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神经母细胞瘤:新加坡国立大学保健系统的经验(1987-2008 年)。

Neuroblastoma: experience from National University Health System, Singapore (1987-2008).

机构信息

Royal College of Surgeons in Ireland, Dublin, Ireland..

出版信息

Singapore Med J. 2012 Jan;53(1):19-25.

PMID:22252178
Abstract

INTRODUCTION

Neuroblastoma is the most common extracranial solid tumour in childhood. We report our experience at National University Health System (NUHS), Singapore.

METHODS

We performed a retrospective chart review of 43 patients diagnosed with neuroblastoma, who were seen and treated at the Department of Paediatrics, NUHS from November 1987 to November 2008.

RESULTS

The median age of the patients at diagnosis was 1.9 (range 0.1-20.2) years. The majority (70.1%) of primary tumours were of abdominal and/or adrenal origin. According to the International Neuroblastoma Staging System, six (14.0%) patients were in stages 1 and 2, 11 (25.6%) in stage 3, 19 (44.2%) in stage 4, and seven (16.2%) in stage 4s. Therapy for all patients included surgery and/or chemotherapy and/or radiation therapy. Patients with stage 4 disease also underwent autologous stem cell transplant. The median follow-up for the cohort was 2.5 (range 0.4-21.0) years. At the time of analysis, 29 (67.4%) patients were alive. The two- and five-year overall survival for the cohort was 65.0% (95% confidence interval [CI] 51.0%-80.0%) and 62.0% (95% CI 45.0%-79.0%), respectively. The five-year overall survival rates according to risk status were 100.0% for low-risk, 75.0% for intermediate risk and 28.2% for high-risk neuroblastoma.

CONCLUSION

The prognosis for those with advanced stage neuroblastoma remains poor. A collaborative effort, with an emphasis on research in detecting biologic characteristics of aggressive disease and tailoring therapy, needs to be strengthened in order to further our understanding of this disease.

摘要

简介

神经母细胞瘤是儿童期最常见的颅外实体瘤。我们报告了在新加坡国立大学健康系统(NUHS)的经验。

方法

我们对 1987 年 11 月至 2008 年 11 月在 NUHS 儿科就诊和治疗的 43 名神经母细胞瘤患者进行了回顾性图表审查。

结果

患者诊断时的中位年龄为 1.9 岁(范围为 0.1-20.2 岁)。大多数(70.1%)原发性肿瘤位于腹部和/或肾上腺。根据国际神经母细胞瘤分期系统,6 例(14.0%)患者处于 1 期和 2 期,11 例(25.6%)处于 3 期,19 例(44.2%)处于 4 期,7 例(16.2%)处于 4s 期。所有患者的治疗均包括手术和/或化疗和/或放射治疗。患有 4 期疾病的患者还接受了自体干细胞移植。该队列的中位随访时间为 2.5 年(范围为 0.4-21.0 年)。在分析时,29 例(67.4%)患者存活。该队列的两年和五年总生存率分别为 65.0%(95%置信区间[CI]为 51.0%-80.0%)和 62.0%(95%CI为 45.0%-79.0%)。根据风险状况,低危组的五年总生存率为 100.0%,中危组为 75.0%,高危组为 28.2%。

结论

晚期神经母细胞瘤患者的预后仍然较差。需要加强协作努力,重点研究检测侵袭性疾病的生物学特征并制定治疗方案,以进一步了解这种疾病。

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