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

• Solitary Infantile Myofibroma
• Solitary Myofibroma

What is Myofibroma? (Definition/Background Information)

• Myofibroma is a rare, benign, perivascular tumor, meaning that it is a tumor of the cells surrounding the blood vessels
• It is considered as a pediatric tumor, since it normally occurs by birth or within 24 months following birth of the child. This tumor frequently affects the limbs and head and neck region
• The cause of tumor formation is unknown and so are the risk factors; however, some reports indicate that Myofibromas are inherited in an autosomal dominant manner
• Surgical treatment of the tumor is generally performed, through a complete excision and removal of the tumor. The prognosis of Myofibroma is generally excellent on adequate treatment, since it is a benign tumor
• Nevertheless, Myofibromas are known to severely damage organs and bones in some cases, leading to severe complications that can be fatal for the child

Who gets Myofibroma? (Age and Sex Distribution)

• Myofibroma is a rare tumor generally seen in the first 2 years following birth of a child. It may be present at birth (in up to 60% of the cases)
• Myofibromas are infrequently seen in adults
• Males are affected more than females
• No specific racial or ethnic preference is observed

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

• Currently, no definitive risk factors for Myofibroma are evident
• Some research indicates that there may be a familial pattern of inheritance for these tumors

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 Myofibroma? (Etiology)

• The exact cause of Myofibroma formation is unknown
• Some study reports inform that these tumors may be inherited in an autosomal dominant form
• Research is being performed to understand the cause of formation of these tumors

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Myofibroma?

The signs and symptoms of Myofibroma include:

• The tumors affect the skin and tissues below the skin (subcutaneous layers)
• The most common location of these lesions are the arms and legs, head and neck region, and chest and back (trunk region), affecting the skeletal muscles
• Solitary or multiple tumors may be observed; the presence of multiple tumors at different sites in the body is known as myofibromatosis
• The benign tumors are well-circumscribed and appear as nodules; some tumors are noted to be infiltrative in nature
• The size of the tumor may range from 1-5 cm; average size 2.5 cm
• The tumors may affect the organs and even the bones, in many cases. This can result in pain in some cases
• The bones that are involved may include the long bones of the arms and legs, the skull, and the ribs and vertebral column

How is Myofibroma Diagnosed?

A diagnosis of Myofibroma may involve the following tests and exams:

• Physical exam with evaluation of medical history
• Imaging studies of the affected region via a CT or MRI scan; areas of calcification may be observed in the study due to bone involvement
• 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

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 Myofibroma?

The complications of Myofibroma may include:

• Occasionally, recurrence of the tumor following surgery
• Severe impairment of body functions due to involvement of vital organs and bones, particularly when multiple tumors are present in the body (myofibromatosis). This is known to cause severe complications and can even be fatal to the child in some cases
• Damage of vital nerves, blood vessels, and surrounding structures, during the surgery
• Side effects of chemotherapy (toxicity)

How is Myofibroma Treated?

The treatment for Myofibroma is decided on case-by-case basis and is based on the location of the tumor. The following measures may be considered:

• The healthcare provider may recommend a ‘wait and watch’ method for small-sized tumors that present no significant symptoms, especially if on the limbs
• Some tumors are seen to reduce in size and disappear on their own (spontaneous regression)
• Complete surgical excision and removal of the entire lesion is normally sufficient treatment. However, this depends upon the tumor location and accessibility for surgery
• Multiple tumors in the body may require to be treated through chemotherapy or interferon therapy
• 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 Myofibroma be Prevented?

Current medical research has not established a way of preventing Myofibroma formation.

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

• The prognosis of Myofibroma is generally excellent with treatment, when the lesions are small and found below the skin surface, at easily accessible locations (for surgery). The recurrence risk is also low
• However, tumors that affect the trunk or head and neck region, or multiple tumors that may involve several body organs are even known to cause death, due to organ dysfunction and severe associated complications. The involvement of the lung usually portrays an adverse outcome

Additional and Relevant Useful Information for Myofibroma:

Myofibroma and myofibromatosis tumors used to be referred to previously as infantile hemangiopericytoma.

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

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