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What

Angiomatoid malignant Fibrous Histiocytoma (AMFH), also called Angiomatoid Fibrous Histiocytoma (AFH), is a rare type of soft-tissue tumor or sarcoma known as malignant Fibrous Histiocytoma (MFH). AMFH tumors can arise in the deep fibrous tissues of the extremities, such as the thighs. AMFH is considered malignant, because it can spread and invade other tissues. Doctors are still trying to determine the origins of the angiomatoid subtype of sarcoma. MFH occurs most commonly in the extremities, such as the legs or arms, followed by the retroperitoneum (the tissue at the back of the abdominal cavity). Tumors typically grow in deep fascia (the tissue surrounding muscles) or skeletal muscle. MFH can also arise in many other places, including the lungs, kidneys, bladder, scrotum, vas deferens, heart, aorta, stomach, small intestine, orbit, central nervous system, paraspinal area, dura mater of the brain, facial sinuses, nasal cavity, oral cavity, nasopharynx and soft tissues of the neck.

Who

MFH occurs mainly in middle-aged people age 50 to 60, but cases have been reported in patients from 2 to 90 years of age. The angiomatoid subtype, AMFH, is the most commonly reported variety in people younger than 20 years. Boys may be more likely than girls to develop it. MFH is more common in Caucasians than in people of Asian or African ancestry.

Signs and Symptoms

Because MFH and its subtype AMFH can arise in many different parts of the body, specific symptoms can vary. Occasionally

Possible Causes

The origins of AMFH are not yet clearly understood. There may be genetic changes in the cells that become cancerous.

Diagnosis

To confirm the diagnosis of AMFH, your child’s doctor will want to take a biopsy. This is a simple surgical procedure in which a single tissue sample is taken from the tumor. Examination using a microscope will determine what kinds of cells are present and enable the doctor to identify exactly what kind of cancer may be present. If the diagnosis is AMFH, the doctor will determine what stage the tumor has reached, which will confirm whether or not the tumor has spread from its original location, and, if so, how far. “Staging” helps doctors decide what kind of treatment is best, and gives an idea as to what the prognosis will be. This is accomplished through noninvasive imaging studies that may be needed, including X-rays, Magnetic Resonance Imaging (MRI), a Computerized Tomography scan (CT or CAT scan) and bone scans. Blood tests probably will be ordered, as well.

Treatment

The main way to treat AMFH is to remove the entire tumor by surgery. Sometimes a second operation will be performed to make sure that all the tumor has been removed, or if the tumor comes back following treatment. Radiation therapy and chemotherapy may also be performed. There may also be clinical trials in progress. These are studies designed to test new and experimental therapies.

Prognosis

The prognosis of the angiomatoid subtype of MFH is usually good if surgery is performed and the tumor is completely removed. Outcomes are better when tumors can be caught and removed when they are small. Generally, AMFH tumors in children tend to be less aggressive than other types of MFH and rarely spread to other body parts.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

e- Medicine
National cancer Institute -

cancer Research Portfolio
A ebsite that provides information about ongoing research and clinical trials.

The sarcoma Alliance 
A general website with information on all types of sarcomas, including some specific to AMFH, that provides education, guidance and support for parents and families affected by the disease.

Google Search for Angiomatoid Malignant Fibrous Histiocytoma (AMFH)

References and Sources

www.choa.org/library/hemonc/soft_tissue_sarcoma.shtml www.emedicine.com/radio/topic420.htm www.researchportfolio.cancer.gov/cgi-bin/countbyterm.pl?Term=4 www.sarcomaalliance.com/links.html