Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland; Department of Experimental and Clinical Physiology, Laboratory of Centre for Preclinical Research, Medical University of Warsaw, Warsaw, Poland.
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.
Surg Oncol. 2020 Dec;35:276-284. doi: 10.1016/j.suronc.2020.09.011. Epub 2020 Sep 12.
Malignant peripheral nerve sheath tumor (MPNST) accounts for about 5% of soft tissue sarcomas. It can occur as sporadic diseases or can be associated with type 1 neurofibromatosis. MPNST is usually associated with poor prognosis, mostly due to their aggressive behavior, high metastatic potential, and resistance to chemotherapy. Our study aimed to determine treatment outcomes and associated prognostic factors in a large cohort of patients with MPNSTs treated at the reference sarcoma center.
239 consecutive patients (114 women and 125 men) diagnosed with MPNST between March 1998 and March 2018 who were treated with surgery with curative intent in the reference sarcoma center were included in the retrospective analysis.
The mean age at diagnosis was 51 years (range 15-86). 28 (11.7%) patients had neurofibromatosis type 1 associated tumors (NF1 positive). Median OS was 126.5 months and 5-year survival rate was 61.9% in the group treated with curative intent. Median DFS, LRFS and DMFS were 91.6, 126.5 and 126.5 months, respectively. We identified tumor size, high tumor grade and positive surgical margins as independent negative predictors of DFS, LRFS, DMFS and OS.
High-quality surgery remains a gold standard of MPNST treatment. High grade, size and quality of surgery are significant independent prognostic factors for overall survival. There is an unmet need for improvement, especially regarding the perioperative treatment and treatment of metastatic disease. Future studies on the biology of MPNST would lead to the development of novel treatment options and improvement of treatment outcomes.
恶性外周神经鞘瘤（MPNST）约占软组织肉瘤的 5%。它可以作为散发性疾病发生，也可以与 1 型神经纤维瘤病相关。MPNST 通常与预后不良相关，主要是由于其侵袭性行为、高转移潜能和对化疗的耐药性。我们的研究旨在确定在参考肉瘤中心治疗的大量 MPNST 患者中的治疗结果和相关的预后因素。
回顾性分析了 1998 年 3 月至 2018 年 3 月期间在参考肉瘤中心接受以治愈为目的的手术治疗且被诊断为 MPNST 的 239 例连续患者（114 名女性和 125 名男性）。
诊断时的平均年龄为 51 岁（范围 15-86 岁）。28 例（11.7%）患者存在神经纤维瘤病 1 型相关肿瘤（NF1 阳性）。在接受以治愈为目的治疗的患者中，中位 OS 为 126.5 个月，5 年生存率为 61.9%。DFS、LRFS 和 DMFS 的中位值分别为 91.6、126.5 和 126.5 个月。我们发现肿瘤大小、高肿瘤分级和阳性手术切缘是 DFS、LRFS、DMFS 和 OS 的独立负预测因素。
高质量的手术仍然是 MPNST 治疗的金标准。高分级、肿瘤大小和手术质量是总生存的显著独立预后因素。需要改善治疗，特别是围手术期治疗和转移性疾病的治疗。未来对 MPNST 生物学的研究将导致新的治疗选择的发展，并改善治疗结果。
