EPIDEMIOLOGIC AND CLINICAL CORRELATIONS WITH GENETIC-CHARACTERISTICS OF WILMS-TUMOR - RESULTS OF THE BRAZILIAN-WILMS-TUMOR-STUDY-GROUP

被引:8
作者
FRANCO, EL
DECAMARGO, B
SABA, L
MARQUES, LA
机构
[1] AC CAMARGO HOSP,DEPT PATHOL,SAO PAULO,BRAZIL
[2] MCGILL UNIV,DEPT EPIDEMIOL & BIOSTAT,MONTREAL H3A 2T5,QUEBEC,CANADA
[3] AC CAMARGO HOSP,DEPT PEDIAT ONCOL,SAO PAULO,BRAZIL
[4] LUDWIG INST CANC RES,SAO PAULO,BRAZIL
关键词
RENAL BLASTEMA NEPHROBLASTOMATOSIS; CONGENITAL-ANOMALIES; HUMAN CARCINOGENESIS; FREQUENT PRECURSOR; RACIAL VARIATION; CANCER; MUTATION; MODEL; CHROMOSOME-11; EMBRYOLOGY;
D O I
10.1002/ijc.2910480502
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
The epidemiological and clinical features of Wilms' tumor (WT) were analyzed in 176 patients admitted to the Brazilian WT Study Group. The occurrence of congenital anomalies (9.1%) and the M:F ratio (0.83) were comparable to those observed by the US National WT Study and the International Society of Pediatric Oncology trials. Bilateral cases were younger on average than unilateral cases (37.2 vs. 45.0 months). Ethnic group and gender were also associated with age, with non-white children being generally older (46.1 months) than whites (39.5 months). and boys being younger than girls (37.0 vs. 46.1 months). However, the most important factor in association with age at diagnosis was disease stage. No early disease patients were diagnosed after 8 years of age (mean: 37.5 months), whereas 10% of those with advanced disease were diagnosed between the ages of 8 and 10 years (mean: 56.3 months). There were no clear distinctions in age distributions on the basis of the presence of tumor multicentricity, intra- or perilobar nephroblastomatosis, and of a combination of putative genetic determinants. The mean age at diagnosis for cases with congenital defects was higher than that for the remaining patients. Some of the study results are in support of the recessive oncogene model for WT. However, the possible confounding effect of disease stage and the strong interdependence of the remaining factors may be masking important relations in regard to mutational events occurring during embryogenesis.
引用
收藏
页码:641 / 646
页数:6
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