SHM Converge Scientific Abstract ePoster Gallery-Cort Is in Session: Unraveling an Endocrine Mystery of Pressure, Protein, and Potassium

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This case describes a 55-year-old woman with hypertension, diabetes, chest pain, hypertensive emergency, severe hypokalemia, hyperglycemia, cushingoid features, anasarca, and nephrotic-range proteinuria. Imaging showed a heterogeneous 4 cm left adrenal mass with lymphadenopathy above and below the diaphragm. Biochemical testing revealed ACTH-independent hypercortisolism with elevated cortisol after dexamethasone suppression testing, suppressed ACTH, elevated adrenal androgens (DHEA-S, androstenedione, testosterone), and low aldosterone with high renin, consistent with a functional adrenal cortical tumor.<br /><br />Because pheochromocytoma had to be excluded before biopsy, metanephrines were checked and were not suggestive of catecholamine excess. A para-aortic lymph node biopsy ultimately confirmed high-grade, poorly differentiated adrenocortical carcinoma (ACC). She also underwent renal evaluation for unexpectedly heavy proteinuria, and kidney biopsy showed focal segmental glomerulosclerosis (FSGS).<br /><br />The case highlights several key teaching points. First, all adrenal tumors require hormonal evaluation, even when malignancy is suspected, because management depends heavily on hormone excess. Standard workup includes screening for cortisol excess, assessing ACTH independence if cortisol is elevated, checking metanephrines when pheochromocytoma is possible, and measuring renin/aldosterone in patients with hypertension or hypokalemia. DHEA-S and testosterone may help identify virilizing tumors or ACC.<br /><br />Second, profound hypercortisolism can cause apparent mineralocorticoid excess. Normally, cortisol is inactivated in the distal nephron by 11β-hydroxysteroid dehydrogenase type 2; however, when cortisol levels are very high, this enzyme is overwhelmed, allowing cortisol to activate mineralocorticoid receptors and causing severe hypertension and hypokalemia.<br /><br />Third, the coexistence of hypercortisolism and FSGS is unusual but may reflect a pathophysiologic link, since prior reports suggest FSGS can improve after correction of hypercortisolism. Initial treatment included blood pressure control, spironolactone, potassium replacement, and insulin. Due to extensive metastatic disease and poor performance status, she was not a candidate for surgery, chemotherapy, or mitotane, so ketoconazole was started for cortisol control and she was discharged to hospice.

Asset Subtitle

Jonathon Heath

Meta Tag

Author List Allison Bridge, David Hemsey, Emily Sturkie, Jonathon Heath

Category Clinical Vignettes

Concept Adrenal Tumor

Concept Cortisol Excess

Concept ACTH-Independent Hypercortisolism

Concept Adrenocortical Carcinoma

Concept Hormonal Evaluation

Distinguished Non-Finalist

Presenter Organization UNC Hospital

Presenting Author Jonathon Heath

Track Adult

Keywords

adrenocortical carcinoma

hypercortisolism

ACTH-independent

adrenal mass

hypokalemia

hypertension

focal segmental glomerulosclerosis

nephrotic-range proteinuria

cushingoid features

metanephrines

Adrenal Tumor

Cortisol Excess

ACTH-Independent Hypercortisolism

Adrenocortical Carcinoma

Hormonal Evaluation