Takami Hirokazu, Matsutani Masao, Suzuki Tomonari, Takabatake Kazuhiko, Fujimaki Takamitsu, Okamoto Michinari, Yamaguchi Shigeru, Kanamori Masayuki, Matsuda Kenichiro, Sonoda Yukihiko, Natsumeda Manabu, Ichinose Toshiya, Nakada Mitsutoshi, Muroi Ai, Ishikawa Eiichi, Takahashi Masamichi, Narita Yoshitaka, Tanaka Shota, Saito Nobuhito, Higuchi Fumi, Shin Masahiro, Mineharu Yohei, Arakawa Yoshiki, Kagawa Naoki, Kawabata Shinji, Wanibuchi Masahiko, Takayasu Takeshi, Yamasaki Fumiyuki, Fujii Kentaro, Ishida Joji, Date Isao, Miyake Keisuke, Fujioka Yutaka, Kuga Daisuke, Yamashita Shinji, Takeshima Hideo, Shinojima Naoki, Mukasa Akitake, Asai Akio, Nishikawa Ryo
Department of Neurosurgery, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan (H.Takami, S.T., N.Saito).
Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center, Saitama, Japan (M.M., T.S., R.N.).
Neuro Oncol. 2025 Mar 7;27(3):828-840. doi: 10.1093/neuonc/noae229.
A previous Phase II clinical trial, conducted from 1995 to 2003, evaluated CNS germ cell tumors (GCTs) using a three-group treatment stratification based on histopathology. The primary objective of the study was to assess the long-term efficacy of standardized treatment regimens, while the secondary objective focused on identifying associated long-term complications.
A total of 228 patients were classified into 3 groups for treatment: germinoma (n = 161), intermediate prognosis (n = 38), and poor prognosis (n = 28), excluding one mature teratoma case. Treatment involved stratified chemotherapy regimens and varied radiation doses/coverage. Clinical data was retrospectively analyzed at a median follow-up of 18.5 years.
The treatment outcomes for germinoma, with or without syncytiotrophoblastic giant cells, were similar. The 10- and 20-year event-free survival rates for the germinoma, intermediate, and poor prognosis groups were 82/76/49% and 73/66/49%, respectively. Overall survival (OS) rates were 97/87/61% at 10 years and 92/70/53% at 20 years. Germinomas in the basal ganglia, treated without whole-brain radiation therapy (WBRT), frequently relapsed but were effectively managed with subsequent WBRT. Deaths in germinoma cases had varied causes, whereas deaths in the poor prognosis group were predominantly disease-related. Nineteen treatment-related complications were identified in 16 patients, with cumulative event rates of 1.9% at 10 years and 11.3% at 20 years. OS rates at 1 and 2 years post-relapse for tumors initially classified as germinoma, intermediate, and poor prognosis were 94/88/18% and 91/50/9%, respectively.
Initial treatment intensity is crucial for managing non-germinomatous GCTs, while long-term follow-up for relapse and complications is imperative in germinomas. Irradiation extending beyond the immediate tumor site is essential for basal ganglia germinomas. Addressing relapse in non-germinomatous GCT remains a significant challenge.
1995年至2003年进行的一项II期临床试验，基于组织病理学采用三组治疗分层评估中枢神经系统生殖细胞肿瘤（GCT）。该研究的主要目的是评估标准化治疗方案的长期疗效，次要目的集中于识别相关的长期并发症。
总共228例患者被分为3组进行治疗：生殖细胞瘤（n = 161）、预后中等（n = 38）和预后不良（n = 28），排除1例成熟畸胎瘤病例。治疗包括分层化疗方案以及不同的放射剂量/范围。在中位随访18.5年时对临床数据进行回顾性分析。
有或无合体滋养层巨细胞的生殖细胞瘤的治疗结果相似。生殖细胞瘤、预后中等和预后不良组的10年和20年无事件生存率分别为82%/76%/49%和73%/66%/49%。总生存率（OS）在10年时为97%/87%/61%，在20年时为92%/70%/53%。基底节区的生殖细胞瘤，在未进行全脑放射治疗（WBRT）的情况下治疗，经常复发，但随后通过WBRT有效控制。生殖细胞瘤病例的死亡原因各不相同，而预后不良组的死亡主要与疾病相关。在16例患者中识别出19例与治疗相关的并发症，10年时累积事件发生率为1.9%，20年时为11.3%。最初分类为生殖细胞瘤、预后中等和预后不良的肿瘤在复发后1年和2年的OS率分别为94%/88%/18%和91%/50%/9%。
初始治疗强度对于非生殖细胞性GCT的管理至关重要，而生殖细胞瘤复发和并发症的长期随访必不可少。对于基底节区生殖细胞瘤，超出肿瘤直接部位的照射至关重要。解决非生殖细胞性GCT的复发仍然是一项重大挑战。
