Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA.
Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
J Neurooncol. 2018 Jan;136(1):95-104. doi: 10.1007/s11060-017-2627-1. Epub 2017 Oct 10.
Medulloblastoma (MB) is rare in adults and treatment guidelines are consequently not well-established. Few modern series have reported long-term follow-up and treatment sequelae. We examined long-term outcomes of adult MB patients at one institution. Records of 29 consecutive patients (18 male, 11 female) aged ≥ 18 years who received radiotherapy (RT) for primary MB from 1990 to 2016 were reviewed. Median age at diagnosis was 28 years (range 18-72 years). Seventeen patients were standard risk and 12 were high risk. Nineteen patients had gross total resection, seven had subtotal resection, and three had biopsy only. Median craniospinal irradiation and boost doses were 36 Gy (range 23.4-39.6 Gy) and 55.8 Gy (range 54-59.4 Gy), respectively. Of 24 patients receiving chemotherapy, 20 received concurrent + adjuvant and 4 received adjuvant only. At median follow-up of 9.0 years (range 1.1-20.5 years), five patients recurred: four in the posterior fossa and one in both the posterior fossa and above the tentorium. Five patients died: two of disease progression and three after possible treatment complications (seizure, lobar pneumonia, and multifactorial sepsis). At last follow-up, 23 patients were alive with no evidence of disease. Long-term effects include executive dysfunction (n = 17), weakness/ataxia (n = 16), and depression/anxiety (n = 13). Kaplan-Meier estimates of 10-year overall survival and failure-free survival are 83% (95% confidence interval [CI] 59-93%) and 79% (CI 55-91%), respectively. Despite encouraging disease control in this cohort, long-term sequelae may limit quality of life. Multimodality pediatric regimens using lower RT doses may be considered to reduce treatment-related morbidity.
髓母细胞瘤(MB)在成年人中较为罕见,因此治疗指南尚未完善。很少有现代系列报道长期随访和治疗后遗症。我们在一家机构检查了成年 MB 患者的长期预后。回顾了 1990 年至 2016 年期间因原发性 MB 接受放疗(RT)的 29 例连续患者(18 名男性,11 名女性)的记录。诊断时的中位年龄为 28 岁(范围 18-72 岁)。17 例为标准风险,12 例为高风险。19 例患者行全切除,7 例次全切除,3 例仅行活检。中位颅脊髓照射和推量剂量分别为 36 Gy(范围 23.4-39.6 Gy)和 55.8 Gy(范围 54-59.4 Gy)。24 例接受化疗的患者中,20 例接受同期+辅助治疗,4 例仅接受辅助治疗。中位随访时间为 9.0 年(范围 1.1-20.5 年),5 例患者复发:4 例在后颅窝,1 例在后颅窝和天幕上。5 例患者死亡:2 例死于疾病进展,3 例死于可能的治疗并发症(癫痫、大叶性肺炎和多因素败血症)。最后一次随访时,23 例患者无疾病存活。长期影响包括执行功能障碍(n=17)、无力/共济失调(n=16)和抑郁/焦虑(n=13)。10 年总生存率和无失败生存率的 Kaplan-Meier 估计值分别为 83%(95%置信区间 [CI] 59-93%)和 79%(CI 55-91%)。尽管该队列的疾病控制令人鼓舞,但长期后遗症可能会限制生活质量。使用较低 RT 剂量的多模式儿科方案可能有助于降低治疗相关发病率。