Hartley A L, Birch J M, Marsden H B, Reid H, Harris M, Blair V
Arch Dis Child. 1987 Jul;62(7):683-9. doi: 10.1136/adc.62.7.683.
Epidemiological data on the 14 cases of adrenal cortical tumour registered with the Manchester Children's Tumour Registry from 1954 and 1985 are presented. The incidence of adrenal cortical carcinomas was 0.3%, mainly in girls, most of whom presented with virilisation. The incidence of neoplastic disease among close relatives was ascertained, but, except in siblings, this was not significantly higher than would be expected. Evidence from extended pedigrees, however, indicates that at least four of the children could be members of families with the SBLA (sarcoma, breast and brain tumour, leukaemia, laryngeal and lung cancer, and adrenal cortical carcinoma) cancer family syndrome, and that other relatives may be at risk of developing such neoplasms.
本文呈现了1954年至1985年间在曼彻斯特儿童肿瘤登记处登记的14例肾上腺皮质肿瘤的流行病学数据。肾上腺皮质癌的发病率为0.3%，主要发生在女孩中，其中大多数表现为男性化。已确定近亲中肿瘤性疾病的发病率，但除了兄弟姐妹外，这一发病率并不显著高于预期。然而，来自扩展家系的证据表明，至少有4名儿童可能是患有SBLA（肉瘤、乳腺癌和脑肿瘤、白血病、喉癌和肺癌以及肾上腺皮质癌）癌症家族综合征家庭的成员，并且其他亲属可能有患此类肿瘤的风险。
