Southern Hills Hospital & Medical Center
adrenocortical carcinoma, malignant tumors, carcinomas
Endocrine System Diseases | Endocrinology, Diabetes, and Metabolism | Internal Medicine | Neoplasms
Introduction: Adrenocortical carcinoma (ACC) is a rare and biologically aggressive malignancy. The incidence of ACC is between 0.7-2 cases per million, accounting for 0.05-2% of all malignant tumors. Cortisol-producing carcinomas are the most common, with clinical features consistent with hypercortisolism: refractory hypertension, glucose intolerance, and central obesity. We present a case of a 75-year-old male with a cortisol-secreting variant of adrenocortical carcinoma.
Case Description: A 75 year-old male with a prior medical history significant for refractory hypertension on multiple anti-hypertensives including hydrochlorothiazide, type two diabetes mellitus, and untreated obstructive sleep apnea, presented to the emergency department with a blood pressure of 259/123 and in respiratory distress. His work up included a chest CTA, revealing a heterogeneous slightly lobulated, macroscopic fat-containing mass in the left adrenal gland, measuring 4cm. This was interval increase from 2.1cm five years ago; radiology favored this to represent either an adrenal myelolipoma or adenoma. Nephrology was consulted for management of his hypertension and assessment of the adrenal mass. Total urine metanephrines were 505 mcg/24h and total urine catecholamines were/24h, inconsistent with pheochromocytoma. CTA abdomen/pelvis revealed no evidence of renal artery stenosis. His mild hypokalemia was attributed to thiazide use. At discharge, he started spironolactone in lieu of thiazides, but plasma-aldosterone-concentration to plasma-renin-activity [PAC/PRA] was pending. The ratio returned equivocal for primary hyperaldosteronism at 23. A year later, he was hospitalized for methicillin-sensitive Staphylococcus aureus pneumonia, extended spectrum beta-lactamase (ESBL) E. coli pneumonia, and ESBL 2020 E. coli bacteremia. The same left adrenal mass now measured 7.1cm x 5.8cm with extensive mediastinal and hilarlymphadenopathy. Pathology of four CT-guided adrenal biopsies was consistent with benign adrenocortical neoplasm, favor adenoma, with a caveat that mass resection would give a definitive diagnosis. Fine-needle aspiration of a sub-carinal lymph node revealed malignant cells consistent with metastatic adrenocortical carcinoma. Immunohistochemical evaluation stained positive for Synaptophysin, Melan-A, and GATA 3. Urine cortisol levels returned at 2259.6 mcg/g, consistent with glucocorticoid hypersecretion. AM cortisol was 147mcg/dL and ACTH was 10pg/mL; dexamethasone suppression test was not performed as the patient transitioned to hospice.
Discussion: Approximately 80% of adrenal incidentalomas will be non-functional; adrenocortical carcinoma accounts for less than 4% of functional tumors. When the mass measured 2.1cm, AM cortisol measured 19.9 mcg/dL. In the following five years, multiple CT scans denoted the incidentaloma as “an adenoma” or “myelolipoma”, despite its growth. Aldosterone was not elevated (<15ng/dL) during evaluation for primary hyperaldosteronism and aldosterone-renin ratio was equivocal. Spironolactone use may have masked the symptoms elevated mineralocorticoids, resulting from excessive cortisol. At the time of diagnosis, his endocrine work up was likely clouded by septic shock necessitating stress-dose steroids. This patient had an elusive diagnosis, with a poor chance of detection, despite longitudinal findings.
Publisher or Conference
American College of Physicians Colorado Chapter Meeting
Khoie S, Scherbak D. Adrenocortical Carcinoma: A stressful diagnosis. Poster presented at: ACP Colorado Chapter Meeting; February 6-9, 2020; Colorado Springs, CO.