Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis.
Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis.
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前庭神经鞘瘤的恶性转化：生存分析的临床研究
DOI：
10.3389/fonc.2021.655260
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发表时间：
2021
影响因子：
4.7
通讯作者：
Hui X
中科院分区：
文献类型：
--
作者：
Li J;Wang Q;Zhang M;Zhang G;Zhang S;Hui X
目的前庭神经鞘瘤（vestibularschwannomas，VS）通常被认为是良性肿瘤，而VS恶变（malignanttransformation，MTVS）是罕见的。其临床特征、治疗策略、结局和预后因素尚不清楚。我们建议分析MTVS的自然病史、处理、结果和预后因素。材料与方法回顾性分析2010年至2019年在我院治疗的4例MTVS患者的临床表现、影像学表现、病理检查和手术结果。评价了截至2019年12月发表的相关文献（63篇文章，67例患者）。作者还进行了汇总分析，以评估总生存期（OS）时间的风险因素。结果本组4例中，3例为手术及放疗后恶变，1例为原发性MTVS。在文献中的71例MTVS中，男性27例，女性39例，平均年龄为47.2 ± 17.5岁。12例患者（18.5%）被诊断为NF 2（15.4%）或NF 1（3.1%）。43例（61.4%）患者在病理诊断MTVS之前接受过既往治疗（手术和/或放疗）。MTVS的平均尺寸为35.1 ± 13.2mm。平均Ki-67指数为30.6% ± 18.8%。24例（49.0%）患者行大体全切除，25例（51.0%）患者行不完全切除。25例（44.6%）术后接受了辅助放疗（RT）。在平均9.9 ± 9.5个月（范围，0-40个月）的随访期间，37例（82.2%）患者发生局部复发或转移。47例（73.4%）患者死于肿瘤进展或术后并发症。总的1年和2年生存率分别为42.3%和18.6%。Kaplan-Meier生存分析的对数秩检验表明，大小（P = 0.047）和辅助放疗（P=0.001）是OS的重要预后因素。多变量分析显示，辅助RT是延长OS的唯一预后因素（P = 0.005）。结论MTVS是一种少见的致死性疾病，易复发，转移快，死亡率高。我们发现GTR并不能提高MTVS的生存率，但术后辅助RT可以显著提高OS，我们建议MTVS的早期术后RT，无论切除程度如何。
Aim Vestibular schwannomas (VSs) are generally considered benign tumors, and malignant transformation of VSs (MTVSs) are rare findings. The clinical features, treatment strategy, outcomes and prognostic factors remain unclear. We endeavored to analyze the natural history, management, outcomes and prognostic factors of MTVSs. Materials and Methods The clinical features, radiologic findings, pathological investigations and surgical outcomes of 4 patients with MTVSs treated at the authors’ institution between 2010 and 2019 were retrospectively collected. Related literature published until December 2019 (63 articles, 67 patients) was evaluated. The authors also made a pooled analysis to evaluate the risk factors for overall survival (OS) time. Results Of the 4 cases in our series, 3 cases were malignant transformation following previous treatment (surgery and radiosurgery) and 1 was primary MTVS. Of the 71 MTVSs from the literature, 27 were male and 39 were female, with the mean age of 47.2 ± 17.5 years old. Twelve patients (18.5%) were diagnosed with NF2 (15.4%) or NF1 (3.1%). Forty-three (61.4%) patients underwent previous treatment (surgery and/or radiotherapy) prior to the pathological diagnosis of MTVSs. The mean size of the MTVSs was 35.1 ± 13.2mm. The mean Ki-67 index was 30.6% ± 18.8%. Twenty-four (49.0%) patients underwent gross total resection, 25 (51.0%) patients underwent incomplete resection. Twenty-five (44.6%) underwent adjuvant radiotherapy (RT) postoperatively. During the average follow-up of 9.9 ± 9.5 months (range, 0-40 months), 37 (82.2%) patients developed a local recurrence or metastasis. Forty-seven (73.4%) patients died of tumor progression or postoperative complications. The overall 1-year and 2-year survival rate was 42.3% and 18.6% respectively. Log-rank testing for Kaplan-Meier survival analysis identified that size (P = 0.047) and adjuvant radiotherapy (P=0.001) were significant prognostic factors for OS. Multivariate analysis revealed that adjuvant RT was the only prognostic factor for longer OS (P = 0.005). Conclusions MTVSs are rare, fatal disease, prone to recur and metastasize rapidly, resulting in death in most of the cases. We found that GTR did not improve the survival in MTVSs but postoperative adjuvant RT can significantly improve the OS, and we recommend early postoperative RT in MTVSs regardless of extent of resection.
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