Anna Michel, Jan Rodemerk, Laurèl Rauschenbach, Pikria Ketelauri, Oleh Danylyak, Ramazan Jabbarli, Philipp Dammann, Anne-Kathrin Uerschels, Marvin Darkwah Oppong, Oliver Gembruch, Yahya Ahmadipour, Andreas Junker, Ulrich Sure, Karsten Henning Wrede
{"title":"中枢神经细胞瘤的治疗。","authors":"Anna Michel, Jan Rodemerk, Laurèl Rauschenbach, Pikria Ketelauri, Oleh Danylyak, Ramazan Jabbarli, Philipp Dammann, Anne-Kathrin Uerschels, Marvin Darkwah Oppong, Oliver Gembruch, Yahya Ahmadipour, Andreas Junker, Ulrich Sure, Karsten Henning Wrede","doi":"10.3390/cancers17122005","DOIUrl":null,"url":null,"abstract":"<p><p><b>Objective:</b> Central neurocytomas (CNs), classified as CNS (central nervous system) grade 2 tumors, are exceptionally rare tumors, accounting for approximately 0.1-0.5% of all intracranial neoplasms, and are typically characterized by a benign clinical course and frequent association with hydrocephalus. This study aims to present a comprehensive analysis of surgical and adjuvant therapies for CN. <b>Methods:</b> The study comprised all patients who underwent microsurgical tumor removal in our center over the past decade (2013-2023). Clinical manifestations, surgical and adjuvant therapy approaches, MRI and histological findings, clinical outcomes, and recurrence-free survival were evaluated. <b>Results:</b> A total of eleven patients (six men, mean age of 28.0 years; five women, mean age of 53.6 years) underwent surgical treatment. Intraventricular tumors were the most common (72.7%, n = 8). The predominant presenting symptoms were headache and visual disturbances. All tumors exhibited contrast enhancement on MRI. Hydrocephalus was present in five patients. The Ki67 proliferation index ranged from 2% to 10%, with nine patients exhibiting Ki67 > 3%. The median recurrence-free survival was 38.0 months (IQR: 25.0-53.0). The most severe postoperative complications included aphasia, hemiparesis, and memory impairment, resulting in a postoperative Karnofsky Performance Status (KPS) below 70% in five patients. Follow-up assessments showed significant symptomatic improvement in all affected patients. <b>Conclusions:</b> Gross total resection is the recommended first-line therapy with favorable neurological outcomes and for atypical CN as well. Adjuvant radiotherapy should be reserved for tumor progression and recurrence. The role of adjuvant chemotherapy remains unclear, but it may be an option for CN with a high proliferation index.</p>","PeriodicalId":9681,"journal":{"name":"Cancers","volume":"17 12","pages":""},"PeriodicalIF":4.5000,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12190501/pdf/","citationCount":"0","resultStr":"{\"title\":\"Treatment of Central Neurocytoma.\",\"authors\":\"Anna Michel, Jan Rodemerk, Laurèl Rauschenbach, Pikria Ketelauri, Oleh Danylyak, Ramazan Jabbarli, Philipp Dammann, Anne-Kathrin Uerschels, Marvin Darkwah Oppong, Oliver Gembruch, Yahya Ahmadipour, Andreas Junker, Ulrich Sure, Karsten Henning Wrede\",\"doi\":\"10.3390/cancers17122005\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p><b>Objective:</b> Central neurocytomas (CNs), classified as CNS (central nervous system) grade 2 tumors, are exceptionally rare tumors, accounting for approximately 0.1-0.5% of all intracranial neoplasms, and are typically characterized by a benign clinical course and frequent association with hydrocephalus. This study aims to present a comprehensive analysis of surgical and adjuvant therapies for CN. <b>Methods:</b> The study comprised all patients who underwent microsurgical tumor removal in our center over the past decade (2013-2023). Clinical manifestations, surgical and adjuvant therapy approaches, MRI and histological findings, clinical outcomes, and recurrence-free survival were evaluated. <b>Results:</b> A total of eleven patients (six men, mean age of 28.0 years; five women, mean age of 53.6 years) underwent surgical treatment. Intraventricular tumors were the most common (72.7%, n = 8). The predominant presenting symptoms were headache and visual disturbances. All tumors exhibited contrast enhancement on MRI. Hydrocephalus was present in five patients. The Ki67 proliferation