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儿童髓母细胞瘤。生存与治疗。

Medulloblastoma in children. Survival and treatment.

作者信息

Mealey J, Hall P V

出版信息

J Neurosurg. 1977 Jan;46(1):56-64. doi: 10.3171/jns.1977.46.1.0056.

Abstract

The authors review treatment and results in 45 cases of medulloblastoma arising in childhood. The surgical mortality rate observed was 11%. Of those completing postoperative cerebrospinal irradiation at this institution, 53% have survived for 3 years, 41% for 5 years, and 22% for 10 years. The extent of surgical resection of the cerebellar tumor had no significant bearing on the prognosis. Those cases remaining free of recurrent disease had received significantly higher doses of postoperative irradiation, approaching 5000 rads to the whole brain or posterior fossa and 4000 rads to the spinal axis. Repeat irradiation and chemotherapy (vincristine, the nitrosoureas, and methotrexate) provided good palliation in most cases and significantly extended the survival time. However, 28 of 29 patients who developed locally recurrent or metastatic disease have died. Vincristine was considered the chemotherapeutic drug of choice and in 14 cases its use was associated with remissions lasting 2 to 18 months. The combination of chemotherapy and repeat irradiation was followed by remissions of longer duration compared to retreatment by irradiation alone when the disease recurred within 2 years. The inherent value of ventricular shunting procedures and steroid therapy for recurrent intracranial disease could not be ascertained. The findings in this study suggest that the primary treatment of medulloblastoma should be extended to include chemotherapy and optimum radiation therapy, since once recurrent disease develops retreatment is essentially palliative and a fatal outcome is virtuallly certain.

摘要

作者回顾了45例儿童髓母细胞瘤的治疗情况及结果。观察到的手术死亡率为11%。在本机构完成术后脑脊髓放疗的患者中,53%存活了3年,41%存活了5年,22%存活了10年。小脑肿瘤的手术切除范围对预后无显著影响。那些无疾病复发的病例接受的术后放疗剂量显著更高,全脑或后颅窝接近5000拉德,脊髓轴接近4000拉德。重复放疗和化疗(长春新碱、亚硝基脲类和甲氨蝶呤)在大多数情况下提供了良好的姑息治疗,并显著延长了生存时间。然而,29例发生局部复发或转移疾病的患者中有28例死亡。长春新碱被认为是首选化疗药物,在14例病例中,其使用与持续2至18个月的缓解相关。当疾病在2年内复发时,与单纯放疗再治疗相比,化疗和重复放疗相结合后缓解持续时间更长。对于复发性颅内疾病,脑室分流手术和类固醇治疗的内在价值无法确定。本研究结果表明,髓母细胞瘤的主要治疗应扩展至包括化疗和最佳放疗,因为一旦发生复发疾病,再治疗基本上是姑息性的,且几乎肯定会导致致命结果。

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