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

• Soft Tissue Angiofibroma

What is Angiofibroma of Soft Tissue? (Definition/Background Information)

• Angiofibroma of Soft Tissue is an uncommon, benign tumor of the soft tissue. The tumor consists of blood vessels and fibrous tissues. These tumors may be observed in both children and adults
• There are no clear risk factors identified for Angiofibroma of Soft Tissue. It is believed to develop due to genetic mutations and chromosomal aberrations
• The signs and symptoms associated with Angiofibroma of Soft Tissue include the presence of slow-growing tumor on the limbs, which presents no pain. Some tumors may grow to large sizes leading to a compression of the adjoining organs and structures
• In a majority of cases, a simple surgical excision and removal of the tumor is considered curative. The prognosis for Angiofibroma of Soft Tissue is excellent with suitable treatment, since these are benign tumors

Who gets Angiofibroma of Soft Tissue? (Age and Sex Distribution)

• Angiofibroma of Soft Tissue is a rare tumor that is observed in both children and adults
• A wide age range of individuals, between 6-86 years, may be affected (median age of 49 years)
• The tumor is known to occur in both males and females; although, a slight predilection for females is observed (female-male ratio 2:1)
• Individuals of all racial and ethnic background may be affected; no geographical preference is observed

What are the Risk Factors for Angiofibroma of Soft Tissue? (Predisposing Factors)

• The risk factors for Angiofibroma of Soft Tissue are generally unknown

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 Angiofibroma of Soft Tissue? (Etiology)

The cause of development of Angiofibroma of Soft Tissue is due to genetic mutations.

• AHRR-NCOA2 gene fusion due to chromosomal translocation, namely t(5;8)(p15;q13), is observed in many tumors
• An isolated case featured the chromosomal translocation t(7;8;14)(q11;q13;q31)

What are the Signs and Symptoms of Angiofibroma of Soft Tissue?

The signs and symptoms of Angiofibroma of Soft Tissue include:

• Presence of benign tumors of size between 1 to 12 cm; average of 3.5 cm
• Some are known to grow to large sizes and can compress the surrounding structures through mass effect
• The angiofibromas are generally located in the soft tissues of the arms and legs, closer to the joints
• The tumors are slow-growing and painless in many cases

How is Angiofibroma of Soft Tissue Diagnosed?

The following are the diagnostic methods to help identify Angiofibroma of Soft Tissue:

• A thorough physical examination and a complete medical history are very crucial
• Radiological studies of the affected limb, including X-rays, CT or MRI scans
• Vascular angiography study: If such a study is undertaken, then the findings of the study are helpful to a surgeon during removal of the tumor, to help minimize bleeding

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

• Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy may be recommended.
• Core biopsy of the tumor
• Open biopsy of the tumor

Tissue biopsy:

• A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together 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 gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

A differential diagnosis to eliminate the following tumors is often considered, before arriving at a definitive diagnosis:

• Cellular angiofibroma
• Low-grade fibromyxoid sarcoma
• Low-grade myxofibrosarcoma
• Myxoid (round cell) liposarcoma
• Solitary fibrous tumor

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 Angiofibroma of Soft Tissue?

Significant complications are not known to typically arise from Angiofibroma of Soft Tissue. In some cases, the complications may include:

• Some tumors may bleed, which can be prolonged and heavy, usually after a biopsy
• Cosmetic issues causing emotional stress
• Local recurrence of the angiofibroma following its incomplete removal
• Damage to vital nerves, blood vessels, and surrounding structures during surgery

How is Angiofibroma of Soft Tissue Treated?

The treatment options for Angiofibroma of Soft Tissue may include the following procedures and measures:

• A complete surgical excision of the tumor is the treatment of choice. A partial removal of the tumor may result in tumor recurrence
• Arterial embolization is a possible treatment option. Here the blood supply to the tumor is blocked resulting in tumor death
• 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 Angiofibroma of Soft Tissue be Prevented?

Currently, there are no specific methods or guidelines to prevent Angiofibroma of Soft Tissues.

What is the Prognosis of Angiofibroma of Soft Tissue? (Outcomes/Resolutions)

Angiofibroma of Soft Tissue is a rare and benign tumor. If completely excised and removed, the prognosis is usually excellent and recurrences are not observed.

Additional and Relevant Useful Information for Angiofibroma of Soft Tissue:

• Juvenile nasopharyngeal angiofibroma (JNA) is an uncommon, benign tumor of the nose that is generally seen in boys. The tumor is locally aggressive and can invade into surrounding structures and cause significant complications

The following DoveMed link will help understand juvenile nasopharyngeal angiofibroma:

http://www.dovemed.com/diseases-conditions/juvenile-nasopharyngeal-angiofibroma/