Kayani Babar, Sharma Aadhar, Sewell Mathew D, Platinum Johnson, Olivier Andre, Briggs Timothy W R, Eastwood Deborah M
The Royal National Orthopaedic Hospital, Stanmore.
Department of Spinal Surgery, The James Cook University Hospital, Middlesbrough, UK.
Am J Clin Oncol. 2018 Jul;41(7):687-694. doi: 10.1097/COC.0000000000000348.
Extrathoracic solitary fibrous tumors (ESFTs) are rare low-to-intermediate grade spindle-cell neoplasms of pluripotent fibroblastic or myofibroblastic origin. This review explores prognostic factors in the management of ESFTs and provides guidance on optimal treatment regimens based on the current literature.
Electronic searches were performed using MEDLINE, Embase, and the Cochrane library to identify studies on prognostic factors in the management of ESFTs published between January 1970 and June 2016. The literature search and review process identified 100 articles that were included in this review article. This included both surgical and nonsurgical studies on the management of ESFTs.
Surgical excision with wide resection margins forms the mainstay of treatment and provides optimal long-term oncological outcomes. Large tumor size (>5 to 10 cm diameter), inadequate resection margins, malignant histologic features, dedifferentiation, and tumor location within the abdomen/pelvis are associated with adverse oncological outcomes. Radiotherapy may be used for preoperative tumor shrinkage and/or as adjuvant therapy in patients with malignant disease or incomplete surgical margins. Chemotherapy with molecular-targeted therapies has produced promising results and the results of further phase 2 trials are awaited.
Routine long-term follow-up is essential for benign and malignant disease to enable early detection and treatment of recurrent disease.
胸外孤立性纤维性肿瘤（ESFTs）是一种罕见的起源于多能成纤维细胞或肌成纤维细胞的低至中等级别梭形细胞肿瘤。本综述探讨了ESFTs治疗中的预后因素，并根据现有文献为最佳治疗方案提供指导。
通过检索MEDLINE、Embase和Cochrane图书馆进行电子搜索，以确定1970年1月至2016年6月期间发表的关于ESFTs治疗中预后因素的研究。文献检索和综述过程确定了100篇纳入本综述文章的文献。这包括ESFTs治疗的手术和非手术研究。
采用宽切缘手术切除是主要治疗方法，并能提供最佳的长期肿瘤学结局。肿瘤体积大（直径>5至10 cm）、切缘不充分、恶性组织学特征、去分化以及肿瘤位于腹部/盆腔与不良肿瘤学结局相关。放疗可用于术前肿瘤缩小和/或作为恶性疾病或手术切缘不完整患者的辅助治疗。分子靶向治疗的化疗已取得了有前景的结果，有待进一步2期试验的结果。
对于良性和恶性疾病，常规长期随访对于早期发现和治疗复发性疾病至关重要。
