Abstract
e20524 Background: Desmoids are a rare, slow growing, soft-tissue tumors with a locally agressive behavior. These tumors are often unresectable at diagnosis and there is no standard first line chemotherapy. Methods: We present a retrospective review of the outcomes of patients treated with the same regimen comprised by doxorubicin (DOX) 20mg/m2 daily plus dacarbazine (DTIC) 150 mg/m2 daily over four days of drip infusions at a single institution from June 2005 to December 2009. Results: A total of five patients were analyzed. All of them had a diagnosis of familial adenomatous polyposis (FAP). There were three men (60%) and the median age at diagnosis was 30 (range, 18 to 38 years). Four patients had intrabdominal tumors (80%) and were considered unresectable. The protocol DOX plus DTIC was the first line therapy for them. The remaining patient had tumor in abdominal wall and it was resected at diagnosis. This patient received tamoxifen to local relapse that occurred thirty-two months after surgery. In this case, the regimen DOX plus DTIC was used as second line therapy after tamoxifen progression. The median number of cycles was five (range, 3 to 6 cycles). All of the patients seemed to tolerate the chemotherapy well, and none patient showed severe treatment-related toxicity (grade 3 or 4). There were two partial responses and three stable disease. None complete response was observed. All except one were administered meloxicam (10 mg/m2) after chemotherapy. With a median follow-up time of 14,3 months (range, 8.5 to 54 months), only one progression disease occurred at 52 months. Conclusions: In our series of cases, doxorubicin plus dacarbazine was a safe and well tolerated regimen. Despite the low downstaging rates, it seems prolong substantially the time to disease progression in patients with desmoid tumours. No significant financial relationships to disclose.
Published Version
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