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What

Fibromatosis, also called desmoplastic fibroma, is a general term for a group of benign (noncancerous) tumors in the fibrous connective tissues of the body. These soft tissue tumors can affect the fasciae (tissue around muscles and organs), ligaments, tendons, sheaths around nerves, and tissues making up blood and lymph vessels. Although benign, these tumors, called fibromatoses, can be a problem, as they tend to invade locally, and may return (recur) after removal by surgery. Desmoid tumors, also called aggressive fibromatosis – a defined, firm, rubbery mass – are the most common kind of fibromatosis. congenital generalized fibromatosis (CGF) is a pediatric condition often referred to as "infantile myofibromatosis" (IM). Here, single or multiple noncancerous tumors grow from cells that normally make up the supporting and binding tissues of the body and involuntary (smooth) muscle. These firm, nodular, potentially locally invasive tumors can involve the skin and underlying (subcutaneous) tissues, muscle tissue, bones and/or certain internal organs (viscera). In many cases, tumors are present at birth (congenital), develop within the first few weeks of life, or become apparent before the age of two years. Following an initial growth phase, the tumors may recede and even disappear. Children with solitary or multiple lesions that don’t involve the viscera typically have a benign outcome. However, infants with severe or widespread involvement of vital internal organs may have potentially life-threatening complications. Infantile digital fibromatosis (IDF) is a benign condition in which nodules of fibrous tissue grow almost exclusively on the tops and sides of the fingers or toes. Because fibrous connective tissue exists throughout the body, fibromatoses can arise in many different places. They may be deep within the abdomen, or superficial (closer to the surface). Tumors often appear in the arms, legs or trunk (main part of the body). Sometimes they appear in the rib cage, shoulders or axilla (armpits). Plantar fibromatosis occurs on the soles of the feet, and palmar fibromatosis occurs on the palms of the hands. The scalp and neck are other favored sites. Tumors can be aggressive and invade other types of tissue, including nearby muscle and soft tissues. Sometimes, bone tissue may be involved as the tumor grows.

Who

Fibromatosis includes a wide array of tumors, and is sometimes divided into two categories: infantile fibromatosis and adult fibromatosis. Infantile fibromatosis exclusively occurs in infants and young children under 2 years of age.

Signs and Symptoms

Usually, fibromatoses are painless, unless they are causing pressure or interfering with normal function of a joint or muscle. They may be noticed as a bump or lump.

Possible Causes

Research studies suggest that cells involved in fibromatosis may have undergone genetic alterations, particularly in the deeper tumors.

Diagnosis

Because fibromatosis can occur in so many parts of the body, diagnosis can involve many different tests. 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 single tissue sample is taken from the tumor. A procedure called Fine Needle Aspiration (FNA) may be used, in which a very thin and fine needle is inserted into the tumor site, and cells are extracted for laboratory studies. Microscopic examination will reveal what kinds of cells are present and enable the doctor to identify exactly what kind of abnormal cells are there. X-rays may also be ordered. 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. This technique uses powerful magnets and a computer to create detailed images of organs and structures. ultrasound is another procedure that may be used. This technique uses sound energy to create an image of organs or other structures inside the body.

Treatment

Treatment will depend on the specific diagnosis, the area affected and how aggressive the fibromatosis appears to be. Surgery is usually required to remove the tumor, often with some surrounding healthy tissue to prevent recurrence. Radiation therapy and chemotherapy are sometimes utilized for the treatment of these tumors. Sometimes both surgery and radiation are used. When chemotherapy is used, the drugs are given in low doses. There are few short-term side effects and almost no long-term side effects.

Prognosis

Prognosis always depends on many factors, such as the extent of the disease, size and location of the tumor, 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. Fibromatosis is not malignant; however, it can cause problems if it invades nearby tissues. In some severe cases of abdominal tumors, there may be involvement of organs that can present more difficulty during surgery. Certain aggressive forms, such as desmoid tumors, may recur after surgery. Each patient’s case may be different, and your doctor will work to ensure the best result for your child.

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

Bone tumor.org
A brief but detailed summary of fibromatosis.

Maxillofacial Center
A detailed website with lots of information about fibromatosis.

Google Search for Fibromatosis

References and Sources

www.orthopaedics.chop.edu/mt_desmoid.shtml www.bonetumor.org/tumors/pages/page54.html www.the doctorsdoctor.com/diseases/fibromatosis.htm