Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis

AimVestibular 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 pro...

Full description

Bibliographic Details
Main Authors: Jiuhong Li, Qiguang Wang, Menglan Zhang, Guisheng Zhang, Si Zhang, Xuhui Hui
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-04-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2021.655260/full
id doaj-4d9c75f3043e4df289c78a573bb213bf
record_format Article
spelling doaj-4d9c75f3043e4df289c78a573bb213bf2021-04-14T05:19:39ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2021-04-011110.3389/fonc.2021.655260655260Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival AnalysisJiuhong Li0Jiuhong Li1Qiguang Wang2Menglan Zhang3Guisheng Zhang4Si Zhang5Xuhui Hui6Department of Neurosurgery of West China Hospital, Sichuan University, Chengdu, ChinaWest China School of Medicine, West China Hospital, Sichuan University, Chengdu, ChinaDepartment of Neurosurgery of West China Hospital, Sichuan University, Chengdu, ChinaDepartment of Pathology of West China Hospital, Sichuan University, Chengdu, ChinaDepartment of Neurosurgery of West China Hospital, Sichuan University, Chengdu, ChinaDepartment of Neurosurgery of West China Hospital, Sichuan University, Chengdu, ChinaDepartment of Neurosurgery of West China Hospital, Sichuan University, Chengdu, ChinaAimVestibular 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 MethodsThe 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.ResultsOf 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).ConclusionsMTVSs 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.https://www.frontiersin.org/articles/10.3389/fonc.2021.655260/fullvestibular schwannoma (acoustic neuroma)malignanttreatmentoutcomesprognostic factor
collection DOAJ
language English
format Article
sources DOAJ
author Jiuhong Li
Jiuhong Li
Qiguang Wang
Menglan Zhang
Guisheng Zhang
Si Zhang
Xuhui Hui
spellingShingle Jiuhong Li
Jiuhong Li
Qiguang Wang
Menglan Zhang
Guisheng Zhang
Si Zhang
Xuhui Hui
Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
Frontiers in Oncology
vestibular schwannoma (acoustic neuroma)
malignant
treatment
outcomes
prognostic factor
author_facet Jiuhong Li
Jiuhong Li
Qiguang Wang
Menglan Zhang
Guisheng Zhang
Si Zhang
Xuhui Hui
author_sort Jiuhong Li
title Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
title_short Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
title_full Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
title_fullStr Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
title_full_unstemmed Malignant Transformation in Vestibular Schwannoma: Clinical Study With Survival Analysis
title_sort malignant transformation in vestibular schwannoma: clinical study with survival analysis
publisher Frontiers Media S.A.
series Frontiers in Oncology
issn 2234-943X
publishDate 2021-04-01
description AimVestibular 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 MethodsThe 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.ResultsOf 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).ConclusionsMTVSs 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.
topic vestibular schwannoma (acoustic neuroma)
malignant
treatment
outcomes
prognostic factor
url https://www.frontiersin.org/articles/10.3389/fonc.2021.655260/full
work_keys_str_mv AT jiuhongli malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT jiuhongli malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT qiguangwang malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT menglanzhang malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT guishengzhang malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT sizhang malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
AT xuhuihui malignanttransformationinvestibularschwannomaclinicalstudywithsurvivalanalysis
_version_ 1721527627135582208