J Orrego John, A Chorny Joseph
Departments of Endocrinology and Metabolism, Kaiser Foundation Health Plan of Colorado, Denver, Colorado, USA.
Pathology, Kaiser Foundation Health Plan of Colorado, Denver, Colorado, USA.
Endocrinol Diabetes Metab Case Rep. 2020 Sep 23;2020. doi: 10.1530/EDM-20-0121.
We describe a 56-year-old postmenopausal woman with hypertension, hypokalemia and severe alopecia who was found to have a 4.5-cm lipid-poor left adrenal mass on CT scan performed to evaluate her chronic right-sided abdominal pain. Hormonal studies revealed unequivocal evidence of primary aldosteronism and subclinical hypercortisolemia of adrenal origin. Although a laparoscopic left adrenalectomy rendered her normotensive, normokalemic and adrenal insufficient for 2.5 years, her alopecia did not improve and she later presented with facial hyperpigmentation acne, worsening hirsutism, clitoromegaly, and an estrogen receptor-positive breast cancer. Further testing demonstrated markedly elevated serum androstenedione and total and free testosterone and persistently undetectable DHEAS levels. As biochemical and radiologic studies ruled out primary adrenal malignancy and obvious ovarian neoplasms, a bilateral salpingo-oophorectomy was undertaken, which revealed bilateral ovarian hyperthecosis. This case highlights how the clinical manifestations associated with hyperaldosteronism and hypercortisolemia masqueraded the hyperandrogenic findings. It was only when her severe alopecia failed to improve after the resolution of hypercortisolism, hyperandrogenic manifestations worsened despite adrenal insufficiency and an estrogen receptor-positive breast cancer was found, did it becomes apparent that her symptoms were due to ovarian hyperthecosis.
As cortisol cosecretion appears to be highly prevalent in patients with primary aldosteronism, the term 'Connshing' syndrome has been suggested. The associated subclinical hypercortisolemia could be the driver for the increased metabolic alterations seen in patients with Conn syndrome. The identification of these dual secretors before adrenal venous sampling could alert the clinician about possible equivocal test results. The identification of these dual secretors before unilateral adrenalectomy could avoid unexpected postoperative adrenal crises. Hyperfunctioning adrenal and ovarian lesions can coexist, and the clinical manifestations associated with hypercortisolemia can masquerade the hyperandrogenic findings.
我们描述了一名56岁的绝经后女性，患有高血压、低钾血症和严重脱发，在为评估其慢性右侧腹痛而进行的CT扫描中发现左肾上腺有一个4.5厘米的低脂质肿块。激素研究明确显示有原发性醛固酮增多症和肾上腺源性亚临床皮质醇增多症的证据。尽管腹腔镜下左肾上腺切除术使她血压正常、血钾正常且肾上腺功能不全持续了2.5年，但她的脱发并未改善，后来还出现了面部色素沉着、痤疮、多毛症加重、阴蒂肥大以及雌激素受体阳性乳腺癌。进一步检查显示血清雄烯二酮、总睾酮和游离睾酮显著升高，而硫酸脱氢表雄酮水平持续检测不到。由于生化和影像学研究排除了原发性肾上腺恶性肿瘤和明显的卵巢肿瘤，遂进行了双侧输卵管卵巢切除术，结果显示双侧卵巢卵泡膜细胞增生症。该病例突出了与醛固酮增多症和皮质醇增多症相关的临床表现如何掩盖了高雄激素血症的表现。直到她在皮质醇增多症缓解后严重脱发仍未改善，尽管肾上腺功能不全但高雄激素血症表现仍加重，且发现了雌激素受体阳性乳腺癌，才明显看出她的症状是由卵巢卵泡膜细胞增生症引起的。
由于在原发性醛固酮增多症患者中皮质醇共分泌似乎非常普遍，因此有人提出了“康辛氏”综合征这一术语。相关的亚临床皮质醇增多症可能是导致康氏综合征患者出现更多代谢改变的原因。在进行肾上腺静脉采血之前识别这些双重分泌者，可以提醒临床医生注意可能出现的模棱两可的检查结果。在进行单侧肾上腺切除术之前识别这些双重分泌者，可以避免意外的术后肾上腺危象。肾上腺和卵巢功能亢进性病变可能共存，与皮质醇增多症相关的临床表现可以掩盖高雄激素血症的表现。
