Endocrine Abstracts

Aldosterone and cortisol coproducing adrenal adenoma is a rare type of primary aldosterone. Previous reports mostly describe single adrenocortical adenomas that simultaneously secrete aldosterone and cortisol. To date, only 7 cases of bilateral adrenocortical adenomas with independent functions of secreting different hormones have been reported in the published English literature. Here, we study an extremely rare case of a left adrenocortical adenoma secreting aldosterone and a right sebaceous adenoma secreting cortisol and explore the pathogenesis. The patient, a 29-year-old female, was diagnosed with gestational hypertension and hypokalemia. The examination showed that his PTC was significantly elevated (0 min PTC: 858 nmol/l) and ACTH was significantly suppressed (ACTH<1 ng/l). CT showed bilateral adrenal nodules/mass shadows, with the largest cross-sections on the right and left sides. They were 3.3×2.2 cm and 1.3×0.8 cm respectively. After the right adrenal tumor was removed, the pathology showed ‘adrenal sebaceous adenoma’. Postoperatively, the patient developed cortical hypofunction, with a PTC of 109 nmol/l at 0800 h, and was given supplementary treatment with hydrocortisone. However, the patient still had hypertension and hypokalemia after the operation. Four months later, biochemical tests showed that the sitting plasma aldosterone and renin concentrations were 14 ng/dl and 0.51 uIU/ml respectively. The captopril test and saline test both showed aldosterone cannot be suppressed. CXCR4 imaging showed that the maximum SUV of the left adrenal adenoma was 8.75 and the maximum SUV of the left normal adrenal tissue was 2.7. Therefore, the left adrenal adenoma was diagnosed as an aldosteronoma and the left adrenal tumor was resected. After surgery, the patient’s blood pressure and serum potassium returned to normal, and orthostatic aldosterone dropped to 10.5 ng/dl. Postoperative immunohistochemistry results showed: CYP11B1(+), CYP11B2(−) in the right tumor, and CYP11B2(+), CYP11B1(−) in the left tumor. Full-exome gene testing of the left and right tumors and peripheral blood revealed that the right cortisol tumor PRKACA c.167(exon7)T>G (p.L206R) and the left aldosteronoma KCNJ5 c.503(exon2)T> G(p.L168R) is considered pathogenic, but no abnormal mutations were found in the blood. At this point, through clinical manifestations and hormone testing, postoperative tumor tissue pathology and immunohistochemistry testing, and genetic testing of tumor tissue, we have identified a case of right-sided adrenal cortisoloma caused by PRKACA gene mutation and left-sided adrenal cortisoloma caused by KCNJ5 gene mutation. Adrenal aldosteronoma A case of an adrenocortical tumor secreting different hormones bilaterally.