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

• Composite HE

What is Composite Hemangioendothelioma? (Definition/Background Information)

• Composite Hemangioendothelioma (HE) is an extremely rare, aggressive tumor, involving blood vessel components. This tumor is considered to be a low-grade malignant proliferation. They are known to manifest during early-to-late adulthood
• It is commonly found on the hands and feet, with another reported site being the tongue. These tumors are normally located on the skin surface, or lie beneath the skin, in subcutaneous location
• Any combination of chemotherapy, radiation therapy, and invasive surgical procedures, are used to treat Composite Hemangioendothelioma. Though, the treatment of choice is a complete surgical removal
• The prognosis of the tumor depends on a variety of factors. However, the tumor is known to have a high recurrence rate

Who gets Composite Hemangioendothelioma? (Age and Sex Distribution)

• Composite Hemangioendothelioma is mostly observed in young, middle-aged, and older adults (between 20-75 years). Only one case has been reported as a congenital type of Composite Hemangioendothelioma
• The tumor is seen to affect both the male and female sexes uniformly
• There is no known ethnic or racial preference

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

Risk factors of Composite Hemangioendothelioma include:

• Composite Hemangioendothelioma is associated with chronic obstruction of the lymph vessels (lymphedema)
• Maffucci syndrome - a disease characterized by multiple hemangiomas and bone marrow cysts. Individuals with this condition have an increased risk of Composite HE

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Composite Hemangioendothelioma? (Etiology)

• The exact cause of Composite Hemangioendothelioma is unknown
• They are thought to occur spontaneously and are regarded as a kind of low-grade skin angiosarcoma (a malignant cancer of the blood vessels)
• It is suggested that Composite Hemangioendothelioma origin might be related to abnormal blood vessel proliferations, occurring due to unknown reasons

What are the Signs and Symptoms of Composite Hemangioendothelioma?

The signs and symptoms of Composite Hemangioendothelioma are based on the location of the tumor. These may include:

• Superficially located tumors might look like birthmarks; red-blue, violet-colored scars
• They grow at a slow rate (over many years) and appear as painful inflammatory nodular lesions. These lesions may cause reduced motion range and discomfort, if present around a joint
• Composite Hemangioendothelioma usually occur in the lower and upper limb extremities, specifically in the fingers and toes. Multiple lesions have also been observed in some individuals
• One published case report has indicated that Composite HE  involved the tongue

How is Composite Hemangioendothelioma Diagnosed?

A diagnosis of Composite Hemangioendothelioma may involve:

• A thorough physical examination, evaluation of patient’s medical history
• Tissue biopsy of mass: Histopathological studies conducted on a biopsy specimen. The tissue biopsy specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
• MRI scan of the affected region may be performed to aid in surgery

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 Composite Hemangioendothelioma?

The complications of Composite Hemangioendothelioma may include:

• Composite Hemangioendothelioma are known to having a high recurrence rate, after surgical excision
• Damage of vital nerves, blood vessels, and surrounding structures during surgery
• There has been one reported case of limb amputation; the amputation procedure was used as a treatment method for Composite HE
• Complications due to associated vascular disorders, like lymphedema

How is Composite Hemangioendothelioma Treated?

Treatment for Composite Hemangioendothelioma includes the following:

• Any combination of chemotherapy, radiation therapy, and invasive surgical procedures are used to treat Composite Hemangioendothelioma
• Wide surgical excision with removal of the entire lesion is the standard treatment mode; if Composite HE is not fully removed, then it may recur
• Arterial embolization of the tumor is used to provide temporary relief from the symptoms and reduce blood loss during ‘tumor removal’ surgical procedure; especially, if the tumors are large
• When the lesions are at inaccessible locations, or it is unsafe for surgical intervention, non-invasive procedures are adopted
• Interferon injections are used to reduce tumor blood supply and limit their growth
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Composite Hemangioendothelioma be Prevented?

• Current medical research have not established a way of preventing Composite Hemangioendotheliomas
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and high chances of recurrence

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

• Composite Hemangioendotheliomas are extremely rare tumors and not yet completely understood. However, no fatalities have been reported so far
• The long-term prognosis depends on a combination of factors, such as:
  • First appearance/detection of the tumor
  • Tumor size and location
  • Stage of the tumor
  • Its response to treatment and medical therapy
• Skin surface and subcutaneous tumors that are well-defined can be completely excised, with low chances of recurrence
• The recurrence period of Composite HE (based on a collection of medical records), has so far varied between 4-11 years; follow-up examinations are a must

Additional and Relevant Useful Information for Composite Hemangioendothelioma:

Composite Hemangioendothelioma anatomically resembles epithelioid hemangioendothelioma and retiform hemangioendothelioma, when examined under a microscope by a pathologist. Hence, additional tests may be performed to arrive at a definitive diagnosis.

The following article links will help you understand epithelioid hemangioendothelioma and retiform hemangioendothelioma tumors:

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

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