Trends in diagnostic and therapeutic strategies for extra-abdominal desmoid-type fibromatosis: Japanese musculoskeletal oncology group questionnaire survey

Abstract

Objective The mainstay of treatment modality for extra-abdominal desmoid-type fibromatosis (DF) has shifted from surgery, which often impairs ADL/QOL, to conservative treatment including active surveillance. In the present study, we conducted a longitudinal survey on the diagnosis and treatment of DF at facilities belonging to the Japanese Musculoskeletal Oncology Group, which is a research group of facilities specializing in the treatment of bone and soft tissue tumors in Japan to clarify the transition of medical care for extra-abdominal DF. Methods The same questionnaire was administered in 2015 and 2018, and responses were obtained from 46 (69%) of 67 facilities and 42 (53%) of 80 facilities in 2015 and 2018, respectively. Results Although immunostaining for β-catenin was often used for the pathological diagnosis in both 2015 and 2018, CTNNB1 mutation analysis was not performed either in 2015 or in 2018. As for the treatment strategy for resectable cases, surgical treatment including wide resection was selected at 11 facilities (24% of respondents) in 2015, and further decreased to 5 facilities (12%) in 2018. Conservative treatment with active surveillance or medical treatment was the most common treatment for both resectable and difficult-to-resect cases. COX-2 inhibitors and tranilast were often used in the drug treatment of both resectable and difficult-to-resect cases. Few facilities provided radiotherapy, methotrexate and vinblastine, or DOX-based chemotherapy for refractory cases in both 2015 and 2018. Conclusions A good trend was found in the questionnaire survey. It will be further necessary to disseminate clinical practice guidelines to physicians more widely, and to have them understand and implement the most up-to-date medical practice strategies for this rare disease.

Mesenchymal fibroblast cell promotes invasiveness of aggressive fibromatosis by remodeling extracellular matrix through CREB3L1/COL1A1 axis

“…With the deepening understanding of AF, treatment strategy has changed greatly in the past decade, which is manifested in the decline of surgery/radiotherapy and the improvement of drug therapy [41][42][43]. A "five-dimension" model was proposed by the collaboration group to guide drug selection [44], commonly used targeted drugs include imatinib, sorafenib, and Pezopanib [45][46][47][48][49][50].…”

Clinical results of active surveillance for extra‐abdominal desmoid‐type fibromatosis

“…Multiple medications were given to 20 lesions. A COX‐2 inhibitor was used for 116 lesions, tranilast for 26 lesions, 16 and tamoxifen for 3 lesions. No patients in this study cohort received radiotherapy.…”