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What

There are two kinds of Fibrous Histiocytoma. The noncancerous form, benign Fibrous Histiocytoma, is a painless, sometimes pigmented mass that can be removed fairly easily by surgery. malignant Fibrous Histiocytoma (MFH) is a cancerous tumor that arises in soft tissues or bones and tends to spread to other tissues and organs. When the disease arises in bone, it resembles a type of bone cancer and may be treated similarly. There are at least five different subtypes of the disease, with different clinical scenarios. One subtype, called angiomatoid, most often affects people under 20 years of age. MFH occurs most commonly in the arms and legs and in the retroperitoneal area (near the kidneys). Tumors typically arise in deep fascia (the membranes that surround muscles) or skeletal muscle. MFH can affect many parts of the body, including the bones, lungs, kidneys, bladder, heart, stomach, small intestine, central nervous system, mouth, sinuses, and soft tissues of the neck.

Who

Males are more often affected than females.

Signs and Symptoms

Most often, soft-tissue MFH will appear as a growing, painless, soft tissue mass in the thigh, typically one to two inches in diameter. Two-thirds of tumors are within muscle tissue. Rarely, there will be episodes of hypoglycemia (low blood sugar). MFH has been associated with other diseases, including Hodgkin’s disease, non-Hodgkin’s lymphoma, multiple myeloma, and malignant histiocytosis. MFH in the retroperitoneal area can exhibit systemic symptoms, including fever, malaise (feeling rundown), and weight loss. Here, tumors can grow larger than two inches in diameter, and can displace and obstruct the bowel, kidney, ureter and/or bladder. In bones, symptoms may appear as pain and swelling in a leg or arm. Pain may worsen at night and may precede the appearance of a lump. The child may initially present with an unexplained limp. In some cases, MFH can weaken the bone structure, leading to a broken bone, which can be the first sign of this type of cancer.

Possible Causes

Research evidence suggests that genetic or chromosomal factors may be involved in the development of MFH. Some MFH tumors may be associated with prior radiation to the area.

Diagnosis

Several methods may be used by your child’s doctor to diagnose MFH. In addition to a complete medical history and physical examination, your child’s doctor may order a biopsy. This is a simple surgical procedure in which a small sample is taken from the tumor. Examination using a microscope will determine what kinds of cells are present and enable the doctor to confirm the diagnosis of MFH. biopsy results will also help determine which of the subtypes of MFH may be present and can help your child’s doctor decide the best course of action. Additional tests will be needed to determine whether the disease has spread from its primary location (metastasized). MFH is an aggressive cancer and often invades other tissues and organs. Different imaging techniques can help identify the extent of the tumor, although the actual diagnosis will come from examining the cell types in the tissue sample. X-rays may be ordered, and another technique, called a bone scan, can help assess the disease in bone tissue. A computed tomography scan (called a CT or CAT scan) may also be useful. This diagnostic imaging procedure combines X-rays and computer technology to produce detailed cross-sectional images (“slices”) of the body. Magnetic resonance imaging (MRI) is an imaging technique that uses powerful magnets and a computer to create detailed images of organs and structures. It can help determine the size and extent of the mass and its relationship to nearby muscle, bone, nerves, and blood vessels. ultrasound, similar to that used to image pregnancies, is another method that your child’s doctor may use for a soft-tissue tumor. ultrasound uses sound waves to create a picture of structures within the body.

Treatment

Surgery will likely be a major part of your child’s treatment. Removing the mass and additional tissue surrounding the affected area is likely to reduce the chance that it will return. Bone and/or skin grafts may be needed. The extent of surgery will depend on the size and location of the tumor and whether or not the cancer has spread. Radiation therapy may be required to further reduce the risk of the recurrence of the tumor where it arose. Chemotherapy may be required to prevent or to treat the spread of the cancer to other areas of the body. Physical therapy may also be needed to restore strength and use of the affected area after surgery and/or radiation.

Prognosis

Prognosis always depends on many factors, such as the extent of the disease, size and location of the tumor, the presence or absence of metastasis, the tumor's response to therapy, the age and overall health of your child, your child's ability to tolerate specific medications, procedures, or therapies, and new developments in treatment. malignant Fibrous Histiocytoma is one of the more aggressive types of cancer, and results are better when it is diagnosed and treated early. Follow-up visits and tests will be necessary to detect whether or not the disease has returned or spread to other tissues.

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
A detailed and somewhat technical summary of malignant Fibrous Histiocytoma.

Cancerbacup
A nice, concise summary of MFH with links to other informational sites.

The sarcoma Alliance
A great website dedicated to providing education, guidance and support for those affected by different types of sarcomas.

Google Search for Fibrous Histiocytoma

References and Sources

www.emedicine.com/radio/topic420.htm www.cancerbacup.org.uk/questions/specific/sarcomas/sts/malignant-fibrous-histiocytoma.htm www.sarcomaalliance.org/splash.html