How common is hemangioendothelioma?

Hemangioendotheliomas are very rare. The National Cancer Institute reported that only 21 people in the United States were diagnosed with hemangioendothelioma from 2003 to 2007.

Last updated on 05-01-20

What is a hemangioendothelioma?

The term hemangioendothelioma describes several types of vascular neosplasms and includes both non-cancerous (benign) and cancerous (malignant) growths. The term has also been applied to those that show "borderline" behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Hemangioendotheliomas are caused by abnormal growth of blood vessel cells, although the exact underlying cause for the abnormal growth is unknown. They can also develop in an organ, such as the liver or lung. They usually grow slowly and can sometimes spread to other tissues in the body (metastasize). Examples of types of hemangioendotheliomas include spindle cell hemangioma; papillary intralymphatic (Dabska tumor); retiform; kaposiform; epithelioid; pseudomyogenic (epithelioid sarcoma-like hemangioendothelioma); and composite. Treatment depends on the type of hemangioendothelioma present but typically includes surgical excision (removal).

Last updated on 05-01-20

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. Likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. 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.

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%.

Last updated on 05-01-20

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.

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.

Last updated on 05-01-20

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