Noordam C, Hermus A R M M, Pesman G, N'Diaye N, Sweep C G J, Lacroix A, Otten B J
Department of Paediatric Endocrinology, University Medical Center, Nijmegen, The Netherlands.
J Pediatr Endocrinol Metab. 2002 Jun;15(6):853-60. doi: 10.1515/jpem.2002.15.6.853.
ACTH-independent Cushing's syndrome may be secondary to the expression of ectopic hormone receptors in adrenal tissue. In food-dependent Cushing's syndrome the adrenals aberrantly express receptors for gastric inhibitory polypeptide (GIP). We present the first case of food- and GIP-dependent adrenal adenoma in an adolescent. A 15 year-old girl presented with signs of hypercortisolism. Urinary cortisol excretion was clearly elevated. Upon the finding of very low fasting plasma cortisol levels with a rise during the morning, food-dependent cortisol secretion was suspected. Hourly measurements of plasma cortisol and GIP levels during a day with and a day without meals showed meal- and GIP-related cortisol secretion. Plasma ACTH levels were undetectable. Abdominal computed tomography showed a tumor of 2.5 x 2.5 x 2 cm in the left adrenal. Unilateral adrenalectomy was performed and microscopic examination of the tumor showed an adrenocortical adenoma. Incubation of adenomatous cells prepared from this tumor with GIP resulted in increased cortisol secretion. Using RT-PCR amplification and cDNA hybridization, the GIP receptor was found to be overexpressed in the adenoma tissue but not in the adjacent adrenal tissue. Twelve months after the operation, hourly measurements of plasma cortisol, GIP and ACTH levels on a day with and a day without meals no longer showed GIP-related cortisol production. The present report shows that in an adolescent with corticotrophin-independent Cushing's syndrome, food-dependent hypercortisolism is a possible diagnosis.
促肾上腺皮质激素（ACTH）非依赖性库欣综合征可能继发于肾上腺组织中异位激素受体的表达。在食物依赖性库欣综合征中，肾上腺异常表达胃抑制多肽（GIP）受体。我们报告了首例青少年食物及GIP依赖性肾上腺腺瘤病例。一名15岁女孩出现皮质醇增多症体征。尿皮质醇排泄明显升高。发现空腹血浆皮质醇水平极低且早晨有所升高后，怀疑存在食物依赖性皮质醇分泌。在有进食和无进食的一天中每小时测量血浆皮质醇和GIP水平，结果显示皮质醇分泌与进食及GIP有关。血浆ACTH水平检测不到。腹部计算机断层扫描显示左肾上腺有一个2.5×2.5×2厘米的肿瘤。进行了单侧肾上腺切除术，肿瘤的显微镜检查显示为肾上腺皮质腺瘤。用GIP孵育从该肿瘤制备的腺瘤细胞导致皮质醇分泌增加。通过逆转录-聚合酶链反应（RT-PCR）扩增和cDNA杂交发现，GIP受体在腺瘤组织中过度表达，但在相邻肾上腺组织中未过度表达。术后12个月，在有进食和无进食的一天中每小时测量血浆皮质醇、GIP和ACTH水平，不再显示与GIP相关的皮质醇产生。本报告表明，在一名患有促肾上腺皮质激素非依赖性库欣综合征的青少年中，食物依赖性皮质醇增多症是一种可能的诊断。
