Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan.
Endocr J. 2009;56(8):975-82. doi: 10.1507/endocrj.k09e-146. Epub 2009 Aug 12.
We present a 6-year-old boy with a virilizing adrenocortical tumor who initially presented with peripheral precocious puberty. Development of facial acne, pubic hair and a growth spurt were noted at the age of five. A low-pitched voice as well as maturation of external genitalia was noted at the age of six. Both serum and urinary levels of adrenal androgens were elevated. Abdominal computed tomography revealed a large right suprarenal mass and he underwent surgical resection without any complications. The histological diagnosis was adrenocortical carcinoma according to the criteria of Weiss. Following surgical removal of the androgen-producing tumor, the patient subsequently developed hypothalamic-pituitary activation and demonstrated central precocious puberty. He was treated with a gonadotropin-releasing hormone agonist in order to delay further pubertal progression. Clinical follow-up of potential secondary effects of excess hormone secretion after removal is important in some pediatric patients with virilizing adrenocortical tumor.
我们报告一例 6 岁男孩患有促雄性化肾上腺皮质肿瘤，最初表现为外周性性早熟。5 岁时出现面部痤疮、阴毛和生长突增。6 岁时出现低音调嗓音和外生殖器成熟。血清和尿液中的肾上腺雄激素水平均升高。腹部计算机断层扫描显示右侧肾上腺有一个大肿块，他没有任何并发症就接受了手术切除。根据 Weiss 的标准，组织学诊断为肾上腺皮质癌。在切除产生雄激素的肿瘤后，患者随后出现了下丘脑-垂体激活，并表现出中枢性性早熟。为了延缓进一步的青春期进展，他接受了促性腺激素释放激素激动剂治疗。对于一些患有促雄性化肾上腺皮质肿瘤的儿科患者，切除后分泌过多激素的潜在继发效应的临床随访非常重要。
