I. Klinik für Kinder und Jugendliche, Klinikum Augsburg.
Dtsch Arztebl Int. 2011 Jun;108(22):390-7. doi: 10.3238/arztebl.2011.0390. Epub 2011 Jun 3.
Multimodal treatment approaches for children with tumors of the central nervous system (CNS) have markedly contributed to improved survival. Before 1970, the survival rate among children with medulloblastoma, the most common malignant CNS tumor in children, was about 20%. At present, in contrast, cure can be achieved in more than 75% of children with a favorable constellation of risk factors. In this review article for clinicians, we give an overview of the current understanding of the pathology, presenting manifestations, early diagnosis, and treatment of CNS tumors in children and adolescents.
We report the research findings of the German Treatment Network "HIT" and selectively review the pertinent literature.
Treatment-optimizing clinical trials have improved survival from all types of CNS tumors in children and adolescents. Biological features of the tumors now serve as the basis for improved stratification for multimodal, risk-adapted treatment. Targeted biological strategies are being developed. Difficulties remain, however, in the care of infants with CNS tumors and in the treatment of metastatic disease, tumors of certain histological types, and tumors in certain anatomical sites. Many of the affected children suffer from late effects of their disease and its treatment that can irreversibly impair their development.
Children with a suspected or confirmed diagnosis of brain tumor should be referred early to a center with the relevant experience. Standardized diagnostic and therapeutic methods have markedly improved the chance of cure. Current research on molecular signaling pathways seems likely to lead to the development of new treatments, particularly for tumors currently associated with lower rates of survival. The long-term side effects of treatment must be systematically monitored so that they can be avoided in future, and so that appropriate support measures can be provided to the affected children.
针对中枢神经系统（CNS）肿瘤患儿的多模式治疗方法显著提高了生存率。在 1970 年以前，儿童中最常见的恶性 CNS 肿瘤髓母细胞瘤患儿的存活率约为 20%。相比之下，目前，在具有良好风险因素组合的患儿中，超过 75%的患儿可以治愈。在这篇面向临床医生的综述文章中，我们概述了目前对儿童和青少年 CNS 肿瘤的病理学、临床表现、早期诊断和治疗的理解。
我们报告了德国治疗网络“HIT”的研究结果，并选择性地回顾了相关文献。
优化治疗的临床试验改善了所有类型 CNS 肿瘤患儿的生存率。肿瘤的生物学特征现在是为多模式、风险适应治疗进行更好分层的基础。正在开发靶向生物学策略。然而，在 CNS 肿瘤患儿的护理以及转移性疾病、特定组织学类型肿瘤和特定解剖部位肿瘤的治疗方面仍然存在困难。许多受影响的儿童患有疾病及其治疗的晚期影响，这些影响可能会不可逆地损害他们的发育。
疑似或确诊脑肿瘤的患儿应尽早转至具有相关经验的中心。标准化的诊断和治疗方法显著提高了治愈的机会。目前对分子信号通路的研究似乎有可能开发出新的治疗方法，特别是对于目前生存率较低的肿瘤。必须系统地监测治疗的长期副作用，以便在未来避免这些副作用，并为受影响的儿童提供适当的支持措施。
