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Desmoid Tumors
Thursday, 22 July 2004
Sunday, 28 November 2004
Desmoplastic Fibroma, Aggressive Fibromatosis, Musculoaponeurotic Fibromatosis

What

Desmoid tumors are tumors derived from muscle and tendon tissue and are an overgrowth of fibrous tissue. These tumors are rare in children and are difficult to treat because they invade and encase other surrounding structures in the body.

Who

Children older than 10 are more frequently affected and the frequency amongst males and females is equal. The overall occurrence of desmoid tumors is estimated to be 0.03% among all other types of tumors. There is no known racial predilection. Desmoid tumors are more common in children with a family history of polyposis coli, or colon cancer.

Signs and Symptoms

Children with desmoid tumors may exhibit some or all of the following:  Painless lump or swelling on the arms, legs, abdomen, or chest.  If located in the abdomen, obstruction of the bowels leading to severe constipation and abdominal enlargement  Pain from nerve or muscle compression  New limp from tumors in the legs.

Possible Causes

Desmoid tumors are thought to arise from the aberrant growth and proliferation of immature muscle cells called myofibroblasts. As these cells grow and divide, they lose their normal cell signals for death and thus are immortalized. Women who are exposed to oral contraceptive agents have a higher risk. No one knows the exact mechanism or gene responsible for this disease.

Diagnosis

Making the diagnosis of a desmoid tumor can be done by a tissue biopsy. Prior to any biopsy or surgery, imaging studies such as CT scans or MRI (magnetic resonance imaging) are usually performed to better delineate the regions involved. Biopsied tissue can be prepared by a trained pathologist to look at special immunologic markers that can help distinguish desmoid tumors from other tumors. Blood tests such as a complete blood count or complete metabolic panel are usually done to detect any other co-existing conditions.

Treatment

The mainstay of treatment is to surgically remove as much tumor as possible while preserving the involved vital structures. Given that desmoid tumors infiltrate structures in the body, it is difficult to completely remove the tumor. Chemotherapy medications in low doses, and radiation therapies are also used to shrink the tumor. For patients who do not respond to surgery, chemotherapy, or radiation, a technique known as radiofrequency ablation (RFA) may be tried. There have been reports in the medical literature of successful patient responses to high doses of vitamin C and an anti-inflammatory agent called indomethacin. Current clinical trials through the Children’s Oncology Group have been studying the effects of two agents: tamoxifen and sulindac, on desmoid tumors that have been unsuccessfully treated with conventional therapy.

Prognosis

If complete surgical removal of the tumor is achieved, children are usually cured of their disease. However if there is even small amount of tumor left, there is a high likelihood of reoccurrence. The severity of any deficits depends on where the tumor is located and the degree to which it has spread in the body.

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

The Desmoid tumor Online Support Group
You must subscribe to this group to search their archives.

e-Medicine
A very comprehesive summary of DT.

Children's Oncology Group
Information for parents on cancers and clinical trials of treatment.

Medicinenet.com
Good general information about the disease.

Diamond Blackfan Anemia
This is a wonderful website that has a good review of DBA, tells of ongoing research, newsletter opportunities, and support from others that are also dealing with DBA.

Family Village
This website allows for family members and patients to chat with others that have been affected by DBA.

National cancer Institute
A detailed summary of the disease with nice definitions of technical terms. National Heart,

Lung and Blood Institute
A website with information about ongoing research on DBA.

Clinical Trials.gov
A website with information about clinical trials involving treatment of DBA.

Google Search for Desmoid Tumors

References and Sources

Wagstaff MJ, Raurell A, and Perks AG. (2004). Multicentric extra-abdominal desmoid tumours. Br J Plast Surg 57(4):362-5. Abdelkader M, Riad M, and Williams A. (2001) Aggressive fibromatosis of the head and neck (desmoid tumours). J Laryngol Otol.115(10):772-6.