Disease Information

Solid Tumor: Desmoid Tumors

Alternate Names: fibromatosis juvenile (infantile) fibromatosis, aggressive fibromatosis, desmoid fibromatosis

Definition

• Desmoid tumors occur in children and young adults.
• They arise from muscle or connective tissue (“soft tissue”) around muscles or bones, and can appear anywhere in the body.

Incidence

• Desmoid tumors are very rare, accounting for less than 1 percent of childhood tumors.
• They can occur at any time throughout childhood.

Influencing Factors

• The cause of desmoid tumors is unknown.
• In some patients, desmoid tumors can occur as part of an inherited syndrome called Gardner Syndrome, in which patients also have colon polyps or colon cancer.
• Desmoid tumors can also occur in pregnant women, which has led to the theory that hormones may influence growth.
• Most of the time, desmoid tumors occur in previously healthy patients with no other medical problems.

Clinical Features and Symptoms

• Desmoid tumors usually appear as visible or palpable masses in the soft tissue beneath the skin.
• They can be painful.
• They can grow to be quite large (4 – 5 inches) in diameter before they are discovered.
• They can spread to involve nearby normal tissues around them.
• Depending on where the tumor is located, other symptoms may occur.
• Desmoid tumors usually occur as a single tumor, but sometimes a patient may have more than one tumor at diagnosis.

Survival Rates

• Desmoid tumors are neither a truly benign nor a truly malignant cancer.
• They can be life-threatening if located next to vital organs like the spine or trachea (windpipe) . 
• Surgical removal of the entire tumor usually achieves a cure. However, if even small amounts of tumor cells remain, the tumor can grow back again.
• Depending on the location of the tumor, surgical removal may not be possible. In these cases, radiation therapy or chemotherapy may prevent further tumor growth or cause the tumor to disappear.

Current Research

• At St. Jude, we are evaluating whether chemotherapy can successfully control desmoid tumors in children or young adults for whom surgery or radiation therapy is not feasible or has failed in the past.

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