Abstract
10567 Background: Desmoid tumors (DT) are rare neoplasms characterized by clonal proliferation of myofibroblasts, that do not metastasize but exhibit an infiltrative pattern, producing tissue destruction and significant functional impairment. Although sporadic desmoids are rare, these tumors are common in patients (pts) with familial adenomatous polyposis (FAP): 3.5 to 32% of the pts are diagnosed with diffuse mesenteric desmoid, which is the second reported lethal complication of FAP. The combination of methotrexate (MTX) + vinblastine (VNB) is known to be active in extraabdominal desmoids. Methods: The dataset of the Istituto Nazionale Tumori, Milan (1,115 Classical FAP) was analyzed. Overall, 167 (14.9%) were diagnosed with DT arising in FAP. 104/167 were females, and age ranged from 29 to 70 years. 96/167 pts received surgery. In this case series analysis, we retrospectively reviewed clinical efficacy of weekly MTX at the dose of 30 mg/m2 + VBL at the dose of 6 mg/m2 in 29 consecutive pts, all of whom but 2 have concluded their therapy. As an external control, we retrospectively evaluated also 43 pts treated with other approaches (hormone therapy, NSAIDs, etc.) in the same period. Tumor response was assessed by RECIST criteria after the first 2 months of treatment and then every other month. Results: As of December 2007, 21 pts (72%) had PR or SD exceeding 6 months. Of pts treated with other approaches, 23 (53%) are still on treatment with PR or SD exceeding 6 months. Overall, 32/167 pts died, 14 of intra-abdominal DT (8.4%), 10 of Colorectal cancer, 6 of extracolonic cancer, and 2 of other causes. Conclusions: The MTX and VBL regimen seems to active also in intra-abdominal DT, as shown in sporadic DT, and may be considered among the medical options available for the advanced stages of this disease. No significant financial relationships to disclose.
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