Treatment

Depending on the extent of the tumor growth and the overall condition of the patient, the following treatment options are utilized. Surgery alone is often the only treatment needed. However, the recurrence rate of desmoid tumor is often as high as 30% and more than one surgery may be needed. The tumor tends to become more aggressive when it recurs after resection. For patients who are not appropriate candidates for surgery or have recurrences not responding to repeated surgeries, the following options may be considered:

Watchful waiting policy: Because desmoid tumors do not metastasize and can be followed closely for growth and because treatment with surgery, radiation, and/or chemotherapy can cause significant morbidity and even mortality, patients with asymptomatic or minimally symptomatic disease that has stable appearance on screening modalities may appropriately be treated with a period of watchful waiting.

Anti-inflammatory drugs may cause the tumor to slowly shrink. Non-steroidal anti-inflammatory drugs (NSAIDs) and drugs such as Imatinib are used to treat desmoid tumors.

Hormone therapy: Some hormones seem to increase the growth of desmoid tumors, so anti-hormonal medications such as anti-estrogens and prostaglandin inhibitors may also be used therapeutically.

Chemotherapy: If surgeons cannot remove the desmoid tumor because of size or location, chemotherapy may be used to reduce tumor size. Agents include Doxorubicin (Adriamycin, Rubex), Dacarabazine (DTIC-Dome) and Carboplatin (Paraplatin).

Novel molecular-targeted therapies: Kinases are regulators of cell growth, differentiation, and motility. Because these processes are deregulated in tumors cells, a new class of drugs called receptor kinase inhibitors has been developed. Gleevec and Sorafenib are two kinase inhibitors that are useful in treating desmoid tumors.

Radiation therapy as a treatment for recurrent disease or as primary therapy to avoid mutilating surgical resection uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.

Monitoring: After surgery, MRI is used to monitor recurrence in the arms and legs. CAT scans are used to monitor intra-abdominal and chest desmoids.