Journal
FRONTIERS IN ENDOCRINOLOGY
Volume 13, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fendo.2022.816754
Keywords
primary aldosteronism; aldosterone-producing adenoma (APA); aldosterone-producing micronodule (APM); autonomous cortisol secretion (ACS); KCNJ5 mutation; PRKACA mutations
Categories
Funding
- Ministry of Science and Technology, Taiwan, R.O.C. [MOST107- 2314-B-002-026-MY3, 108-2314-B-002-058, 109-2314-B-002-174-MY3]
- National Health Research Institutes [PH-102-SP-09]
- National Taiwan University Hospital [109-S4634, PC-1264, PC-1309, VN109-09, UN109-041, UN110-030, MOHW110-TDU-B-212-124005]
- Mrs. Hsiu-Chin Lee Kidney Research Fund [N10]
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This study reported a PA patient with concomitant ACS, showing that bilateral APM may coexist with histopathologically classical and non-classical PA adenomas with different somatic mutations.
BackgroundNon-classical multiple aldosterone-producing micronodules/nodules (mAPM/mAPN) could be the pathogenesis of primary aldosteronism (PA). The co-existence of mAPM with adenomas harboring somatic mutations has not previously been reported. MethodsWe presented a PA patient with bilateral mAPM and concomitant autonomous cortisol secretion (ACS). ResultsA 46-year-old Taiwanese woman presented with hypertension, hypokalemia, and bilateral adrenal adenomas. A 1 mg low-dose dexamethasone suppression test showed elevated morning serum cortisol. An adrenal vein sampling (AVS) suggested a left-sided lateralization of hyperaldosteronism. A right partial adrenalectomy and a left total adrenalectomy were performed. The patient showed biochemical and hypertension remission after the operation. This patient had bilateral mAPM with concomitant ACS, a right histopathologically classical PA adenoma, and a left non-classical PA adenoma. The right adrenal adenoma showed CYP11B1-negative and CYP11B2-positive staining and harbored the KCNJ5-L168R mutation. The left adrenal adenoma showed CYP11B1-positive and CYP11B2-negative staining and harbored the PRKACA-L206R mutation. ConclusionIn a PA patient with concomitant ACS, bilateral APM could coexist with both histopathologically classical and non-classical PA adenomas, each with different somatic mutations. The presence of ACS could lead to the misinterpretation of AVS results.
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