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神经母细胞瘤:英国一个区域中心 20 年的经验。

Neuroblastoma: a 20-year experience in a UK regional centre.

机构信息

Academic Paediatric Surgery, Division of Child Health, University of Liverpool, Liverpool, UK.

出版信息

Pediatr Blood Cancer. 2011 Dec 15;57(7):1254-60. doi: 10.1002/pbc.23149. Epub 2011 Apr 26.

DOI:10.1002/pbc.23149
PMID:21523901
Abstract

PURPOSE

The role of surgery in the management of neuroblastoma yields conflicting reports. We report a 20-year experience from a UK centre in the context of evolving cancer therapies for neuroblastoma.

METHODS

Hospital records of 91 neuroblastoma patients from 1985 to 2005 were studied. Patient demographics, data from operating notes and tumour biology (MYCN status) where available were analysed.

RESULTS

Surgery consisted of primary resection or delayed operation following tumour biopsy/chemotherapy. Overall survival was 100% for stage 1(n = 3), 90% for stage 2 (n = 10), 46% for stage 3 (n = 13), 13% for stage 4 (n = 55) and 56% for stage 4S disease (n = 9). During the eras 1985-1994 versus 1995-2005, survival for stage 3 lesions was 25% and 80% (P = 0.04) with marginal increase in survival observed in stage 4 disease (12% vs. 22%, P = 0.083). Delayed tumour resection was not performed in 20 (36%) stage 4 patients due to progressive disease. Complete tumour resection was achieved in 62% of stage 3-4 patients during 1995-2005 compared to 38% in 1985-1994. The extent of surgical resection (complete vs. partial) showed no significant differences in overall survival or relapse rates. Postoperative morbidity occurred in 15.7% of cases emphasising technical challenges in resection of neuroblastoma. No child with MYCN amplification survived versus 59% survival in non-amplified cases (P = 0.012).

CONCLUSIONS

While complete tumour resection may be desirable in advanced neuroblastoma (stage 3-4) these findings suggest that the radicality of operation is not significantly associated with better overall survival/relapse. Improving outcomes in the 1995-2005 era for patients with stage 3-4 tumours complements the introduction of new high dose-intensive chemotherapy regimens and other adjuvant therapies for this enigmatic disease.

摘要

目的

手术在神经母细胞瘤治疗中的作用存在相互矛盾的报告。我们报告了英国一家中心在神经母细胞瘤癌症治疗不断发展的背景下,20 年来的经验。

方法

对 1985 年至 2005 年 91 例神经母细胞瘤患者的医院记录进行了研究。分析了患者的人口统计学资料、手术记录中的数据以及肿瘤生物学(MYCN 状态)。

结果

手术包括原发肿瘤切除或肿瘤活检/化疗后的延迟手术。1 期(n = 3)患者的总生存率为 100%,2 期(n = 10)为 90%,3 期(n = 13)为 46%,4 期(n = 55)为 13%,4S 期(n = 9)为 56%。在 1985-1994 年与 1995-2005 年期间,3 期病变的生存率为 25%和 80%(P = 0.04),4 期疾病的生存率略有增加(12%和 22%,P = 0.083)。由于疾病进展,20 例(36%)4 期患者未行延迟肿瘤切除术。1995-2005 年期间,62%的 3-4 期患者实现了肿瘤完全切除,而 1985-1994 年期间为 38%。手术切除的范围(完全切除与部分切除)在总生存率或复发率方面无显著差异。术后并发症发生率为 15.7%,强调了神经母细胞瘤切除的技术挑战。扩增的 MYCN 患儿无一例存活,而非扩增患儿的存活率为 59%(P = 0.012)。

结论

尽管在晚期神经母细胞瘤(3-4 期)中完全肿瘤切除可能是理想的,但这些发现表明手术的彻底性与总体生存/复发无显著相关性。在 1995-2005 年期间,对 3-4 期肿瘤患者采用新的大剂量强化化疗方案和其他辅助治疗,使患者的预后得到改善,这与这种神秘疾病的引入相吻合。

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