Kunschner L J, Kuttesch J, Hess K, Yung W K
Department of Neuro-Oncology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Neuro Oncol. 2001 Jul;3(3):167-73. doi: 10.1093/neuonc/3.3.167.
Medulloblastoma is a rare adult primary brain tumor for which limited retrospective studies are available to elucidate natural history or to guide therapy. A retrospective chart and imaging review of adult patients (aged >18 years) with medulloblastoma was performed to identify survival and prognostic factors. Fifty-seven patients were evaluated at the University of Texas M.D. Anderson Cancer Center from 1978-1998. Statistical analysis of prognostic factors and overall survival was performed for a subgroup of 28 patients who were followed exclusively at our institution from the time of diagnosis until death or last follow-up. These 28 patients had an overall survival of 91% at 3 years and 84% at 5 years, whereas median survival was not reached after a median follow-up of 168 weeks (range, 9-602 weeks). Progression-free survival for all patients was 68% at 3 years and 62% at 5 years, and was not statistically different between poor- and standard-risk patients. Univariate analysis of clinical features, such as age, sex, extent of local disease, extent of resection, and use of adjuvant chemotherapy, did not identify any prognostic variables for survival among the 28 patients. Patterns of recurrence revealed that the posterior fossa was the most common site (56%), followed by bone marrow (25%). Adult medulloblastoma appears to have a favorable prognosis after treatment with maximally surgically feasible resection followed by craniospinal irradiation. Optimal treatment remains to be clarified, as both standard-risk and poor-risk patients have prolonged disease-free survival. The marked difference between survival and progression-free survival suggests that salvage therapy, usually with combination chemotherapy in this cohort of patients, is of benefit. More formal analysis of the survival benefit was not possible, however, because of the small number of patients treated at recurrence with any one therapeutic regimen.
髓母细胞瘤是一种罕见的成人原发性脑肿瘤,目前仅有有限的回顾性研究可用于阐明其自然病史或指导治疗。我们对成年(年龄>18岁)髓母细胞瘤患者进行了回顾性病历和影像学检查,以确定生存及预后因素。1978年至1998年期间,德克萨斯大学MD安德森癌症中心对57例患者进行了评估。对其中28例从诊断至死亡或最后随访一直在我们机构接受随访的患者亚组进行了预后因素及总生存的统计分析。这28例患者3年总生存率为91%,5年为84%,而在中位随访168周(范围9 - 602周)后未达到中位生存期。所有患者的无进展生存率3年时为68%,5年时为62%,低危和标准风险患者之间无统计学差异。对年龄、性别、局部病变范围、切除范围及辅助化疗使用等临床特征进行单因素分析,未在这28例患者中发现任何生存预后变量。复发模式显示后颅窝是最常见部位(56%),其次是骨髓(25%)。经最大程度手术切除后行全脑全脊髓放疗,成人髓母细胞瘤似乎预后良好。由于标准风险和低危患者均有延长的无病生存期,最佳治疗方案仍有待明确。生存与无进展生存之间的显著差异表明挽救治疗(该队列患者通常采用联合化疗)是有益的。然而,由于复发时接受任何一种治疗方案治疗的患者数量较少,无法对生存获益进行更正式的分析。