Angiomatoid fibrous histiocytoma is a rare soft tissue tumor that usually affects children, adolescents and young adults. Tumors — which may be benign (noncancerous) or malignant (cancerous) — typically develop on arms, legs, hands or feet. Preferred treatment is surgical removal, but chemotherapy or radiation therapy may also be necessary.
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.
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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.
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).
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.
AFH is rare. It makes up approximately 0.3% of soft tissue tumors.
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.
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.
It’s possible, but rare. AFH metastasizes (spreads) to other parts of the body in less than 1% of cases.
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.
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.
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.
There’s no known way to prevent 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.
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.
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.
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:
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.
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