Angiomatoid Fibrous Histiocytoma

Overview

What is angiomatoid fibrous histiocytoma?

Angiomatoid fibrous histiocytoma (AFH) is a rare type of soft tissue tumor. It commonly affects your extremities, such as your arms, legs, hands or feet.

A histiocytoma is a tumor that contains normal immune cells (histiocytes) that have traveled from one area of your body to another. The term “angiomatoid” describes the vascular-like appearance of the tumor. “Fibrous” refers to the ropey, sinewy nature of the mass.

Who does angiomatoid fibrous histiocytoma affect?

Angiomatoid fibrous histiocytoma most commonly affects children, adolescents and young adults. Approximately 88% of all people diagnosed with AFH are age 30 or younger. The average age of diagnosis is 14.

Where do angiomatoid fibrous histiocytomas occur?

Angiomatoid fibrous histiocytomas generally occur in the dermis (skin) of your arms and legs. In extremely rare cases, AHF can develop in the somatic soft tissues, including your brain, lung, mediastinum (the part of your body that contains your heart and thymus gland) and omentum (the fold of tissue that connects your stomach to your other abdominal organs).

Is angiomatoid fibrous histiocytoma cancerous?

Angiomatoid fibrous histiocytomas are often benign soft tissue tumors, meaning they’re noncancerous. But they can be malignant (cancerous) soft tissue tumors in some cases. When tumors are malignant, they might be called soft tissue sarcomas. Overall, AFH is considered a low-grade malignant tumor. This means there’s a low probability that it’ll spread to other parts of your body.

How common is angiomatoid fibrous histiocytoma?

AFH is rare. It makes up approximately 0.3% of soft tissue tumors.

Symptoms and Causes

What are angiomatoid fibrous histiocytoma symptoms?

Many people with AFH don’t have symptoms at all. If warning signs develop, however, they usually include fever, anemia and weight loss. Pain and tenderness are extremely rare and only affect a small percentage of people with the condition.

What causes angiomatoid fibrous histiocytoma?

Histiocytomas develop when histiocytes (normal immune cells found in various parts of your body) grow rapidly and produce more histiocytes. AFH has been linked to genetics, inherited conditions and radiation treatment, but the exact cause is unknown.

Can angiomatoid fibrous histiocytoma spread?

It’s possible, but rare. AFH metastasizes (spreads) to other parts of the body in less than 1% of cases.

Diagnosis and Tests

How is angiomatoid fibrous histiocytoma diagnosed?

Due to the nature of angiomatoid fibrous histiocytomas, they’re often mistaken for hematomas (when blood collects outside of the blood vessels) or hemangiomas (bright red birthmarks that form during the first or second week of life).

If your healthcare provider suspects AFH, they’ll likely have all or part of the AFH removed (biopsied) and examined under a microscope. AFH is commonly characterized by solid nests of histiocyte-like cells, cyst-like spaces and masses of chronic inflammatory cells. If any of these hallmarks are noted, an AFH diagnosis is made.

Management and Treatment

How is angiomatoid fibrous histiocytoma treated?

Angiomatoid fibrous histiocytoma treatment usually involves surgical removal of the tumor. Your surgeon removes the entire tumor, as well as some healthy tissue around it. This method reduces the risk of recurrence (return).

If the tumor is located in an area that makes surgical removal challenging (such as your head or neck) — or if the tumor has spread to distant areas of your body — then chemotherapy or radiation therapy may be recommended.

Are there complications regarding angiomatoid fibrous histiocytoma treatment?

Like any surgical procedure, tumor removal carries the risk of certain side effects, such as infection, nerve damage, pain and bleeding.

People who undergo chemotherapy or radiation therapy may experience a wide range of side effects, including anemia, hair loss, fatigue, and nausea and vomiting. Your healthcare provider can tell you what to expect regarding your treatment.

Prevention

Can I prevent angiomatoid fibrous histiocytoma?

There’s no known way to prevent angiomatoid fibrous histiocytoma.

Outlook / Prognosis

What can I expect if I have angiomatoid fibrous histiocytoma?

If you or your child has been diagnosed with AFH, your healthcare provider will discuss your options with you in detail. If the tumor hasn’t spread, then surgery will likely be the suggested treatment. In the majority of cases, there isn’t a recurrence (return) following surgical removal of the tumor.

If the tumor has spread beyond the point of origin to distant areas of your body, then chemotherapy or radiation therapy may be recommended. This requires multiple appointments and follow-ups to monitor your progress.

Can angiomatoid fibrous histiocytoma be cured?

In most cases, AFH can be cured with surgery. In rare instances, the disease spreads to other areas of your body. If this happens, AFH is usually fatal.

Living With

When should I see my healthcare provider?

Anytime you notice sudden changes in the appearance of your skin, schedule a visit with your healthcare provider. They’ll monitor the area and run any necessary tests.

What questions should I ask my doctor?

Learning all you can about your diagnosis can help you make informed decisions about your health. Here are some questions you may want to ask your healthcare provider:

  • Where is my tumor located?
  • What is the size of my tumor?
  • Is my tumor benign or malignant?
  • Has my tumor spread?
  • What are my treatment options?
  • What are the chances that surgery will work?
  • Should I consider chemotherapy or radiation therapy?
  • Can you recommend resources so I can learn more about my diagnosis?

A note from Cleveland Clinic

Most angiomatoid fibrous histiocytomas are noncancerous and treatable with surgery. In rare cases, you may require chemotherapy or radiation therapy. If you have malignant AFH, ask your healthcare provider about support groups in your area. Talking with others who are going through the same thing can be beneficial for your mental, emotional and spiritual health.

Last reviewed by a Cleveland Clinic medical professional on 01/12/2022.

References

  • Bauer A, Jackson B, Marner E, Gilbertson-Dahdal D. Angiomatoid fibrous histiocytoma: a case report and review of the literature. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3558265/) J Radiol Case Rep. 2012;6(11):8-15. Accessed 1/12/2022.
  • Bernini JC, Fort DW, Pritchard M, Rogers BB, Winick NJ. Adjuvant chemotherapy for treatment of unresectable and metastatic angiomatoid malignant fibrous histiocytoma. (https://pubmed.ncbi.nlm.nih.gov/8039125/) Cancer. 1994 Aug 1;74(3):962-4. Accessed 1/12/2022.
  • Huerter ME, Hammadeh R, Zhou Q, Riker AI. Recurrent angiomatoid fibrous histiocytoma: a case report and review of the literature. (https://pubmed.ncbi.nlm.nih.gov/25249813/) Ochsner J. 2014 Fall;14(3):441-4. Accessed 1/12/2022.
  • Morgan LM, Miller ER, Raj AB, Coventry SC, Elster JD. Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency. (https://publications.aap.org/pediatrics/article/141/3/e20162065/37602/Angiomatoid-Fibrous-Histiocytoma-With?autologincheck=redirected) Pediatrics. 2018:141;3. Accessed 1/12/2022.
  • Saito K, Kobayashi E, Yoshida A, Araki Y, et al. Angiomatoid fibrous histiocytoma: a series of seven cases including genetically confirmed aggressive cases and a literature review. (https://bmcmusculoskeletdisord.biomedcentral.com/articles/10.1186/s12891-017-1390-y#citeas) BMC Musculoskelet Disord. 2017;18, 31. Accessed 1/12/2022.

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