Korwutthikulrangsri Manassawee, Wattanatranon Duangkamon, Teeraratkul Sumate, Wijarn Piyathida, Mahachoklertwattana Pat, Poomthavorn Preamrudee
a Faculty of Medicine, Department of Pediatrics , Ramathibodi Hospital, Mahidol University , Bangkok , Thailand.
b Faculty of Medicine, Department of Pathology , Ramathibodi Hospital, Mahidol University , Bangkok , Thailand.
Paediatr Int Child Health. 2018 Feb;38(1):66-68. doi: 10.1080/20469047.2016.1262577. Epub 2017 Jan 23.
Adrenocortical tumours are rare in children. Virilisation caused by overproduction of adrenal androgens is the most common presentation. The testes of pre-pubertal boys with this tumour are usually small or of pre-pubertal size. A 4.8-year-old boy with an adrenocortical tumour and symmetrical pubertal-sized testes is reported. The serum testosterone level was 204 nmol/L (<0.7), dehydro-epiandrosterone-sulphate 56.7 μmol/L (<1.5) and luteinising and follicle-stimulating hormones were at suppressed levels. Histology demonstrated a diffusely increased mean tubular diameter of 90 μm (the size in a 12-year-old boy) and hyperplasia of Sertoli cells. There were no Leydig cells in the interstitial area. Prolonged exposure to an extraordinarily high testosterone level could have had stimulating effects on the seminiferous tubules and Sertoli cell growth and thus contributed to testicular enlargement.
肾上腺皮质肿瘤在儿童中较为罕见。肾上腺雄激素分泌过多导致的男性化是最常见的表现。患有这种肿瘤的青春期前男孩的睾丸通常较小或处于青春期前大小。本文报告了一名4.8岁患有肾上腺皮质肿瘤且睾丸大小如青春期的男孩，其双侧睾丸对称。血清睾酮水平为204 nmol/L（<0.7），硫酸脱氢表雄酮为56.7 μmol/L（<1.5），黄体生成素和促卵泡激素水平均受到抑制。组织学检查显示生精小管平均直径弥漫性增加至90μm（12岁男孩的大小），支持细胞增生。间质区域未见 Leydig 细胞。长期暴露于异常高的睾酮水平可能对生精小管和支持细胞的生长产生刺激作用，从而导致睾丸增大。
