Harvard Radiation Oncology Program, Boston, Massachusetts, USA.
Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Neuro Oncol. 2022 Dec 1;24(12):2180-2189. doi: 10.1093/neuonc/noac126.
Medulloblastoma (MB) is a rare central nervous system malignancy of adults, with limited contemporary studies to define treatment guidelines and expected late toxicity.
A single-center, retrospective study was conducted of patients age ≥18 years from 1997-2019 with MB and who were treated with postoperative radiotherapy. Late toxicity was defined as a minimum of 18 months from diagnosis. Overall survival (OS) and progression-free survival (PFS) were characterized using Kaplan-Meier and Cox regression analyses.
Fifty-nine patients met criteria, with median age of 25 years (range 18-62 y) and median follow-up of 6.5 years (range 0.7-23.1 y). At diagnosis, 68% were standard-risk, 88% Chang M0, and 22% with anaplastic histology. Gross total resection was achieved in 75%; median craniospinal irradiation dose was 30.6 Gy (relative biological effectiveness [RBE]), median total dose was 54.0 Gy (RBE), 80% received proton radiotherapy; 81% received chemotherapy. 5 year PFS and OS were 86.5% and 95.8%, respectively; 10 year PFS and OS were 83.9% and 90.7%, respectively. Anaplastic histology was associated with worse PFS (P = .04). Among eight recurrences, 25% presented after 5 years. Most common grade ≥2 late toxicities were anxiety/depressive symptoms (30%), motor dysfunction (25%), and ototoxicity (22%). Higher posterior fossa radiation dose was associated with increased risk of late toxicity, including worse cognitive dysfunction (P = .05).
Adults with MB have favorable survival outcomes, but late failures and toxicity are not uncommon. Better understanding of prognostic factors, possibly from molecular subtyping, may help to define more personalized treatments for patients with high risk of recurrence and long-term treatment sequelae.
成神经管细胞瘤(MB)是一种罕见的成人中枢神经系统恶性肿瘤,目前仅有有限的研究来定义治疗指南和预期的迟发性毒性。
对 1997 年至 2019 年间年龄≥18 岁、接受术后放疗的 MB 患者进行了一项单中心回顾性研究。迟发性毒性定义为从诊断起至少 18 个月。使用 Kaplan-Meier 和 Cox 回归分析来描述总生存率(OS)和无进展生存率(PFS)。
59 名患者符合标准,中位年龄为 25 岁(范围 18-62 岁),中位随访时间为 6.5 年(范围 0.7-23.1 年)。诊断时,68%为标准风险,88%为 Chang M0,22%为间变型组织学。75%的患者达到了大体全切除;中位颅脊髓照射剂量为 30.6Gy(相对生物效应[RBE]),总剂量中位数为 54.0Gy(RBE),80%的患者接受质子放疗,81%的患者接受化疗。5 年 PFS 和 OS 分别为 86.5%和 95.8%,10 年 PFS 和 OS 分别为 83.9%和 90.7%。间变性组织学与较差的 PFS 相关(P=0.04)。8 例复发中,25%发生在 5 年后。最常见的≥2 级迟发性毒性为焦虑/抑郁症状(30%)、运动功能障碍(25%)和耳毒性(22%)。后颅窝照射剂量较高与迟发性毒性风险增加相关,包括认知功能障碍恶化(P=0.05)。
成人 MB 患者的生存结果良好,但迟发性失败和毒性并不少见。更好地了解预后因素,可能来自分子分型,可能有助于为复发风险高和长期治疗后遗症的患者制定更个性化的治疗方案。