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

• Epithelioid Sarcoma-Like Hemangioendothelioma
• Fibroma-Like Variant of Epithelioid Sarcoma
• Pseudomyogenic Haemangioendothelioma

What is Pseudomyogenic Hemangioendothelioma? (Definition/Background Information)

• Pseudomyogenic Hemangioendothelioma (PMH) is a very uncommon tumor of the soft tissues that are potentially malignant; they are known to rarely metastasize. It is also known as Epithelioid Sarcoma-Like Hemangioendothelioma
• Most of these tumors are observed in young adult males, though individuals of a wide age category may be affected. Currently, the cause and risk factors for the formation of Pseudomyogenic Hemangioendothelioma is unknown
• The tumor may be single or present as multiple nodules at various locations in the body. The most common tumor sites are the lower limbs and upper limbs
• Pseudomyogenic Hemangioendothelioma may occur on the skin surface, in the subcutaneous tissue, or may be present deep in the body tissues. The tumors may or may not present pain
• The treatment of choice is a complete surgical removal through simple excision. However, the presence of multiple tumors at various sites in the body may necessitate additional treatment measures
• The prognosis of Pseudomyogenic Hemangioendothelioma is based on several factors that include the size, location, and numbers of the tumor. Small-sized tumors at easily accessible locations that can be easily removed generally have a better prognosis than large-sized tumors at inaccessible body sites

Who gets Pseudomyogenic Hemangioendothelioma? (Age and Sex Distribution)

• Pseudomyogenic Hemangioendothelioma is an extremely rare tumor with only about 60 cases being reported in the medical literature
• It can occur in individuals of all age group; however, a majority of the tumors are observed in young adults (average age of 30 years)
• Middle-aged and older adults are less commonly affected; only 1 in 5 cases are seen in those over 40 years
• The tumor is present in both gender (male and female), although 80% of the cases are seen in males; thus, a strong male preference is observed
• There is no predilection to any ethnic group or a particular race

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

• Presently, no risk factors are defined or suggested for Pseudomyogenic Hemangioendothelioma

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 Pseudomyogenic Hemangioendothelioma? (Etiology)

The exact cause of Pseudomyogenic Hemangioendothelioma development is unknown. These tumors are thought to occur spontaneously.

What are the Signs and Symptoms of Pseudomyogenic Hemangioendothelioma?

The presentations are based on the location of the tumor. Pseudomyogenic Hemangioendothelioma may occur at several locations in the body. The signs and symptoms of the tumor may include:

• The most common location of the tumor is the leg followed by the arm (together accounting for nearly 80% of the cases)
• Other body sites include the chest and back (trunk); sometimes, the face and scalp, and very rarely, the spine or bone may be involved
• Most small-sized tumors are asymptomatic; however, in others, a wide-range of behavior is observed. Tumors located deep in the body may be difficult to diagnose due to absence of any significant signs and symptoms
• The tumors may grow at a slow rate and appear as painless, nodular masses on or below the skin surface (subcutaneous), with poorly-defined boundaries. Some may also be present as deep-seated tumors
• Studies indicate that 1 in 2 tumors are found in the muscles and 1 in 5 tumors may involve the bone. Also, 50% of the individuals with the tumor may experience pain at the tumor site
• 35% of the tumors are solitary in nature, while around 65% of the tumors are multi-centric. This means that they occur at several body locations simultaneously. Of these, about 50% of the tumors were seen to affect several tissue layers
• The size of the PMH may range from 1 cm to 5.5 cm, with an average size of 1.9 cm. Most tumors are less than 3 cm in size; 10% of the tumors are found to be larger than 3 cm

How is Pseudomyogenic Hemangioendothelioma Diagnosed?

A diagnosis of Pseudomyogenic Hemangioendothelioma is made using the following tools:

• Complete physical examination with a thorough evaluation of medical history
• X-ray studies of the affected region
• 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
• Differential diagnosis to eliminate other tumor types is 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 Pseudomyogenic Hemangioendothelioma?

The complications of Pseudomyogenic Hemangioendothelioma are dependent on the site and size of the tumor. These may include:

• Severe pain, if internal organs are affected
• Organ dysfunction due to tumor growth and invasion
• Recurrence of PMH after surgery; nearly 2 in 3 individuals may experience local recurrence. Multiple recurrences are not uncommon
• Sometimes, additional nodules are seen to form and develop at the original tumor site
• In very rare cases, tumor metastasis to various body sites including to the lungs, bones, and to other soft tissues of the body is noted. Involvement of the lymph nodes is rarely observed
• Damage to vital nerves, blood vessels, and surrounding structures, during surgery
• Side effects from chemotherapy (such as toxicity), radiation therapy

How is Pseudomyogenic Hemangioendothelioma Treated?

The treatment measures for Pseudomyogenic Hemangioendothelioma include the following:

• Wide surgical excision of Pseudomyogenic Hemangioendothelioma with removal of the entire tumor is adopted, if necessary. If it is not fully removed, then it might recur
• Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• When the tumors are at inaccessible locations, or are unsafe for surgical interventions; radiation therapy, chemotherapy, or oral medications may be considered
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Long-term follow-up care with regular screening and check-ups are very important

How can Pseudomyogenic Hemangioendothelioma be Prevented?

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

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

The prognosis of Pseudomyogenic Hemangioendothelioma, which is an extremely rare tumor, depends upon a set of several factors, which include:

• Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
• Location of tumor: Tumors at locations with potential to damage vital organs (such as tumors in the head and neck region) have poor prognosis than tumors that are located in the extremities (arms or legs)
• The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
• Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
• Age of the individual: Older individuals generally have poorer prognosis than younger individuals
• Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
• Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment

Pseudomyogenic Hemangioendothelioma recurrence is observed in 60% of the cases, usually within 18-24 months following surgical removal of the primary tumor. Metastasis is observed very infrequently, but this can result in a poor prognosis. Metastasis may occur after 5-15 years of primary tumor diagnosis, and hence, long-term follow-up is very important.

Additional and Relevant Useful Information for Pseudomyogenic Hemangioendothelioma:

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/cancer/