肾母细胞瘤及其他恶性肾肿瘤的治疗结果：来自智利国家儿童癌症项目（PINDA）的报告

Results of Therapy for Wilms Tumor and Other Malignant Kidney Tumors: A Report From the Chilean Pediatric National Cancer Program (PINDA).

作者信息

Joannon Pilar, Becker Ana, Kabalan Paola, Concha Emma, Beresi Victoria, Salgado Carmen, Martínez Pilar, Olate Paola, Arriagada Mónica, Espinoza Felipe, Varas Mónica, Benavides Pablo, Valero Miguel, Reyes Mauricio

机构信息

*Department of Hematology-Oncology, Hospital Roberto del Río †Department of Hematology-Oncology, Hospital Sótero del Río ‡Faculty of Medicine, University of Chile §Department of Hematology-Oncology, Hospital Luis Calvo Mackenna ∥Department of Hematology-Oncology, Hospital Exequiel González Cortés, Santiago ¶Department of Hematology-Oncology, Hospital Regional de Valdivia, Valdivia #Department of Hematology-Oncology, Hospital Grant Benavente, Concepción **Department of Hematology-Oncology, Hospital Regional de Temuco, Temuco ††Department of Hematology-Oncology, Hospital San Borja Arriarán, Santiago ‡‡Department of Hematology-Oncology, Hospital San Juan de Dios, Santiago §§Department of Hematology-Oncology, Hospital Gustavo Fricke, Viña del Mar ∥∥Department of Hematology-Oncology, Hospital Van Buren, Valparaíso ¶¶Department of Radiation Oncology, Instituto Nacional del Cáncer, Santiago, Chile.

出版信息

J Pediatr Hematol Oncol. 2016 Jul;38(5):372-7. doi: 10.1097/MPH.0000000000000576.

DOI:10.1097/MPH.0000000000000576

PMID:27164527

Abstract

OBJECTIVE

The aim of this study was to analyze the survival of children with Wilms tumor and other malignant renal tumors treated with the TWPINDA-99 protocol.

MATERIALS AND METHODS

Between January 1999 and December 2013, 226 patients were registered on this trial, based on National Wilms Tumor Study-5. Patient characteristics and survival were evaluated.

RESULTS

Two hundred seven patients were diagnosed with Wilms tumor, which represented 91.6% of renal tumors. The male to female ratio was 0.7:1. The median age at diagnosis was 3.3 years. Stage III was the most frequent (39.2%). Metastatic disease was present in 16.7% of the cases. Synchronous bilateral disease was observed in 9.3% of the cases. Favorable histology was diagnosed in 93.6% and anaplastic histology in 6.4% of the patients. Median follow-up was 7.5 years. Ten-year event-free survival and overall survival (OS) for assessable patients with Wilms tumor (n=192) were 82.0% and 89.9%, respectively. OS for patients with stage I was 100% (n=36), stage II: 97.1% (n=35), stage III: 88.6% (n=71), stage IV: 77.9% (n=32), and stage V: 80.8% (n=18). OS for favorable histology (n=180) and anaplastic histology tumors (n=12) were 91.0% and 72.9%, respectively. Other malignant renal tumors had a poorer survival.

CONCLUSION

Prognosis for patients with Wilms tumor treated on TWPINDA-99 seems to be better than previous national trials and is similar to developed countries.

摘要

目的

本研究旨在分析采用TWPINDA - 99方案治疗的肾母细胞瘤及其他恶性肾肿瘤患儿的生存率。

材料与方法

1999年1月至2013年12月期间，基于国家肾母细胞瘤研究 - 5，226例患者登记参与了本试验。对患者特征及生存率进行了评估。

结果

207例患者被诊断为肾母细胞瘤，占肾肿瘤的91.6%。男女比例为0.7:1。诊断时的中位年龄为3.3岁。III期最为常见（39.2%）。16.7%的病例存在转移性疾病。9.3%的病例观察到同步双侧疾病。93.6%的患者诊断为预后良好的组织学类型，6.4%为间变组织学类型。中位随访时间为7.5年。可评估的肾母细胞瘤患者（n = 192）的10年无事件生存率和总生存率（OS）分别为82.0%和89.9%。I期患者的OS为100%（n = 36），II期：97.1%（n = 35），III期：88.6%（n = 71），IV期：77.9%（n = 32），V期：80.8%（n = 18）。预后良好组织学类型（n = 180）和间变组织学类型肿瘤（n = 12）的OS分别为91.0%和72.9%。其他恶性肾肿瘤的生存率较差。