Kanamori Masayuki, Kumabe Toshihiro, Saito Ryuta, Yamashita Yoji, Sonoda Yukihiko, Ariga Hisanori, Takai Yoshihiro, Tominaga Teiji
Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
J Neurosurg Pediatr. 2009 Dec;4(6):506-14. doi: 10.3171/2009.7.PEDS08288.
This study retrospectively analyzed the long-term outcomes of 108 consecutive patients to establish the classification and optimal treatment strategy for each subgroup of newly diagnosed germ cell tumors (GCTs).
A retrospective review of medical records from the authors' department between April 1989 and March 2007 identified 108 patients with newly diagnosed intracranial GCT. The diagnoses were germinoma in 83 patients, and nongerminomatous GCT (NGGCT) in 25 patients.
In patients with germinoma, the 10-year overall and progression-free survival (PFS) rates at a median follow-up period of 99 months were 86 and 74%, respectively. Recurrences developed during a range of 6 to 153 months (median 26 months) after starting the initial therapy. Patients treated only with chemotherapy demonstrated a shorter PFS rate, and patients treated with chemotherapy followed by reduced-dose radiation therapy to the whole ventricle, whole brain, or craniospinal axis showed significantly better PFS than patients treated with only radiation or reduced-dose radiation therapy to the focal field. Nongerminomatous GCTs were divided into good, intermediate, and poor prognosis groups as proposed by the Japanese Pediatric Brain Tumor Study Group. In the good and intermediate prognosis groups, the 10-year overall and PFS rates were 100 and 93%, respectively. In the poor prognosis group, the 3-year overall and PFS rates were 56 and 29%, respectively. All patients with NGGCTs, in whom the lesions on MR imaging disappeared after combination therapies consisting of resection, radiation therapy, and chemotherapy, remained alive.
Chemotherapy followed by reduced-dose radiation therapy covering the whole ventricle improves the prognosis for patients with germinoma. Combined therapy of radiation therapy, chemotherapy, and radical resection as an initial or salvage treatment achieved excellent tumor control in the intermediate prognosis NGGCT group. The outcomes were still dismal in the poor prognosis NGGCT group, so initial therapy should target complete disappearance of all lesions on MR imaging.
本研究对108例连续患者的长期预后进行回顾性分析,以确立新诊断的生殖细胞肿瘤(GCT)各亚组的分类及最佳治疗策略。
回顾性分析作者所在科室1989年4月至2007年3月间的病历,确定108例新诊断的颅内GCT患者。其中83例为生殖细胞瘤,25例为非生殖细胞性GCT(NGGCT)。
生殖细胞瘤患者中,中位随访期99个月时,10年总生存率和无进展生存率(PFS)分别为86%和74%。复发发生在初始治疗开始后的6至153个月(中位26个月)。单纯化疗的患者PFS较短,而化疗后对全脑室、全脑或全脊髓轴进行减量放疗的患者,其PFS明显优于单纯放疗或局部野减量放疗的患者。非生殖细胞性GCT根据日本小儿脑肿瘤研究组的提议分为预后良好、中等和不良组。在预后良好和中等组中,10年总生存率和PFS分别为100%和93%。在预后不良组中,3年总生存率和PFS分别为56%和29%。所有接受手术切除、放疗和化疗联合治疗后磁共振成像(MR)上病变消失的NGGCT患者均存活。
化疗后对全脑室进行减量放疗可改善生殖细胞瘤患者的预后。放疗、化疗及根治性手术联合治疗作为初始或挽救性治疗,在预后中等的NGGCT组中实现了良好的肿瘤控制。预后不良的NGGCT组预后仍然很差,因此初始治疗应旨在使MR成像上所有病变完全消失。