What are the other Names for this Condition? (Also known as/Synonyms)

• Haemangioendothelioma

What is Hemangioendothelioma? (Definition/Background Information)

• The term Hemangioendothelioma describes several types of vascular neoplasms 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)

(Source: Hemangioendothelioma; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Hemangioendothelioma? (Age and Sex Distribution)

• Hemangioendothelioma is a tumor that may be diagnosed in both children and adults
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Hemangioendothelioma? (Predisposing Factors)

• The risk factors depend on the specific type and location of the Hemangioendothelioma
• In some cases, no clearly-established risk factors are noted

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hemangioendothelioma? (Etiology)

• The exact cause of Hemangioendothelioma development is unknown; they are thought to occur spontaneously
• It is proposed that tumor formation may be related to abnormal blood vessel proliferations originating from the veins

What are the Signs and Symptoms of Hemangioendothelioma?

The signs and symptoms of Hemangioendothelioma depend on the size and location of the tumor. It also depends on the type of tumor - whether benign, malignant, or having a borderline behavior. The signs and symptoms include:

• Most tumors are asymptomatic, while they are small in size; however, in others, a wide-range of behavior is observed 
• They may grow at a slow rate and appear as painless, irregular/nodular masses below the skin surface (subcutaneous). They may also be present as deep-seated tumors
• They may be present as well-defined, single or multiple nodules at different locations
• Some are locally-aggressive and may damage the surrounding tissues
• Most common locations include the liver, lungs, bones, feet, and skin. Other locations include the spine, ribs, and leg

How is Hemangioendothelioma Diagnosed?

Hemangioendothelioma is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hemangioendothelioma?

The complications of Hemangioendothelioma may include:

• Spread of tumor to other body sites (metastasis)
• Large-sized tumors causing severe pain and organ dysfunction
• Tumor recurrence following its surgical removal

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Hemangioendothelioma 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 these tumors

(Source: Hemangioendothelioma; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Hemangioendothelioma be Prevented?

• Current medical research have not established a method of preventing Hemangioendothelioma formation
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are mandatory for those who have already endured the tumor, due to its metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Hemangioendothelioma? (Outcomes/Resolutions)

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%

(Source: Hemangioendothelioma; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Additional and Relevant Useful Information for Hemangioendothelioma:

The following DoveMed website links are useful resources for additional information:

https://www.dovemed.com/diseases-conditions/epithelioid-hemangioendothelioma-ehe/

https://www.dovemed.com/diseases-conditions/retiform-hemangioendothelioma/

https://www.dovemed.com/diseases-conditions/composite-hemangioendothelioma/

https://www.dovemed.com/diseases-conditions/pseudomyogenic-hemangioendothelioma/

https://www.dovemed.com/diseases-conditions/kaposiform-hemangioendothelioma-khe/

https://www.dovemed.com/article-synonyms/malignant-hemangioendothelioma/