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[神经母细胞瘤:生物标志物、手术及临床病程]

[Neuroblastoma: biological markers, surgery, and clinical course].

作者信息

Martínez Ibáñez V, Abad P, Gallego S, Sánchez de Toledo J, Marqués A, Boix Ochoa J

机构信息

Unidad de Oncología, Dpto. Cirugía Pediátrica, Hospital Materno-Infantil Vall d'Hebron, Po Vall d'Hebron, 119-129, 08035 Barcelona.

出版信息

Cir Pediatr. 2000 Apr;13(2):47-53.

Abstract

INTRODUCTION

The treatment of neuroblastoma is basically chemotherapy, and surgery, in spite of advances, this kind of tumor is nowadays a surgical challenge.

PURPOSE

The aim of this study was to evaluate the impact of our therapy in this kind of pediatric tumors.

MATERIAL AND METHODS

32 consecutive patients with abdominal neuroblastoma, aged between 1 month and 10 years old, median age 3 years old, observed from 1993 through 1997 have been studied. Several parameters: age, ferritin, deletion of the chromosome 1p36, chromosomic ploidy, LDH, N-myc gene amplification and enolase neuron specific were studied and were related with the histology by Joshi and the International Neuroblastoma Staging System (INSS) in order to know the prognosis. All the patients were treated by means of chemotherapy and surgery, and some cases with radiotherapy and bone marrow transplantation.

RESULTS

Two patients presented the tumor in stage I (INSS) and three in stage II. All the patients had a total resection and they live free of disease. In the state III, two patients did not maintain the follow-up; five live with disease (two with QT without surgery yet, two local recurrences, and one metastasis), and four live free of disease. In the stage IV: five died, two live with disease (1 local recurrence and one metastasis), five live free of disease, and one did not maintain the follow-up. In the stage IV-S, the three patients live free of disease. The method of Kaplan-Meier at 5 years shows a mean of 49 months and a median of 60 months.

CONCLUSIONS

  1. Of all the parameters studied, we consider the ones with biggest prognostic efficacy are, the age, the stage INSS, the histology Shimada and the N-myc amplification. 2. The total resection of the tumor keeps being essential for a more favourable prognostic.
摘要

引言

神经母细胞瘤的治疗基本为化疗,尽管外科手术有了进展,但这类肿瘤如今仍是外科手术的一大挑战。

目的

本研究旨在评估我们的治疗方法对这类儿科肿瘤的影响。

材料与方法

对1993年至1997年间观察的32例连续腹部神经母细胞瘤患者进行了研究,年龄在1个月至10岁之间,中位年龄3岁。研究了几个参数:年龄、铁蛋白、1p36染色体缺失、染色体倍性、乳酸脱氢酶、N - myc基因扩增和神经元特异性烯醇化酶,并通过乔希组织学分类和国际神经母细胞瘤分期系统(INSS)将其与组织学相关联,以了解预后情况。所有患者均接受化疗和手术治疗,部分病例还接受了放疗和骨髓移植。

结果

两名患者的肿瘤处于I期(INSS),三名处于II期。所有患者均进行了肿瘤全切,且无病生存。在III期,两名患者未坚持随访;五名患者带瘤生存(两名QT患者尚未手术,两名局部复发,一名转移),四名患者无病生存。在IV期:五名患者死亡,两名患者带瘤生存(1例局部复发和1例转移),五名患者无病生存,一名患者未坚持随访。在IV - S期,三名患者无病生存。采用Kaplan - Meier法计算5年生存率,平均为49个月,中位数为60个月。

结论

  1. 在所有研究的参数中,我们认为预后效果最大的参数是年龄、INSS分期、岛田组织学分类和N - myc扩增。2. 肿瘤全切对于更有利的预后仍然至关重要。

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