van den Heuvel-Eibrink Marry M, Grundy Paul, Graf Norbert, Pritchard-Jones Kathy, Bergeron Christophe, Patte Catherine, van Tinteren Harm, Rey Annie, Langford Carolyn, Anderson Jim R, de Kraker Jan
Department of Pediatric Oncology/Hematology; Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Pediatr Blood Cancer. 2008 Jun;50(6):1130-4. doi: 10.1002/pbc.21389.
To review the clinical characteristics and survival of infants diagnosed with a primary renal tumor in the first 7 months of life.
A retrospective data review of patients registered in five large international protocols (SFOP/GPOH/SIOP9/93-01, UKW3 and NWTSG 4 and 5) spanning 1985-2002.
750 (7.2%) of 10,430 registered patients were diagnosed with a renal tumor before age 213 days. Tumor types were Wilms tumor (WT) 58%; congenital mesoblastic nephroma (CMN) 18%; malignant rhabdoid tumor (MRTK) 8%; clear cell sarcoma (CCSK) 2%; non-Wilms tumor (unspecified) 6%; histology unknown, 9%. CMN predominated among tumors diagnosed in the first month of life (54%) but its relative contribution diminished to <10% of all cases diagnosed after the age of 3 months (P < 0.001). Among 639 cases with specified histology and stage, 9/11 stage IV tumors were MRTK, 37/39 bilateral tumors were WT. In 626 children where surgical approach was specified, 522 had immediate nephrectomy. For all cases, 5 years event-free survival (EFS) was 80% and overall survival (OS) 86%. Five years EFS and OS respectively by tumor type were WT (86%, 93%), CMN (94%, 96%), CCSK (49%, 51%), MRTK (16%, 16%).
Renal tumors diagnosed in the first 7 months of life generally have an excellent prognosis though histology is an important prognostic factor. In the first 2 months of life the prevalence of CMN is high. The relative occurrence of WT increases rapidly with age thereafter. Bilateral tumors are usually WT. Tumors with metastases at diagnosis are usually MRTK.
回顾出生后7个月内诊断为原发性肾肿瘤的婴儿的临床特征和生存情况。
对1985年至2002年期间登记在五个大型国际方案（SFOP/GPOH/SIOP9/93 - 01、UKW3以及NWTSG 4和5）中的患者进行回顾性数据审查。
10430名登记患者中有750名（7.2%）在213天龄之前被诊断为肾肿瘤。肿瘤类型为肾母细胞瘤（WT）58%；先天性中胚层肾瘤（CMN）18%；恶性横纹肌样瘤（MRTK）8%；透明细胞肉瘤（CCSK）2%；非肾母细胞瘤（未明确）6%；组织学未知，9%。CMN在出生后第一个月诊断出的肿瘤中占主导（54%），但在3个月龄后诊断出的所有病例中其相对比例降至<10%（P < 0.001）。在639例明确组织学和分期的病例中，11例IV期肿瘤中有9例为MRTK，39例双侧肿瘤中有37例为WT。在626名明确手术方式的儿童中，522例行即刻肾切除术。所有病例的5年无事件生存率（EFS）为80%，总生存率（OS）为86%。按肿瘤类型划分的5年EFS和OS分别为：WT（86%，93%）、CMN（94%，96%）、CCSK（49%，51%）、MRTK（16%，16%）。
出生后7个月内诊断出的肾肿瘤总体预后良好，尽管组织学是一个重要的预后因素。出生后头2个月内CMN的患病率较高。此后WT的相对发生率随年龄迅速增加。双侧肿瘤通常为WT。诊断时伴有转移的肿瘤通常为MRTK。
