Freeman C R, Perilongo G
Departments of Oncology and Pediatrics, McGill University, 1650 Cedar Avenue, Room D5-400, Montreal, Quebec, Canada, H3G 1A4.
Childs Nerv Syst. 1999 Oct;15(10):545-53. doi: 10.1007/s003810050542.
Approximately 80% of tumors arising in the brain stem are diffuse intrinsic lesions. Patients typically present with a short duration of symptoms and signs with significant neurological impairment. Imaging findings are typical, and biopsy is not usually necessary to make a diagnosis. Standard treatment consists of radiotherapy alone. Although the majority of patients will show a significant improvement in neurological status following such treatment, the prognosis is very poor. The median time to disease progression is of the order of 5-6 months, the median survival time less than 1 year, and survival at 2 years and beyond, less than 10%. Over the last 10-15 years, numerous studies have been undertaken in an attempt to develop more effective treatment for children with diffuse intrinsic brain stem tumors. Using a hyperfractionated (twice-daily) schedule, doses of radiotherapy as high as 78 Gy have been given without success. Other approaches use chemotherapy combined with radiotherapy in a variety of different ways, including up-front, concurrent, and adjuvant chemotherapy, and high-dose chemotherapy with rescue. The results of these studies using chemotherapy, as well as other approaches using other systemic agents, are reviewed in detail.
脑干中出现的肿瘤约80%为弥漫性固有病变。患者通常症状和体征持续时间短，伴有严重神经功能损害。影像学表现典型，通常无需活检即可做出诊断。标准治疗仅包括放疗。尽管大多数患者经此类治疗后神经状态会有显著改善，但预后很差。疾病进展的中位时间约为5 - 6个月，中位生存时间不到1年，2年及以后的生存率不到10%。在过去10 - 15年里，人们进行了大量研究，试图为弥漫性固有脑干肿瘤患儿开发更有效的治疗方法。采用超分割（每日两次）方案，给予高达78 Gy的放疗剂量但未成功。其他方法以各种不同方式将化疗与放疗联合使用，包括 upfront、同步和辅助化疗，以及大剂量化疗加挽救治疗。本文详细综述了这些使用化疗的研究结果，以及使用其他全身药物的其他方法的结果。
