Evolving Practice Patterns Over Two Decades (1993-2013) in the Management of Desmoid-type Fibromatosis in British Columbia

摘要

abstract_textpAims: Due to the rarity and varied natural history of desmoid-type fibromatosis, evidence-based treatment standards for this disease remain lacking. This study evaluated outcomes in patients with desmoid-type fibromatosis managed at a Canadian institution over two decades./ppMaterials and methods: Records of 227 patients with desmoid-type fibromatosis referred from 1990 to 2013 were retrospectively reviewed to investigate management strategies including active surveillance, surgery, radiation therapy, cryoablation, and systemic therapy, including tamoxifen and chemotherapy./ppResults: Thirty-two per cent of cases were men, median age 40 years, median tumour size 5.4 cm. Initial treatments were surgery (79), tamoxifen (13), radiation therapy (5.0), chemotherapy (1.8) and cryoablation (1.2). Active surveillance was used upfront in 26 of cases, most after 2005. At a median followup of 77 months, one patient died of disease, 13 died of unrelated causes and the remainder were alive with no evidence of disease (56), stable/responding disease (33) or progressive disease (4). The recurrence rate was 25 after upfront surgery. Response rates and disease control rates were 40 and 76 for active surveillance; 68 and 96 for radiation therapy; 31 and 67 for tamoxifen; and 53 and 80 for chemotherapy. On univariable analysis, factors associated with a higher recurrence after initial surgery were young age (P = 0.012), male gender (P = 0.012) and extremity location (P = 0.005). On multivariable analysis, only young age was significantly associated with recurrence risk (P = 0.010)./ppConclusions: Active surveillance was associated with spontaneous regression and long-term disease control consistent with other studies. Primary radiation therapy appeared to provide a similar response and disease control compared with systemic treatments and may be a viable option for patients who are not candidates for surgery or active surveillance. (C) 2019 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved./p/abstract_text