected individual and the risk of recurrence or metastases. In most reported cases, surgical excision (removal) of the mass has been the only treatment. For spindle cell hemangioma, simple excision is reportedly curative; however, new growths develop in adjacent skin and soft tissues in 60% of affected individuals. For individuals with papillary intralymphatic angioendothelioma (PILA), excision of the involved lymph nodes, as well as the mass, has been recommended. Surgical excision is reportedly also the usual treatment for individuals with retiform hemangioendothelioma (although local recurrence with this type is common), epithelioid hemangioendothelioma, and composite hemangioendothelioma (with the exception of 1 case treated with interferon).

Most individuals with pseudomyogenic hemangioendothelioma have been treated with simple excision, but a few individuals have also received post-surgical radiotherapy (RT).

With regard to kaposiform hemangioendothelioma, some large lesions cannot be completely removed and may cause fatal complications due to the associated Kasabach–Merritt syndrome. In these cases, several medical therapies have been used, including systemic corticosteroids; alfa interferon; RT; embolization; and several other therapies, both alone and in various combinations.[1]

A study by Scott et al published in 2012 in the American Journal of Clinical Oncology evaluated the effectiveness of RT as either an alternative or adjunct to surgery. The authors stated that the effectiveness of definitive RT in the treatment of hemangioendothelioma in their study implies that radiation may be an acceptable alternative when surgical resection will compromise function or cosmetic result. They concluded that with no local recurrences and minimal risk of toxicity, their long-term data suggest that RT offers a highly effective management option for this disease.[2]
Last updated: 7/10/2013

What is the long-term outlook for individuals with hemangioendothelioma?

The long-term outlook (prognosis) for individuals with hemangioendothelioma is not predictable and differs among affected individuals. The prognosis in each case depends on the type of hemangioendothelioma present, the risk of recurrence and whether the condition has metastasized (spread) to nearby tissues or other parts of the body. For example, although surgical removal of lesions of spindle cell hemangioendothelioma is considered curative, new masses develop in adjacent skin and soft tissues in 60% of the cases. However, among the approximately 200 cases of spindle cell hemangioma described in the literature, no patient has developed regional or distant metastases.[1] Likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare.[1] On the other hand, a follow-up study of 30 patients with cutaneous epithelioid hemangioendothelioma showed that 21% of the affected individuals developed systemic metastases, and 17% of these patients died as a consequence.[1]

Survival rates in the literature for individuals with hemangioendothelioma generally range from 62% to 83% and local control rates (stopping cancer growth at the site of origin) range from 80% to 100%.[2]
Last updated: 7/10/2013

  • Requena L, Kutzner H. Hemangioendothelioma. Semin. Diagn. Pathol. February 2013; 30(1):29-44.
  • Michael T. Scott et al. Radiation Therapy for Hemangioendothelioma: The University of Florida Experience. American Journal of Clinical Oncology. 2012;
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