Brandes A A, Palmisano V, Monfardini S
Divisione di Oncologia Medica, Azienda Ospedaliera, Padova, Italy.
Cancer Treat Rev. 1999 Feb;25(1):3-12. doi: 10.1053/ctrv.1998.0096.
Long-term survival is possible in adults with medulloblastoma with rates of approximately 50-60% at 5 years, and 40-50% at 10 years. As the literature data are based on retrospective studies, treatments are neither randomized nor uniform, however, some treatment cornerstones have been identified. The first step is surgery, which should be as radical as possible; adjuvant radiotherapy must be 55 Gy on the posterior fossa, and 36 Gy on the remaining cranial-spinal axis; adjuvant chemotherapy may be useful in patients at high risk of recurrence provided it is administered before radiotherapy in moderate-high dosages and includes cisplatin, etoposide and cyclophosphamide. This chemotherapy program should not overly delay the start of radiotherapy, be recycled as soon as blood count permits and not exceed two or three cycles. Adjuvant chemotherapy after radiotherapy, even if indicated in cases with persistent tumour, may have an adverse effect due to the poor marrow reserves of these patients. At recurrence the prospects of cure are very poor due to the deficient hematopoietic reserve, but in very young patients high dose chemotherapy with marrow rescue might be usefully employed.
成神经管细胞瘤患者有可能实现长期生存，5年生存率约为50%-60%，10年生存率约为40%-50%。由于文献数据基于回顾性研究，治疗既未随机化也不统一，不过，已确定了一些治疗基石。第一步是手术，应尽可能彻底；辅助放疗后颅窝剂量必须为55 Gy，其余颅脊髓轴剂量为36 Gy；对于复发高危患者，辅助化疗可能有用，前提是在放疗前以中高剂量给药，且包括顺铂、依托泊苷和环磷酰胺。该化疗方案不应过度延迟放疗开始时间，一旦血细胞计数允许应尽快重复，且不超过两三个周期。放疗后辅助化疗，即使在肿瘤持续存在的情况下有指征，也可能因这些患者骨髓储备差而产生不良影响。复发时，由于造血储备不足，治愈前景非常渺茫，但在非常年轻的患者中，高剂量化疗联合骨髓挽救可能会有用。
