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Angiomatoid Fibrous Histiocytoma (AFH)

Last updated May 1, 2018

Approved by: Maulik P. Purohit MD, MPH

Angiomatoid Fibrous Histiocytoma (AFH) is an infrequent tumor of the soft tissues, with a low-grade metastatic ability.

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

  • AFH
  • AMFH
  • Angiomatoid Malignant Fibrous Histiocytoma (AMFH)

What is Angiomatoid Fibrous Histiocytoma? (Definition/Background Information)

  • Angiomatoid Fibrous Histiocytoma (AFH) is an infrequent tumor of the soft tissues, with a low-grade metastatic ability
  • Children, teenagers, and young adults are most prone to this disorder. The lesions are painless and give an impression of small lymph nodes, lying deep beneath the skin surface. These tumors are not lymph nodes
  • The most common location for these tumors are near the lymph nodes of the knees and elbows; followed by the body torso, head & neck region

Who gets Angiomatoid Fibrous Histiocytoma? (Age and Sex Distribution)

  • Angiomatoid Fibrous Histiocytoma is mostly observed during childhood and in the young-adult phase (less than 30 years old). However, it may be present in a much wider age group, from infants to the very old
  • Both males and females are affected; though there is a slight predisposition towards the female sex
  • There is no known ethnic/racial preference

What are the Risk Factors for Angiomatoid Fibrous Histiocytoma? (Predisposing Factors)

Risk factors are currently unknown. Nevertheless, an external injury or trauma is sometimes mentioned as a possible reason for Angiomatoid Fibrous Histiocytoma.

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  foralways important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Angiomatoid Fibrous Histiocytoma? (Etiology)

  • The exact cause and mechanism of Angiomatoid Fibrous Histiocytoma formation is unknown. They are occasionally thought to occur in response to an injury/trauma
  • It is considered that the origin may be muscular-related, or connected to certain types of cells present in the lymph nodes, which synthesize collagen

What are the Signs and Symptoms of Angiomatoid Fibrous Histiocytoma?

The presentations are based on the location of Angiomatoid Fibrous Histiocytoma. Signs and symptoms of these include:

  • Most tumors are asymptomatic, with no indication of pain or any external sensation
  • The well-defined benign nodules (usually containing multiple cysts) grow at a slow rate. They may be located as superficial tumors (just below the skin) or deep-seated ones (lying deep inside the body tissues)
  • AFH usually occurs in the limbs (at the knee and elbow joints). The head, neck, and trunk are other regions where it is noticed from time to time. There have been rare instances of AFHs being found in the lungs, abdominal cavities, and female genitalia
  • Occasionally, there have been signs of anemia, fatigue, loss of weight, and fever due to Angiomatoid Fibrous Histiocytoma

How is Angiomatoid Fibrous Histiocytoma Diagnosed?

Diagnostic tests vary, based on location of the tumor. Angiomatoid Fibrous Histiocytoma is diagnosed by:

  • Physical examination, evaluation of patient’s medical history
  • Histopathological studies conducted on a biopsy specimen
  • X-ray studies (of the affected region, in non-skin tumors)
  • MRI scan (of the affected region, in non-skin 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 Angiomatoid Fibrous Histiocytoma?

The complications from Angiomatoid Fibrous Histiocytoma could include:

  • Deep-seated tumors (those buried in the body tissues), may create problems for adjoining tissues and organs, hindering their normal function
  • Recurrence of the tumor after surgery
  • There is a very small risk of malignancy (about 1% chance), which has led to a few deaths (malignant transformation of AFH)
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy (such as radiation fibrosis)

How is Angiomatoid Fibrous Histiocytoma Treated?

Treatment measures for Angiomatoid Fibrous Histiocytoma include:

  • If there are no symptoms associated with Angiomatoid Fibrous Histiocytoma, then non-operative measures are adopted. Periodic observations are maintained
  • Surgical treatment: Wide surgical excision of AFH tumor with removal of the entire lesion is to be performed. If the tumor is not fully removed, then it might recur
  • Chemotherapy and radiation therapy have been used with a certain degree of success; when surgical methods have not been feasible
  • Post-operative care is important: 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 Angiomatoid Fibrous Histiocytoma be Prevented?

  • Current medical research have not established a way of preventing Angiomatoid Fibrous Histiocytoma occurrence
  • Regular medical screening at periodic intervals with scans and physical examinations are mandatory for those who have already endured AFH; due to its chances of recurrence. Often several years of active vigilance may be necessary

What is the Prognosis of Angiomatoid Fibrous Histiocytoma? (Outcomes/Resolutions)

  • Prognosis is excellent when lesions are well-defined and located just below the skin surface and are completely removed by surgical procedures; this reduces their recurrence risk too
  • The probability of Angiomatoid Fibrous Histiocytoma recurrence is moderate at 11%, after surgical removal of the tumor
  • Nevertheless, since AFH possesses metastatic potential (in rare cases); regular follow-up is certainly advised

Additional and Relevant Useful Information for Angiomatoid Fibrous Histiocytoma:

  • Angiomatoid Fibrous Histiocytoma are primarily composed of a kind of fibrous connective tissue cells (called histiocytes), spindle-shaped cells, and cystic cavities containing blood
  • Angiomatoid Fibrous Histiocytoma is sometimes confused with Aneurysmal Fibrous Histiocytoma of Skin; a kind of soft tissue (benign) tumor that mimics other fibrous histiocytoma types. This creates a higher possibility of misdiagnosis.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: June 16, 2013
Last updated: May 1, 2018