Pang S, Becker D, Cotelingam J, Foley T P, Drash A L
Pediatrics. 1981 Aug;68(2):242-6.
An adrenal cortical tissue tumor developed in a patient with poorly controlled salt-losing congenital adrenal hyperplasia. A 16-year-old girl became progressively virilized from 13 to 16 years of age. Base line serum progesterone, 17-hydroxyprogesterone, and testosterone levels were high and there was a diurnal pattern of the hormones. Initially elevated urinary 17-ketosteroid and serum steroid levels were decreased by high dose dexamethasone therapy, and at laparotomy an adenoma was found in the cortex of the hyperplastic left adrenal gland. It is inferred that persistent adrenocorticotrophic hormone stimulation may result in neoplastic transformation of hyperplastic adrenal cortical tissue in patients with congenital adrenal hyperplasia.
一名失盐型先天性肾上腺皮质增生症控制不佳的患者发生了肾上腺皮质组织肿瘤。一名16岁女孩在13至16岁期间逐渐出现男性化。基线血清孕酮、17-羟孕酮和睾酮水平升高，且激素存在昼夜节律。高剂量地塞米松治疗使最初升高的尿17-酮类固醇和血清类固醇水平降低，剖腹手术时在增生的左肾上腺皮质发现了一个腺瘤。据推测，持续性促肾上腺皮质激素刺激可能导致先天性肾上腺皮质增生症患者增生的肾上腺皮质组织发生肿瘤转化。
