Department of Neurosurgery, School of Medicine, Emory University Hospital, 101 Woodruff Circle, Suite 6204, Atlanta, GA, 30322, USA.
Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, GA, USA.
Neurosurg Rev. 2020 Aug;43(4):1039-1046. doi: 10.1007/s10143-019-01135-y. Epub 2019 Jun 17.
Malignant peripheral nerve sheath tumors (MPNST) are a rare and aggressive group of tumors that are challenging to treat. Neurofibromatosis type 1 (NF-1)-associated MPNSTs have been associated with poorer clinical outcomes. The treatment options for NF-1-associated MPNSTs broadly include surgery (SG), chemotherapy (CT), and adjuvant radiotherapy (RT). Overall, the role and efficacy of CT and RT are unclear. Examination of existing literature for studies reporting on NF-1-associated MPNSTs and respective treatment-related outcomes was conducted. We conducted a systematic review according to PRISMA guidelines in PubMed/Medline and Cochrane databases of studies which reported treatment-specific outcomes in NF-1-associated MPNSTs. The literature search found 444 records after removal of duplicates. The present study included 50 patients across 12 observational studies. All of the included studies reported data on overall survival (OS 52%, n = 26/50) but mean follow-up in months among the studies and among patients varied widely, between 10.85 (SD, ± 10.38) and 192 (SD, ± 98.22). From the included studies, patients underwent either SG alone (n = 21), SG + CT (n = 10), SG + RT (n = 7), or SG + CT + RT (n = 12). The quality of evidence in the literature regarding optimal treatment options for NF-1-associated MPNSTs remains tenuous. Future retrospective and prospective comparative trials should consider adherence to a set of reporting guidelines to improve the quality of evidence in the literature with respect to individual treatment-related outcomes. The need for prospective multi-institutional efforts cannot be overstated.
恶性外周神经鞘瘤 (MPNST) 是一组罕见且侵袭性很强的肿瘤，治疗难度大。神经纤维瘤病 1 型 (NF-1) 相关的 MPNST 与较差的临床结局相关。NF-1 相关的 MPNST 的治疗选择包括手术 (SG)、化疗 (CT) 和辅助放疗 (RT)。总的来说，CT 和 RT 的作用和疗效尚不清楚。对报告 NF-1 相关 MPNST 及其各自治疗相关结局的现有文献进行了检查。我们按照 PRISMA 指南在 PubMed/Medline 和 Cochrane 数据库中进行了系统评价，以研究报告 NF-1 相关 MPNST 中特定治疗的结果。文献检索在去除重复项后发现了 444 条记录。本研究纳入了 12 项观察性研究中的 50 名患者。所有纳入的研究都报告了总体生存 (OS 52%，n=26/50) 的数据，但研究和患者之间的平均随访时间差异很大，从 10.85(SD，±10.38) 到 192(SD，±98.22)。从纳入的研究中，患者接受了单独的 SG(n=21)、SG+CT(n=10)、SG+RT(n=7)或 SG+CT+RT(n=12)。文献中关于 NF-1 相关 MPNST 最佳治疗选择的证据质量仍然脆弱。未来的回顾性和前瞻性比较试验应考虑遵守一套报告指南，以提高文献中关于个别治疗相关结局的证据质量。前瞻性多机构合作的必要性怎么强调都不为过。
