What is it? These tumors generally occur in the extremities, but can arise anywhere. They are usually localized but can occasionally spread to local lymph nodes and rarely metastatasize (spread) to lungs and other organs. Gardner’s syndrome and familial polyposis coli are conditions that predispose individuals to desmoids. They can occur in regions of trauma.
What are the symptoms? Often present as a painless, slow growing mass.
How is it treated? Surgery is the primary treatment for desmoids tumors. If the tumor cannot be completely removed or recurs, other treatment can be considered. Radiation can be used to limit local recurrence. Clinical investigations have shown that methotrexate and velban have activity against Desmoid tumors. Other treatments under investigation include sulindac with taxotere and gleevec. Participating in a clinical investigation for patients with this disease may be of value.
What is the outcome? With complete resection the outcome is excellent. Young age at diagnosis, aggressive features on pathology, incomplete resection and underlying Gardner’s syndrome or familial polyposis coli increase the risk of recurrence. It is recommended that if you have this type of tumor you should be evaluated for a specific mutation (change) in the APC gene and you may need to be screened for colon cancer, usually by a procedure called endoscopy.
Additional information about soft tissue sarcomas
Robert Goldsby MD
University of California, San Francisco
March 2007