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避免辐射对婴儿髓母细胞瘤患者生存和神经认知结果的影响。

Impact of radiation avoidance on survival and neurocognitive outcome in infant medulloblastoma.

机构信息

Department of Pediatric Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, Calgary, AB.

出版信息

Curr Oncol. 2009 Dec;16(6):21-8. doi: 10.3747/co.v16i6.435.

Abstract

PURPOSE

Concerns about radiotherapy-related neurocognitive sequelae in young children have led to deferral or avoidance of radiation in contemporary treatment for this fragile group of patients. We compared survival and neurocognitive outcome in two groups of infants with medulloblastoma who received adjuvant conventional craniospinal irradiation (CSI) or reduced or no radiotherapy during an era of change in the philosophy of infant medulloblastoma treatment.

PATIENTS AND METHODS

From 1985 to 2007, 29 patients 3 years of age or younger were diagnosed and treated with curative intent in our institution. Children treated before 1994 received adjuvant radiation with chemotherapy; subsequently, radiation was prescribed essentially for disease progression or relapse.

RESULTS

Median age at diagnosis was 24 months (range: 1-36 months); 15 patients (52%) presented with metastatic disease at diagnosis. As part of initial treatment, 8 children received adjuvant radiotherapy with chemotherapy, and 21 children received postoperative chemotherapy only. Five children treated with chemotherapy alone are in prolonged remission. The 5-year event-free and overall survivals were 35.9% +/- 9.8% and 50.2% +/- 9.6% respectively. Extent of resection, metastatic status, and desmoplastic histology were not found to be significant prognostic factors. On serial neurocognitive evaluations, patients treated with chemotherapy with or without reduced radiotherapy demonstrated improvement of intellectual function over time. Patients treated with conventional csi exhibited significantly lower intelligence quotient scores and academic performance, with the exception of receptive vocabulary.

CONCLUSIONS

Avoidance of conventional CSI in treatment of very young children with medulloblastoma appears to be associated with a preserved neurocognitive profile. Neurocognitive evaluation should be integrated into the primary objectives of future infant protocols.

摘要

目的

由于担心放疗相关的神经认知后遗症,目前在治疗这组脆弱的患儿时,对于接受传统全脑脊髓照射(CSI)的患儿,会推迟或避免放疗。我们比较了在小儿髓母细胞瘤治疗理念转变的时代,两组接受辅助常规 CSI 或减少或不接受放疗的婴儿患者的生存和神经认知结局。

患者和方法

1985 年至 2007 年,我们机构共诊断和治疗了 29 例年龄在 3 岁或以下的患儿,他们的疾病均为治愈性的。1994 年前接受治疗的患儿接受辅助放化疗;随后,放疗主要用于疾病进展或复发。

结果

中位诊断年龄为 24 个月(范围:1-36 个月);15 例(52%)患儿初诊时即存在转移。作为初始治疗的一部分,8 例患儿接受辅助放化疗,21 例患儿仅接受术后化疗。5 例单纯化疗的患儿处于长期缓解中。5 年无事件生存和总生存分别为 35.9%±9.8%和 50.2%±9.6%。肿瘤切除范围、转移状态和促纤维组织增生性组织学均不是显著的预后因素。在连续的神经认知评估中,接受化疗加或不加减少放疗的患儿的智力功能随时间推移而提高。接受常规 CSI 治疗的患儿的智商评分和学业成绩明显较低,除了接受能力词汇测试外。

结论

在治疗非常年幼的髓母细胞瘤患儿时,避免常规 CSI 似乎与保持神经认知特征有关。神经认知评估应纳入未来婴儿方案的主要目标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eda3/2794676/2b7892b15914/co16-6-414f1.jpg

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