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

• ALHE (Angiolymphoid Hyperplasia with Eosinophilia)
• Histiocytoid Hemangioma
• Inflammatory Angiomatoid Nodule

What is Epithelioid Hemangioma? (Definition/Background Information)

• An Epithelioid Hemangioma (EH) is a rare, benign tumor involving the blood vessels and surrounding epithelioid cells. The blood vessels are lined by epithelioid cells, which grow abnormally to form the tumor
• EH frequently affects the head and scalp region. Other affected areas include the wrists and fingers. Individuals between the age 20-50 years are most commonly affected; nevertheless, it can occur in any age group
• An individual may experience a painful nodular mass at the site. In others, the tumor may be asymptomatic, causing no signs or symptoms
• The diagnosis of Epithelioid Hemangioma is confirmed through a tissue biopsy.
• The treatment is a complete surgical removal; the prognosis of Epithelioid Hemangioma is excellent, with suitable treatment
• Currently, no known methods exist to prevent Epithelioid Hemangioma occurrence

Who gets Epithelioid Hemangioma? (Age and Sex Distribution)

• Epithelioid Hemangioma may occur in individuals of all age group. However, the peak age range is the mid-adult period, between 20-50 years
• Females are more often affected by EH, than males
• There is no predilection to any ethnic group or a particular race

What are the Risk Factors for Epithelioid Hemangioma? (Predisposing Factors)

Risk factors are not well-established for Epithelioid Hemangioma development, but the following are suggested:

• Any developmental tissue abnormality involving the soft tissues (hamartomas) may be a risk factor
• Epithelioid Hemangiomas may occur spontaneously, due to an injury or a trauma
• Some EHs are seen to accompany HIV infection, pregnancy, and a regular use of oral contraceptives

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 Epithelioid Hemangioma? (Etiology)

• The exact cause of Epithelioid Hemangioma formation is unknown
• They are thought to occur spontaneously, or as a reaction to any injury/trauma. This may lead to abnormal blood vessel proliferations, generally around the bones and in tissues beneath the skin
• The hormones, estrogen and progesterone, are said to be associated with the formation of EH during pregnancy, and due to long-standing hormonal contraceptive use

What are the Signs and Symptoms of Epithelioid Hemangioma?

Signs and symptoms of Epithelioid Hemangioma include:

• The tumor grows at a slow rate and may appear as a painful mass
• They may be present as well-defined, single or multiple nodules
• Most common locations the tumors are found are the face, scalp, and neck. Other locations include the wrists and fingers
• Occasionally, these tumors may affect the bones. Involvement of the bone may result in bone pain, or an increased bone fracture risk at the tumor site

How is Epithelioid Hemangioma Diagnosed?

A diagnosis of Epithelioid Hemangioma is made using the following tools:

• Complete physical examination with a thorough evaluation of medical history
• X-ray studies of the affected region: Though, by use of this method, it is generally difficult to distinguish between other kinds of hemangiomas, such as telangiectatic osteosarcoma, aneurysmal bone cyst, fibrous dysplasia, etc.
• CT, MRI scan of the affected region
• A tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together the clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be the gold standard, in arriving at a conclusive diagnosis
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely, electron microscopic studies
• Differential diagnosis, to eliminate other tumor types are often considered, before arriving at a definitive diagnosis

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 Epithelioid Hemangioma?

The complications from Epithelioid Hemangioma could include:

• Recurrence of Epithelioid Hemangioma after surgery
• Blood loss during invasive treatment methods may be heavy; blood may accumulate outside the blood vessels, causing hematomas
• Severe pain at the tumor site
• Damage to the muscles, vital nerves, and blood vessels, during surgery

How is Epithelioid Hemangioma Treated?

Treatment measures for Epithelioid Hemangioma include the following:

• Wide surgical excision of Epithelioid Hemangioma with removal of the entire lesion is the preferred treatment method. If the tumor is not fully removed, then it might recur
• Bone tumors may require the use of bone grafts
• Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• When EH is at an inaccessible location or is unsafe for surgical intervention; radiation therapy, cryotherapy, or sclerotherapy is employed. This also reduces the need for post-surgical reconstruction and cosmetic procedures, particularly if the tumors are on the face and neck region
• 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 Epithelioid Hemangioma be Prevented?

• Current medical research has not established a way of preventing Epithelioid Hemangioma occurrence
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are mandatory for those, who have already endured EH, due to its chances of recurrence

What is the Prognosis of Epithelioid Hemangioma? (Outcomes/Resolutions)

• The prognosis of Epithelioid Hemangioma is generally good, when the lesions are small and found below the skin surface (subcutaneous tissue). In such cases, their recurrence risk (after surgery) is also very low
• The probability of Epithelioid Hemangioma recurrence for a large-sized tumor is higher at 30%, after surgical removal of the tumor. Hence, a regular follow-up with medical screening should be ensured
• The prognosis of EH with bone involvement varies; it is dependent upon each individual case

Additional and Relevant Useful Information for Epithelioid Hemangioma:

Epithelioid Hemangiomas have strong clinical and histopathological resemblance to certain features of Kimura’s disease. Hence, occasionally EH may be misdiagnosed as Kimura’s disease.