10585 Background: Desmoids are rare, slow growing, histologically benign tumors without metastatic potential. Methods: We examined outcomes of patients with desmoid tumors receiving systemic therapy at a single institution, to provide a basis for examination of newer agents. Retrospective chart review of 682 patients with desmoid tumors (1982–2006) from a prospectively collected sarcoma database. The activity of NSAIDs was not addressed. Patients without measurable disease, those receiving therapy we could not document, and those receiving prophylactic therapy were excluded. Results: A total of 70 patients received 163 lines of well-documented systemic therapy starting before 1/1/2007. Nine patients died, 7 of progressive disease/surgical complications, and two with Gardner syndrome-related malignancies. Demographics: 45 females (65%), median age 30 (range 15–75), 21 with Gardner syndrome (30%), median follow-up 68 months, and median of 2 lines of therapy (1–7). Intra-abdominal primary location was most common (29/70=41%). Tamoxifen, doxorubicin (including Caelyx/Doxil), imatinib, and methotrexate combinations were most commonly used. Five partial responses were observed with anthracyclines (43 courses administered), 2 with hormonal therapy (32 courses), and 1 with imatinib (35 courses), for 8 PR s. Conclusions: Anthracycline-containing regimens, hormonal therapy, and tyrosine kinase inhibitors have modest activity against desmoid tumors. The choice of therapy for these morbid and potentially fatal tumors should balance the efficacy of treatments with their short- and long-term side effects. No significant financial relationships to disclose.