Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult to completely eliminate them without adjacent healthy tissue injury. Surgical excision of these locally aggressive tumors without clear resection margins often leads to local recurrence. The aim of this thorough review was to assess the current treatment concepts for these rare tumors. A comprehensive search of articles published in the Cochrane Library, MEDLINE (PubMed), and EMBASE databases between January 2008 and February 2023 was conducted. Surgical intervention is no longer the first-line approach for most cases; instead, strategies like active surveillance or systemic therapies are used as initial treatment options. With the exception of EADTFs situated near vital structures, a minimum of 6–12 months of active surveillance is currently advocated for, during which some disease progression may be considered acceptable. Non-surgical interventions such as radiation or cryoablation may be employed in certain patients to achieve local control. The currently preferred systemic treatment options include tyrosine kinase inhibitors, low-dose chemotherapy, and gamma-secretase inhibitors, while hormone therapy is not advised. Nonsteroidal anti-inflammatory drugs are utilized primarily for pain management.
腹外型硬纤维瘤是一种罕见的单克隆成纤维细胞增殖性肿瘤，其特点是局部侵袭性强且具有明显的复发倾向。尽管该疾病属于良性范畴，但此类肿瘤易浸润周围正常组织，若不对邻近健康组织造成损伤则难以完全清除。对于这类局部侵袭性肿瘤，若手术切除未能达到清晰切缘，常导致局部复发。本综述旨在系统评估当前针对此类罕见肿瘤的治疗理念。通过对2008年1月至2023年2月期间收录于Cochrane图书馆、MEDLINE（PubMed）及EMBASE数据库的文献进行全面检索，发现手术干预已不再是多数病例的首选方案，取而代之的是主动监测或全身性治疗等初始治疗策略。除位于重要结构附近的腹外型硬纤维瘤外，目前普遍建议至少进行6-12个月的主动监测，在此期间可允许一定程度的疾病进展。对于特定患者可采用放射治疗或冷冻消融等非手术干预手段以实现局部控制。当前首选的全身治疗方案包括酪氨酸激酶抑制剂、低剂量化疗及γ-分泌酶抑制剂，而不建议采用激素疗法。非甾体抗炎药主要用于疼痛管理。
Current Treatment Concepts for Extra-Abdominal Desmoid-Type Fibromatosis: A Narrative Review
肿瘤(癌症)患者之家