Park T S, Hoffman H J, Hendrick E B, Humphreys R P, Becker L E
J Neurosurg. 1983 Apr;58(4):543-52. doi: 10.3171/jns.1983.58.4.0543.
The authors review the cases of 144 children with medulloblastoma treated between 1950 and 1980. Duration of time between onset of symptoms and initial treatment was less than 1 1/2 months in 51% of cases, and less than 3 months in 76%. The tumor was located in the cerebellar vermis in 93% of patients. Brainstem infiltration was noted in 32%. Classical medulloblastomas comprised 82% of the total number reviewed, and desmoplastic medulloblastomas 15%. The majority of desmoplastic medulloblastomas were found in the midline of the cerebellum and in patients under 10 years of age. The prognosis for patients with desmoplastic medulloblastomas was worse than that for children with classical medulloblastomas. Spontaneous hemorrhage associated with primary or recurrent medulloblastoma occurred in 5.6% of the patients. Supratentorial metastases were found in 14.6% of cases, spinal cord metastases in 12.5%, and systemic metastases in 9%. The overall 5-year survival rate was 47%, and the 10-year survival rate 42%. Extent of surgical excision proved to be a statistically significant prognostic factor. Two patients developed recurrence after the "period of risk" as defined by Collins' rule. Delayed complications of radiotherapy were found to be substantial. Intelligence quotient (IQ) testing on 16 survivors revealed verbal IQ, performance IQ, and full-scale IQ to be within the normal range in 11, seven, and nine cases, respectively. Two were retarded on all scores.
作者回顾了1950年至1980年间接受治疗的144例髓母细胞瘤患儿的病例。51%的病例症状出现至初始治疗的时间间隔小于1.5个月，76%的病例小于3个月。93%的患者肿瘤位于小脑蚓部。32%的病例发现脑干浸润。经典型髓母细胞瘤占所回顾病例总数的82%，促结缔组织增生型髓母细胞瘤占15%。大多数促结缔组织增生型髓母细胞瘤位于小脑中线，且见于10岁以下患者。促结缔组织增生型髓母细胞瘤患者的预后比经典型髓母细胞瘤患儿更差。5.6%的患者发生与原发性或复发性髓母细胞瘤相关的自发性出血。14.6%的病例发现幕上转移，12.5%的病例发现脊髓转移，9%的病例发现全身转移。总体5年生存率为47%，10年生存率为42%。手术切除范围被证明是一个具有统计学意义的预后因素。两名患者在柯林斯法则定义的“危险期”后出现复发。放疗的延迟并发症很严重。对16名幸存者进行的智商（IQ）测试显示，言语智商、操作智商和总智商分别在11例、7例和9例中处于正常范围。两名患者各项得分均偏低。
