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Additional entries in our database that you might find useful:   Congenital Fibrosarcoma

What

Fibrosarcoma is a tumor of fibroblasts, the cells that create fibrous tissues. In newborns and infants up to about three years old, it is called Infantile Fibrosarcoma or congenital Infantile Fibrosarcoma (CFS). Fibrosarcoma affecting older children, adolescents and adults is sometimes called Adult-Type Fibrosarcoma (ATFS). Fibrosarcoma tumors often arise in the arms or legs, but may arise in the head, neck, and trunk as well. Fibrosarcomas are malignant and will often invade local tissues surrounding the tumor. They can sometimes spread to the lungs, and occasionally other sites via the bloodstream. Fibrosarcoma tumors can grow in bones, tendons, ligaments, fasciae that cover muscle tissue, and other connective tissues. Tumors usually appear in the arms, legs or torso, but the head and neck can also be affected.

Who

The “adult” form of fibrosarcoma can occur in children and adolescents between the ages of 10 and 15. In these patients, fibrosarcoma tends to be more aggressive than the infantile form and usually requires more complex treatment.

Signs and Symptoms

The symptoms of fibrosarcoma vary depending on size, location and spread of the tumor. Although each child may experience symptoms differently, some symptoms may include a painless or tender mass in an extremity or on the trunk, pain or soreness caused by nerves and muscles being squeezed by the tumor, or limping or other using legs, feet, arms or hands. In the bones, fibrosarcomas may become evident by pain and swelling. They may even grow large enough to cause the bone to fracture.

Possible Causes

While doctors do not completely understand what causes fibrosarcoma and other soft-tissue tumors, genetic alterations may play a role. In some fibrosarcomas, the chromosomes in the cells have been rearranged. Studies show that in a few families, several members of the same generation can develop soft-tissue sarcomas. Some fibrosarcomas may be associated with burns or radiation therapy that has caused earlier trauma to the area. In addition, there may be a link between soft-tissue sarcomas and the development of other types of cancer.

Diagnosis

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 tissue 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 fibrosarcoma. It will also help determine how aggressive the tumor is. X-rays may be ordered immediately. This diagnostic test uses invisible energy beams to create images on film of internal tissues, bones, and organs. X-rays are very helpful in diagnosing bone tumors and may help doctors tell the difference between a benign and malignant tumor. They also help the doctor decide which tests to perform next. A computed tomography scan (called a CT or CAT scan) may 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) may also be ordered. Using powerful magnets and a computer, MRI creates 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. Bone scanning, a nuclear imaging technique, is used to detect bone tumors and metastatic tumors (spread from the primary area). These scans can also help determine if there are abnormalities in other bones. Once a diagnosis of fibrosarcoma is confirmed, the tumor is staged. Staging will assess whether the tumor has spread from its original location, and if so, how far. Determining the stage (size and extent of spread) and grade of the tumor (how aggressive the tumor appears when examined microscopically) will help your child’s doctor determine the best course of treatment. A fibrosarcoma is called localized if it has not spread beyond the bone where it arose or beyond nearby tissues. If it is metastatic, it has spread to other areas, such as the lungs, other bones, or to other organs or structures of the body.

Treatment

Dealing with fibrosarcoma typically requires surgery to remove the cancerous tissue with a wide margin of healthy tissue that surrounds it. This reduces the risk of the tumor recurring at the site where it arose. The extent of surgery will vary depending on the location and the degree to which the cancer has advanced. Today, doctors frequently recommend “limb-sparing surgery” instead of amputation. Bone/skin grafts and reconstruction may also be needed, depending on the size and location of the tumor, and whether or not the cancer has spread. For soft-tissue and bone fibrosarcomas, chemotherapy and radiation therapy may also be used in conjunction with surgery to improve the chances that the disease will be eliminated. Older children with fibrosarcoma, and more advanced cases of the disease, tend to require more complex therapies involving a combination of surgery, chemotherapy and/or radiation. Chemotherapy for fibrosarcoma is usually given intravenously. After treatment, your child may need rehabilitation, including physical and occupational therapy to regain strength and use of the affected area after surgery. Some psychosocial therapy may also be needed, depending on the extent of the treatments. There may be special supportive care needed to prevent or treat infections, to help with the side effects of treatments and complications, and to keep your child comfortable during this time.

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, the child's ability to tolerate specific medications, procedures, or therapies, and new developments in treatment. The younger the child, the lower the risk of recurrence and spreading to other tissues. In older children and adults, tumors may recur (in the primary site or on another site). Post- and/or preoperative radiation therapy may help reduce the risk of local recurrence. Children with fibrosarcoma require follow-up visits, often with a schedule of repeat examinations and diagnostic studies. Patients may be monitored for a minimum of five years. If the disease recurs, additional treatments may be necessary.

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

Thorough technical review of fibromatosis
Provides a thorough, very technical summary of Fibrosarcoma. sarcoma Alliance sarcomaalliance.com A parent friendly website which provides information, research information, personal accounts and support links.

Google Search for Adult-Type Fibrosarcoma (ATFS)

References and Sources

E-Medicine www.emedicine.com/orthoped/topic599.htm#section~relevant_ anatomy_and_contraindications sarcoma Alliance www.sarcomaalliance.com/whatis.html