Desmoid tumors (DTs), also known as aggressive fibromatosis, are rare neoplasms characterized by local invasiveness and a high risk of recurrence, despite their lack of metastatic potential. The management of these tumors remains challenging due to their unpredictable behavior and heterogeneous presentations. In this two-part study, we first provide a comprehensive review of the scientific evidence on diagnosis and emerging therapeutic strategies for DT. In the second part, we will present a retrospective analysis of our experience at a national reference center for sarcoma treatment, focusing on diagnostic strategies, therapeutic interventions, and clinical outcomes.
硬纤维瘤(Desmoid tumors, DTs),亦称侵袭性纤维瘤病,是一种罕见肿瘤,其特征为局部侵袭性强且复发风险高,但无转移潜能。由于该肿瘤具有不可预测的生物学行为和异质性临床表现,其临床管理仍具挑战性。本研究分为两部分:首先系统综述硬纤维瘤诊断及新兴治疗策略的科学证据;第二部分将基于国家肉瘤诊疗参考中心的临床实践,回顾性分析诊断策略、治疗干预及临床结局。