National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Hemangioendothelioma


Categories:
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.[1] 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).[2][1] Examples of types of hemangioendotheliomas include spindle cell hemangioma; papillary intralymphatic (Dabska tumor); retiform; kaposiform; epithelioid; pseudomyogenic (epithelioid sarcoma-like hemangioendothelioma); and composite.[1] Treatment depends on the type of hemangioendothelioma present but typically includes surgical excision (removal).
Last updated: 7/10/2013
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.[3]
Last updated: 7/10/2013
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%.[3]
Last updated: 7/10/2013

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Hemangioendothelioma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The American Cancer Society provides information on hemangioendothelioma in a fact sheet on soft-tissue sarcomas.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hemangioendothelioma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new 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? See answer

  • What is a hemangioendothelioma?  How common is it? See answer


  1. Requena L, Kutzner H. Hemangioendothelioma. Semin. Diagn. Pathol. February 2013; 30(1):29-44.
  2. Sarcoma - Adult Soft Tissue Cancer. American Cancer Society. July 6, 2010; http://www.cancer.org/Cancer/Sarcoma-AdultSoftTissueCancer/DetailedGuide/sarcoma-adult-soft-tissue-cancer-soft-tissue-sarcoma. Accessed 2/14/2011.
  3. Michael T. Scott et al. Radiation Therapy for Hemangioendothelioma: The University of Florida Experience. American Journal of Clinical Oncology. 2012;