青少年和年轻成人颅内生殖细胞肿瘤：40 年多机构回顾性研究结果。

Intracranial Germ Cell Tumors in Adolescents and Young Adults: A 40-Year Multi-Institutional Review of Outcomes.

机构信息

Princess Margaret Cancer Centre, Toronto, Ontario; British Columbia Cancer, Vancouver, British Columbia; University of Toronto, Toronto, Ontario; University of British Columbia, Vancouver, British Columbia.

Princess Margaret Cancer Centre, Toronto, Ontario; University of Toronto, Toronto, Ontario; The Hospital for Sick Children, Toronto, Ontario.

出版信息

Int J Radiat Oncol Biol Phys. 2020 Feb 1;106(2):269-278. doi: 10.1016/j.ijrobp.2019.10.020. Epub 2019 Oct 22.

DOI:10.1016/j.ijrobp.2019.10.020

PMID:31654785

Abstract

PURPOSE

The aim of this study was to determine the practice patterns and outcomes of intracranial germ cell tumors (IGCT) in adolescents and young adults according to different therapeutic approaches.

METHODS AND MATERIALS

One-hundred twelve patients with IGCT aged 15 to 39 years were managed at either XX or the XY center from 1975 to 2015. The charts were retrospectively reviewed and data collected.

RESULTS

Median duration of follow-up was 8.3 years. Ninety-four patients had germinomas, and 18 had nongerminomatous germ cell tumors (NGGCT). The primary disease sites were pineal gland (37 of 94 germinoma, 14 of 18 NGGCT) and suprasellar region (23 of 94 germinoma, 2 of 18 NGGCT). Eleven patients with germinoma (12%) and 2 patients with NGGCT (11%) had radiographic spinal metastases or positive lumbar cerebrospinal fluid cytology. Event-free survival (EFS) was 84% and overall survival (OS) was 90% at 10 years for germinoma; EFS was 71% and OS was 86% at 10 years for NGGCT. For patients with germinoma, 10-year EFS was 100% after craniospinal radiation therapy (CSRT) with chemotherapy (N = 10); 100% after whole-ventricular radiation therapy (WVRT), whole-brain radiation therapy (WBRT), or focal radiation therapy (FRT) with chemotherapy (N = 22); 90% after CSRT alone (N = 46); and 41% after WVRT, WBRT, or FRT alone (N = 16) (P < .0005). Ten-year OS was 100%, 100%, 90%, and 72%, respectively (P = .032). For patients with NGGCT, 10-year EFS was 80% after CSRT, WBRT, or WVRT plus chemotherapy (N = 10) versus 58% after FRT plus chemotherapy (N = 8) (P = .31); 10-year OS was 90% versus 58%, respectively (P = .16).

CONCLUSIONS

We report excellent overall outcomes according to treatment approach in the largest study of IGCT in adolescents and young adults to our knowledge. EFS and OS were inferior after non-CSRT without chemotherapy in germinoma.

摘要

目的

本研究旨在根据不同的治疗方法，确定青少年和年轻成人颅内生殖细胞肿瘤（IGCT）的治疗模式和结果。

方法和材料

1975 年至 2015 年，我们在 XX 或 XY 中心对 15 至 39 岁的 112 名 IGCT 患者进行了管理。对这些患者的病历进行了回顾性分析，并收集了数据。

结果

中位随访时间为 8.3 年。94 例患者为生殖细胞瘤，18 例为非生殖细胞瘤性生殖细胞肿瘤（NGGCT）。主要疾病部位为松果体（37 例生殖细胞瘤，14 例 NGGCT）和鞍上区（23 例生殖细胞瘤，2 例 NGGCT）。11 例生殖细胞瘤患者（12%）和 2 例 NGGCT 患者（11%）出现了放射性脊柱转移或阳性腰椎脑脊液细胞学检查。生殖细胞瘤患者的无事件生存（EFS）为 84%，10 年总生存（OS）为 90%；NGGCT 患者的 EFS 为 71%，OS 为 86%。对于生殖细胞瘤患者，在接受化疗的颅脊髓放疗（CSRT）后，10 年 EFS 为 100%（N=10）；在接受全脑室放疗（WVRT）、全脑放疗（WBRT）或局部放疗（FRT）加化疗后，10 年 EFS 为 100%（N=22）；在接受 CSRT 单一治疗后，10 年 EFS 为 90%（N=46）；在接受 WVRT、WBRT 或 FRT 单一治疗后，10 年 EFS 为 41%（N=16）（P<0.0005）。10 年 OS 分别为 100%、100%、90%和 72%（P=0.032）。对于 NGGCT 患者，在接受 CSRT、WBRT 或 WVRT 联合化疗后，10 年 EFS 为 80%（N=10），而在接受 FRT 联合化疗后，10 年 EFS 为 58%（N=8）（P=0.31）；10 年 OS 分别为 90%和 58%（P=0.16）。