Department of Surgical Oncology, Bone & Soft Tissue Service, Tata Memorial Hospital and Homi Bhabha National Institute (HBNI), Mumbai, 400012, India.
Department of Medicine, Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK.
Future Oncol. 2021 Sep;17(25):3397-3408. doi: 10.2217/fon-2021-0003. Epub 2021 Jul 6.
Desmoid fibromatosis is a locally aggressive tumor with an unpredictable clinical course. Surgery was once the mainstay of treatment, but the treatment protocol has been constantly evolving and currently active surveillance is the front-line approach. There have been significant insights into the molecular biology with the addition of mutational analysis of adding to prognostic information. We present a review of the literature with current practice guidelines, also including novel therapeutic targets and ongoing clinical trials, to unravel the next step in the management of sporadic desmoid fibromatosis.
硬纤维瘤病是一种局部侵袭性肿瘤，其临床病程不可预测。手术曾经是主要的治疗方法，但治疗方案一直在不断发展，目前主动监测是一线治疗方法。随着突变分析的加入，分子生物学有了重大的认识，增加了预后信息。我们对文献进行了回顾，并结合当前的实践指南，包括新的治疗靶点和正在进行的临床试验，以阐明散发性硬纤维瘤病治疗的下一步。
