Background/Objectives: Adult medulloblastoma is a rare entity, with management data extrapolated from pediatric medulloblastoma cases. We aim to report the clinical characteristics, prognostic factors, and treatment outcome of a cohort of adult patients with medulloblastoma.Methods: Fifty-three patients aged ≥ 18 years with medulloblastoma treated at King Hussein Cancer Center between 2007 and 2019 were retrospectively reviewed. Patients’ diseases were staged according to modified Chang’s staging system. All patients received adjuvant craniospinal irradiation followed by a posterior fossa boost. Baseline disease characteristics, including molecular subgrouping, were tested as prognostic factors of progression-free survival (PFS) and overall survival (OS) by using univariate analysis.Results: Median follow-up was 70 months (range 37.5–104.5 months). Twenty-two tumors were of the SHH-activated subtype. Conversely, WNT-activated and group 4 tumors had three cases each. Only 37.7% of patients died. The mean 3-year, 5-year, and 10-year OS were 85% (75–95%), 74% (62–87%), and 50% (33–75%), respectively. Significant differences in OS were associated with the extent of surgery (p= 0.017), M stage (p= 0.009), and risk status (p< 0.001). Relapses were detected in 28.3% of cases. The 3-year, 5-year, and 10-year PFS were 81% (71–92%), 75% (63–88%), and 66% (52–83%), respectively. Significant differences in PFS were associated with the extent of surgery (p= 0.008) and risk status (p= 0.012). Molecular subgrouping did not correlate with OS or PFS.Conclusions: Our results revealed poor survival of patients with high-risk disease, which may necessitate the intensification of chemotherapy. Molecular subgrouping did not correlate with the outcome in this cohort.
背景/目的：成人髓母细胞瘤较为罕见，其治疗数据多从儿童病例中推导得出。本研究旨在报告一组成人髓母细胞瘤患者的临床特征、预后因素及治疗结果。方法：回顾性分析2007年至2019年间在侯赛因国王癌症中心接受治疗的53例年龄≥18岁的髓母细胞瘤患者。根据改良Chang分期系统对患者疾病进行分期。所有患者均接受辅助性全脑全脊髓放疗及后颅窝推量照射。通过单因素分析检验基线疾病特征（包括分子亚型）对无进展生存期（PFS）和总生存期（OS）的预后影响。结果：中位随访时间为70个月（范围37.5-104.5个月）。22例肿瘤为SHH激活亚型，WNT激活亚型与第4组亚型各3例。患者总死亡率为37.7%。3年、5年和10年平均OS分别为85%（75-95%）、74%（62-87%）和50%（33-75%）。手术切除范围（p=0.017）、M分期（p=0.009）和风险状态（p<0.001）与OS存在显著相关性。28.3%的病例出现复发。3年、5年和10年PFS分别为81%（71-92%）、75%（63-88%）和66%（52-83%）。手术切除范围（p=0.008）和风险状态（p=0.012）与PFS存在显著相关性。分子亚型与OS或PFS无显著相关性。结论：本研究显示高危疾病患者生存率较差，可能需要强化化疗方案。在该队列中，分子亚型与治疗结局无显著相关性。
肿瘤(癌症)患者之家