Lim Kia Teng, Loh Amos H P
Ministry of Health Holdings, Singapore 139691, Singapore.
VIVA-KKH Paediatric Brain and Solid Tumour Programme, Children's Blood and Cancer Centre, KK Women's and Children's Hospital, Singapore 229899, Singapore.
Cancers (Basel). 2024 Sep 1;16(17):3051. doi: 10.3390/cancers16173051.
Wilms tumor is the commonest primary renal malignancy in children and demonstrates substantial inter-ethnic variation in clinical, pathological, and molecular characteristics. Wilms tumor occurs at a lower incidence and at a younger age in Asians compared to Caucasians and Africans. Asians also present at an earlier stage of disease, with a higher incidence of favorable histology tumors and a lower incidence of perilobar nephrogenic rests compared to Caucasians, while African children present with more advanced disease. Studies have implicated population differences in the incidence of WT1 mutations, loss of imprinting of the IGF2 locus, and loss of heterozygosity of 1p/16q, or 1q gain as possible bases for epidemiological differences in the disease profile of Wilms tumors in various ethnic groups. Yet, evidence to support these associations is confounded by differences in treatment protocols and inequalities in the availability of treatment resources and remains limited by the quality of population-based data, especially in resource-limited settings.
肾母细胞瘤是儿童最常见的原发性肾脏恶性肿瘤,在临床、病理和分子特征方面存在显著的种族间差异。与白种人和非洲人相比,亚洲人肾母细胞瘤的发病率较低,发病年龄也较小。与白种人相比,亚洲人疾病分期更早,预后良好组织学类型肿瘤的发病率更高,叶旁肾源性残留的发病率更低,而非洲儿童的疾病则更为晚期。研究表明,WT1突变的发生率、IGF2基因座印记缺失、1p/16q杂合性缺失或1q获得方面的人群差异,可能是各民族肾母细胞瘤疾病谱流行病学差异的潜在原因。然而,支持这些关联的证据因治疗方案的差异以及治疗资源可及性的不平等而受到混淆,并且仍然受到基于人群数据质量(尤其是在资源有限环境中)的限制。