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

• PHAT (Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts)

What is Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts? (Definition/Background Information)

• Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts (PHAT) is an infrequent tumor of the soft tissues, occurring beneath the skin surface, having a potential for aggressive behavior
• Generally, individuals in the middle to late adult phase are prone to this disorder
• This painful or painless tumor appears as a single, well-defined, long-standing mass. The most frequent location of PHAT is the lower limbs, followed by the trunk
• Wide surgical excision, with or without chemotherapy and/or radiotherapy, are used to treat the tumor. The prognosis is excellent, however the tumor might recur

Who gets Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts? (Age and Sex Distribution)

• Most Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts occurs in adults, who are between 40-80 years old. However, children, teenagers, and young adults, are also known to be affected
• It is believed that females are affected more than males (4:3 female-male ratio)
• There is no known ethnic or racial preference

What are the Risk Factors of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts? (Predisposing Factors)

No risk factors have been identified with regards to Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts.

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 Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts? (Etiology)

The exact cause and mechanism of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts formation, is unknown.

What are the Signs and Symptoms of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts?

Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts formation and growth occurs over many years. The signs and symptoms may include:

• They may be present as well-defined painless mass. Some individuals may experience tenderness and pain
• They may also cause reduced motion range and discomfort, if present around joints
• The skin over subcutaneous tumors may turn dark tan to bluish tan
• In rare cases, the tumors may be located deep inside the tissues
• PHAT occurs mainly in the lower limbs (ankle and foot). Other locations include the chest wall, breast, groin, buttock, and arms

How is Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts Diagnosed?

A diagnosis of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts may involve:

• Physical examination with evaluation of patient’s medical history
• Tissue biopsy of the tumor: Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
• CT scan, MRI scan of the affected region
• Differential diagnosis, to eliminate other tumor types is 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 Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts?

The possible complications of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts may include the following:

• Deep-seated tumors (those buried in the body tissues), may create problems for adjoining tissues and organs by compressing them (due to mass effect of the tumor)
• Damage to muscles, vital nerves, and blood vessels, during surgery
• Recurrence of PHAT after surgery, which is a high possibility
• There have been cases of limb (parts) amputations

How is Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts Treated?

Treatment measures for Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts include:

• Wide surgical excision of the tumor with removal of the entire lesion is the preferred method to treat PHAT. If it is not fully removed, then the tumor might recur
• Chemotherapy and radiation therapy have been used with a certain degree of success
• 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 Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts be Prevented?

• Current medical research have not established a way of preventing Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts
• 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 high chances of recurrence

What is the Prognosis of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts? (Outcomes/Resolutions)

• Prognosis of Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts is excellent with surgical intervention. A complete excision usually results in a low tumor recurrence
• Generally, the tumor recurrence rate is known to be high (about 30-35%), which may take place after many years, sometimes even decades; especially, if the tumor is partially removed

Additional and Relevant Useful Information for Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts:

• Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts in simple terms means “a soft tissue tumor of variable size and shape, with a glass-like appearance having dilated blood vessels”.
• PHAT are abnormal masses characterized by spindle-shaped cells in groups of dilated blood vessels, lined with a fibrous protein