摘要
韧带样纤维瘤病(desmoid fibromatosis,DF),又称侵袭性纤维瘤病(aggressive fibromatosis,AF)、硬纤维瘤(desmoid tumor,DT),是一种罕见疾病,组织学上表现为单克隆成纤维细胞增生,临床上不同患者自然病程各异[1]。据报道,DF的年发病率约为0.4/10万[2],该病通常在30~40岁左右出现发病高峰,而在育龄期女性中更为常见。患者常因慢性疼痛、功能障碍、心理问题以及生活质量普遍下降而受到日常生活的限制。尽管DF呈现出侵袭性、浸润性生长的临床特征,且易复发,但并不具有远处转移的潜能,属于交界性软组织肿瘤,通常并不危及生命[3]。长期以来,外科治疗一直被视为DF的首选治疗方法,甚至在经典的《Musculoskeletal Tumor Surgery》一书中,也通过一个反复复发的DF病例最终接受截肢来说明“Law of Three”外科治疗原则[4]。然而,近年来随着对该疾病分子机制和临床病程的认识不断深入以及药物临床试验的开展,DF的治疗策略已逐渐发生根本性的理念转变[5-8]。现对DF的几个关键问题进行述评。
Desmoid fibromatosis is a rare soft tissue tumor characterized by chronic pain and functional impairment,impacting patients'quality of life.Despite its invasive and recurrent nature,it typically does not metastasize and is not life-threatening.Treatment strategies have shifted from predominantly surgical intervention in the past to active surveillance,with consideration of pharmacotherapy,surgical intervention,radiotherapy,or their combinations.This article provides an overview of several key issues in the management of desmoid fibromatosis.
作者
徐海荣
牛晓辉
XU Hai-rong;NIU Xiao-hui(Department of Orthopaedic Oncology Surgery,Beijing Jishuitan Hospital,Capital Medical University,Beijing,100035,China)
出处
《中国骨与关节杂志》
CAS
2024年第5期321-324,共4页
Chinese Journal of Bone and Joint
基金
北京积水潭医院“学科骨干”人才资助项目(XKGG202106)
北京积水潭医院院级科研基金创新转化项目(LH-202203)。
