侵袭性纤维瘤病多学科综合诊疗研究进展CSCD

侵袭性纤维瘤病(aggressive fibromatosis,AF)是一种临床罕见的、起源于软组织的交界性肿瘤,尽管不具备远处转移能力,然而其局部侵袭性强,手术切除后复发率高,使临床治疗存在一定难度。近些年,随着对AF研究的不断深入,其治疗措施与管理模式发生了较大的变化。鉴于AF不可预测的自然病史,目前观察等待是指南推荐的一线治疗方案。此外,目前以手术根治性切除为主联合局部与全身系统性治疗等多学科综合诊疗模式可以在保留患者组织器官功能的同时,最大程度地控制肿瘤,延缓疾病的进展甚至达到根治。本文就AF的临床特征与诊疗进展展开综述。

Research Progress of Multidisciplinary Comprehensive Diagnosis and Treatment ofAggressive Fibromatosis

Aggressive fibromatosis (AF) is a clinically rare borderline tumor originating in soft tissue. Although AF does not have the ability of distant metastasis, it is locally aggressive and has a high recurrence rate after surgical resection, which makes clinical treatment difficult. In recent years, with the continuously deepening research on AF, great changes have taken place in its treatment measures and management models. Due to the unpredictable natural history of AF, wait-and-see is currently the first-line treatment recommended by the guidelines. In addition, the current multidisciplinary comprehensive diagnosis and treatment mode which mainly focuses on surgical radical resection combined with local and systemic treatment can control the tumor to the greatest extent, delay the progress of the disease and even achieve radical treatment while preserving the function of the tissues and organs. This article reviews the clinical features and the research progress in diagnosis and treatment of AF.