Desmoid tumor (DT) is a rare and locally invasive proliferative disease. Although, the absence of metastatic potential, it has the propensity for locally invasive growth and recur. Chemotherapy may be considered in inoperable and/or recurrent disease.
Patients with histological diagnosis of DT and treated with weekly low-dose chemotherapy with vinblastine and methotrexate between January 1998 and December 2015 were identified and their medical records were analyzed.
Of 23 patients analyzed, most of them were women (female-to-male ratio 2.8:1). The median age at presentation was 29 years (range, 18-59 years). Tumors location was: thoracoabdominal wall (n: 11, 47.8%), extremities (n: 7, 30.4%), abdominal cavity (n: 1; 4%), and head and neck (n: 4, 17.3%). Tumor sizes were documented in 18 cases and ranged from 3 to 20 cm in largest linear dimension (median, 10 cm). Eight (34.7%) female had pregnancy history and 2 (8%) had familial adenomatous polyposis history. Eleven (47,8%) underwent surgery as first-line treatment. Five (21.7%) patients received first-line treatment with vinblastine and methotrexate, four (17.3%) patients as second-line, and 14 (60.8%) patients as third and fourth-line. Fourteen (60.8%) patients had stable disease, four (17.3%) had partial response, and five (21.7%) patients had progressive disease during chemotherapy treatment. After a median follow-up of 58 months, 12 patients had progression disease and 2 patients died. The median PFS was 29 months, without any progression after 32 months.
Discussion: Weekly low-dose chemotherapy with vinblastine and methotrexate appears to have significant activity. Chemotherapy could be an acceptable alternative to radical surgery in selected patients with desmoid tumors.
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All authors have declared no conflicts of interest.