Ribi K, Relly C, Landolt M A, Alber F D, Boltshauser E, Grotzer M A
Division of Oncology, University Children's Hospital of Zurich, Zurich, Switzerland.
Neuropediatrics. 2005 Dec;36(6):357-65. doi: 10.1055/s-2005-872880.
To study the outcomes in long-term survivors of paediatric medulloblastoma (MB), we followed 51 consecutive children who were treated between 1980 and 2000 in a single institution. In 18 of 26 survivors (mean follow-up time 12.2 years), tumour control, neurological, endocrine, and neurocognitive complications and their impact on behavioural and psychological adjustment, and health-related quality of life (QoL) were comprehensively assessed using qualitative and quantitative measures. Endocrine deficits occurred in 61 %, neurological complications in 72 %, and significant school problems in 72 %. All patients had significant deficits in neurocognitive functioning: attention and processing speed was impaired in 79 %, learning and memory in 88 %, language in 56 %, visual perception in 50 %, and executive functions in 64 %. In comparison with healthy controls, social functioning was rated by the patients as the QoL dimension most affected. Parents' ratings were considerably lower than those of the patients. No MB survivor > 18 years of age (n = 12) had a boy- or girlfriend. Because of their treatment, including craniospinal radiotherapy, MB long-time survivors are not only at great risk for neurological, endocrine, and neurocognitive complications, but also of social isolation thereby decreasing self-rated QoL substantially.
为研究小儿髓母细胞瘤（MB）长期存活者的预后情况，我们对1980年至2000年间在单一机构接受治疗的51名连续儿童进行了随访。在26名存活者中的18名（平均随访时间12.2年）中，使用定性和定量方法对肿瘤控制、神经、内分泌和神经认知并发症及其对行为和心理调适的影响以及健康相关生活质量（QoL）进行了全面评估。内分泌缺陷发生率为61%，神经并发症发生率为72%，严重学习问题发生率为72%。所有患者在神经认知功能方面均有显著缺陷：79%的患者注意力和处理速度受损，88%的患者学习和记忆受损，56%的患者语言能力受损，50%的患者视觉感知受损，64%的患者执行功能受损。与健康对照组相比，患者将社会功能评为受QoL影响最大的维度。父母的评分明显低于患者。没有一名年龄大于18岁的MB存活者（n = 12）有男女朋友。由于包括颅脊髓放疗在内的治疗，MB长期存活者不仅面临神经、内分泌和神经认知并发症的高风险，还面临社会孤立，从而显著降低了自我评定的QoL。
