Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. However, tumor invasion into vital structures and/or organs can result in substantial morbidity and may be fatal. Local control using surgery and radiation has traditionally been the mainstay of therapy for these tumors but some desmoid tumors may not be resectable and for them, systemic therapy is recommended. NCCN on p/ SARC-E,1 lists Sulindac or other nonsteroidals, tamoxifen or toremafine, methotrexate and vinblastine, low dose interferon, doxorubicin based regimens and imatinib. Sorafenib has been reported by Gounder et al to be effective but is not NCCN recommended.
Nieuwenhuis MH, Mathus-Vliegen EM, Baeten CG, et al. Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients. Br J Cancer 2011; 104:37.
Gounder MM, Lefkowitz RA, Keohan ML, et al. Activity of Sorafenib against desmoid tumor/deep fibromatosis. Clin Cancer Res 2011; 17:4082.
nccn.org, Desmoid 2011