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

• GCA of Soft Tissue
• Soft Tissue GCA
• Soft Tissue Giant Cell Angiofibroma

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

• Giant Cell Angiofibroma (GCA) of Soft Tissue is an uncommon tumor of the soft tissues. This benign tumor consists of giant cells, blood vessels, and fibrous tissues
• GCA of Soft Tissue is observed in young, middle-age, and older adults. It is described as a type of hemangiopericytoma-solitary fibrous tumor. Some researchers consider it to be a variant of solitary fibrous tumor (SFT)
• There are no clear risk factors identified for Giant Cell Angiofibroma of Soft Tissue. It may develop due to genetic aberrations
• Giant Cell Angiofibroma of Soft Tissue are denoted as orbital or extra-orbital. Most tumors are present around the eye (orbital tumors), some are located at extra-orbital sites including the head and neck region, chest or abdominal cavity
• The signs and symptoms associated with Giant Cell Angiofibroma of Soft Tissue include the presence of slow-growing tumor in the head and neck region or at other sites, 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. However, incompletely removed tumors are known to recur locally

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

• Giant Cell Angiofibroma of Soft Tissue is a rare tumor that is observed in both children and adults
• A wide age range of individuals, between 18-81 years, may be affected (mean age 45-48 years)
• The tumor is known to occur in both males and females in equal incidence
• Individuals of all racial and ethnic background may be affected

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

• The risk factors for Giant Cell 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 one’s 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 Giant Cell Angiofibroma of Soft Tissue? (Etiology)

• The cause of development of Giant Cell Angiofibroma of Soft Tissue is unknown
• The tumor may be caused by genetic mutations that are yet to be well-characterized

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

The signs and symptoms of Giant Cell Angiofibroma of Soft Tissue include:

• Presence of benign, well-circumscribed tumors of size between 1 to 11 cm; average of 3-4 cm
• Most tumors are slow-growing; while some may grow rapidly, rising a concern for malignancy
• Some are known to grow to large sizes and can compress the surrounding structures through mass effect
• The tumors are painless in many cases, though some may be painful (especially large-sized tumors)
• Usually, the tumors are present around the eye (orbital region) and are called Giant Cell Angiofibroma of Orbit
• Extra-Orbital Giant Cell Angiofibromas may involve the nasolacrimal duct, lacrimal sac, buccal mucosa/oral cavity, other head and neck areas, the vulva, mediastinum, retroperitoneum, and axilla (armpits)
• In males, the tumors are mostly seen around the eye; in females, it is more often present in extra-orbital sites

How is Giant Cell Angiofibroma of Soft Tissue Diagnosed?

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

• A thorough physical examination and a complete medical history are very crucial
• Radiological studies of the affected region, including CT and 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

Note: When the tumor specimen is examined by a pathologist under the microscope, it is characterized by the presence of numerous blood vessels and giant cells. Hence, it is known as a Soft Tissue Giant Cell Angiofibroma.

A differential diagnosis to eliminate the following tumors is often considered, before arriving at a definitive diagnosis (for extra-orbital tumor sites):

• Deep fibrous histiocytoma
• Fibrosarcoma
• Giant cell fibroblastoma
• Hemangiopericytoma
• Malignant fibrous histiocytoma
• Schwannoma
• Synovial sarcoma
• Solitary fibrous tumor (for orbital tumors)

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 Giant Cell Angiofibroma of Soft Tissue?

Significant complications are not known to typically arise from Giant Cell 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
• Large tumors may severely affect the functioning of the involved organ through mass effect
• Local recurrence of the angiofibroma following its removal
• Damage to vital nerves, blood vessels, and surrounding structures during surgery
• Side effects from radiation therapy (if administered)

How is Giant Cell Angiofibroma of Soft Tissue Treated?

The treatment options for Giant Cell 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
• In case recurrence of the tumor is observed, radiation therapy may be given. Radiation may also be administered for tumors that are at surgically-inaccessible sites (or when surgery is not a feasible option)
• 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 Giant Cell Angiofibroma of Soft Tissue be Prevented?

• Currently, there are no specific methods or guidelines to prevent Giant Cell Angiofibroma of Soft Tissues
• Since, the tumors are known to recur, long-term follow-up is recommended

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

Giant Cell 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 Giant Cell Angiofibroma of Soft Tissue:

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