Variant of pre-clinical Cushing's syndrome: hypertension and hypokalemia associated with normoreninemic normoaldosteronism.
The case of a 48-year-old woman with a left adrenocortical adenoma and showing hypokalemia, hypertension and normoreninemic normoaldosteronism is reported. Basal plasma adrenocorticotrophic hormone (ACTH) and cortisol levels were within the reference ranges. The patient's plasma cortisol level decreased insufficiently at night, and was insufficiently decreased by nighttime administration of dexamethasone. She showed no Cushingnoid stigmata. Iodocholesterol scintigraphy revealed tumor-sided uptake alone. The plasma dehydroepiandrosterone-sulfate level was low-to-normal for her age. Metabolic alkalosis and increased potassium clearance after sodium thiosulfate loading were revealed. The plasma aldosterone level was within the normal range, but it was statistically higher than the range for patients with pre-clinical Cushing's syndrome. However, peripheral plasma renin activity (PRA) increased normally after the patient resumed an upright posture following furosemide administration. After adenomectomy the hypokalemia and hypertension were resolved, and the plasma ACTH, cortisol, and PRA remained within the reference ranges. The plasma aldosterone level decreased slightly, but also remained within the reference range after adenomectomy. Paradoxical hyperplasia in the non-neoplastic adrenal glomerulosa zone, which indicates primary aldosteronism, and slight atrophy of the non-neoplastic adrenal cortex, which indicates pre-clinical Cushing's syndrome, were demonstrated. These findings satisfied the criteria of pre-clinical Cushing's syndrome, but did not completely satisfy those of primary aldosteronism. However, the level of CYP11 B2 mRNA in the tumor was in the lower-limit of the range for adenomas associated with primary aldosteronism and was higher than the ranges for adenomas associated with pre-clinical Cushing's syndrome and overt Cushing's syndrome. Based on these results, this case was suspected to constitute a variant of pre-clinical Cushing's syndrome with slight hypersecretion of aldosterone.
D000236:Adenoma / Q000473:pathology; D000302:Adrenal Cortex / Q000201:enzymology; D000310:Adrenal Gland Neoplasms / Q000473:pathology; D000311:Adrenal Glands / Q000378:metabolism; D000450:Aldosterone / Q000097:blood*; D003480:Cushing Syndrome / Q000097:blood* / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D019405:Cytochrome P-450 CYP11B2 / Q000235:genetics; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications*; D007008:Hypokalemia / Q000150:complications*; D007150:Immunohistochemistry; D008854:Microscopy, Electron; D008875:Middle Aged; D012333:RNA, Messenger / Q000378:metabolism; D012083:Renin / Q000097:blood*
D012333:RNA, Messenger; D000450:Aldosterone; D019405:Cytochrome P-450 CYP11B2; D012083:Renin