index ranged from 2% to 10%, with nine patients exhibiting Ki67 > 3%. The median recurrence-free survival was 38.0 months (IQR: 25.0-53.0). The most severe postoperative complications included aphasia, hemiparesis, and memory impairment, resulting in a postoperative Karnofsky Performance Status (KPS) below 70% in five patients. Follow-up assessments showed significant symptomatic improvement in all affected patients. <b>Conclusions:</b> Gross total resection is the recommended first-line therapy with favorable neurological outcomes and for atypical CN as well. Adjuvant radiotherapy should be reserved for tumor progression and recurrence. The role of adjuvant chemotherapy remains unclear, but it may be an option for CN with a high proliferation index.</p>\",\"PeriodicalId\":9681,\"journal\":{\"name\":\"Cancers\",\"volume\":\"17 12\",\"pages\":\"\"},\"PeriodicalIF\":4.5000,\"publicationDate\":\"2025-06-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12190501/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cancers\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.3390/cancers17122005\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"ONCOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cancers","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3390/cancers17122005","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
目的:中枢神经细胞瘤(Central neurocytomas, CNs)属于中枢神经系统2级肿瘤,是一种极为罕见的肿瘤,约占所有颅内肿瘤的0.1-0.5%,临床表现为良性,常伴有脑积水。本研究旨在对CN的手术和辅助治疗进行综合分析。方法:本研究纳入近十年(2013-2023年)在我中心行显微外科肿瘤切除术的所有患者。评估临床表现、手术和辅助治疗方法、MRI和组织学结果、临床结果和无复发生存期。结果:共11例患者(男性6例,平均年龄28.0岁;5名女性,平均年龄53.6岁)接受了手术治疗。脑室内肿瘤最常见(72.7%,n = 8)。主要表现为头痛和视力障碍。所有肿瘤MRI表现为对比增强。5例患者出现脑积水。Ki67增殖指数从2%到10%不等,9例患者Ki67增殖指数为3%。中位无复发生存期为38.0个月(IQR: 25.0-53.0)。最严重的术后并发症包括失语、偏瘫和记忆障碍,导致5例患者术后Karnofsky Performance Status (KPS)低于70%。随访评估显示,所有受影响患者的症状均有显著改善。结论:对于非典型CN而言,全切除是推荐的一线治疗方法,具有良好的神经系统预后。辅助放疗应保留在肿瘤进展和复发时。辅助化疗的作用尚不清楚,但对于增殖指数高的CN可能是一种选择。
Objective: Central neurocytomas (CNs), classified as CNS (central nervous system) grade 2 tumors, are exceptionally rare tumors, accounting for approximately 0.1-0.5% of all intracranial neoplasms, and are typically characterized by a benign clinical course and frequent association with hydrocephalus. This study aims to present a comprehensive analysis of surgical and adjuvant therapies for CN. Methods: The study comprised all patients who underwent microsurgical tumor removal in our center over the past decade (2013-2023). Clinical manifestations, surgical and adjuvant therapy approaches, MRI and histological findings, clinical outcomes, and recurrence-free survival were evaluated. Results: A total of eleven patients (six men, mean age of 28.0 years; five women, mean age of 53.6 years) underwent surgical treatment. Intraventricular tumors were the most common (72.7%, n = 8). The predominant presenting symptoms were headache and visual disturbances. All tumors exhibited contrast enhancement on MRI. Hydrocephalus was present in five patients. The Ki67 proliferation index ranged from 2% to 10%, with nine patients exhibiting Ki67 > 3%. The median recurrence-free survival was 38.0 months (IQR: 25.0-53.0). The most severe postoperative complications included aphasia, hemiparesis, and memory impairment, resulting in a postoperative Karnofsky Performance Status (KPS) below 70% in five patients. Follow-up assessments showed significant symptomatic improvement in all affected patients. Conclusions: Gross total resection is the recommended first-line therapy with favorable neurological outcomes and for atypical CN as well. Adjuvant radiotherapy should be reserved for tumor progression and recurrence. The role of adjuvant chemotherapy remains unclear, but it may be an option for CN with a high proliferation index.
期刊介绍:
Cancers (ISSN 2072-6694) is an international, peer-reviewed open access journal on oncology. It publishes reviews, regular research papers and short communications. Our aim is to encourage scientists to publish their experimental and theoretical results in as much detail as possible. There is no restriction on the length of the papers. The full experimental details must be provided so that the results can be reproduced.