Department of Radiation Oncology and ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India.
Department of Pathology Disease Management Group, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India.
J Adolesc Young Adult Oncol. 2022 Feb;11(1):68-77. doi: 10.1089/jayao.2021.0034. Epub 2021 Apr 23.
Medulloblastomas, comprising 20%-25% of all primary brain tumors in children are much rarer in adulthood. Disease biology varies substantially across different age groups; however, owing to rarity, adults with medulloblastoma are traditionally treated using pediatric protocols. This is a retrospective audit of adolescent and adult medulloblastoma from a comprehensive cancer center. Data regarding demography, clinical presentation, imaging characteristics, histopathological features, molecular profiling, risk stratification, treatment details, and outcomes were retrieved from medical records. All time-to-event outcomes were analyzed using Kaplan-Meier method and compared with the log-rank test. Univariate and multivariate analysis of relevant prognostic factors was done with value <0.05 being considered statistically significant. A total of 162 patients ≥15 years of age with medulloblastoma were included. The median age was 25 years (range: 15-59 years) with leptomeningeal metastases seen in 31 (19%) patients at initial diagnosis. Following surgery, patients were treated with appropriate risk-stratified adjuvant therapy comprising of craniospinal irradiation plus boost with or without systemic chemotherapy. At a median follow-up of 50 months, 5-year Kaplan-Meier estimates of progression-free survival and overall survival were 53.5% and 59.5%, respectively. The addition of adjuvant systemic chemotherapy did not impact upon survival in standard-risk medulloblastoma. High-risk (HR) disease and anaplastic histology emerged as significant and independent predictors of poor survival on multivariate analysis. Medulloblastoma is a rare tumor in adolescents and adults with key differences in disease biology and resultant outcomes compared with the pediatric population. Contemporary management comprising maximal safe resection followed by appropriate risk-stratified adjuvant therapy provides acceptable survival outcomes.
成神经管细胞瘤，占儿童所有原发性脑肿瘤的 20%-25%，在成年期则要少见得多。疾病生物学在不同年龄组之间有很大的差异；然而，由于其罕见性，传统上使用儿科方案治疗成人大脑成神经管细胞瘤。这是对综合癌症中心的青少年和成人大脑成神经管细胞瘤的回顾性审计。从病历中检索了有关人口统计学、临床表现、影像学特征、组织病理学特征、分子分析、风险分层、治疗细节和结果的数据。所有生存时间终点均采用 Kaplan-Meier 法进行分析，并与对数秩检验进行比较。对相关预后因素进行单因素和多因素分析，以 P 值<0.05 为有统计学意义。共有 162 名年龄≥15 岁的成人大脑成神经管细胞瘤患者被纳入。中位年龄为 25 岁（范围：15-59 岁），31 名（19%）患者在初次诊断时已有脑膜转移。手术后，患者接受了适当的风险分层辅助治疗，包括全脑脊髓照射加或不加全身化疗。中位随访 50 个月后，5 年无进展生存和总生存的 Kaplan-Meier 估计值分别为 53.5%和 59.5%。辅助全身化疗的加入并未影响标准风险成神经管细胞瘤的生存。高危（HR）疾病和间变组织学在多因素分析中是不良生存的显著且独立的预测因素。青少年和成人大脑成神经管细胞瘤是一种罕见的肿瘤，其疾病生物学与儿科人群有很大的不同，结果也不同。最大安全切除后辅以适当的风险分层辅助治疗是一种可行的治疗方法，可以提供可接受的生存结果。
