When Cortisol Overwhelms the Heart: A Fatal Case of Metastatic Adrenocortical Carcinoma Presenting as Acute Heart Failure
https://doi.org/10.15605/jafes.041.S1
- Author:
Tze Liang Lee
1
;
Shaleni Nagappen
1
;
Deviga Latchumanan
1
Author Information
1. Department of Internal Medicine, Hospital Port Dickson
- Publication Type:Journal Article
- MeSH:
Adrenocortical Carcinoma;
Hydrocortisone;
Heart Failure
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):31-
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Cushing’s syndrome is a multisystem disorder with significant cardiovascular morbidity, yet presentation as acute
heart failure is uncommon. When driven by adrenocortical
carcinoma (ACC), the clinical course is often aggressive and
rapidly fatal, with particularly poor out-comes in resourcelimited settings where access to therapy is constrained.
Case:A 39-year-old previously well female presented with acute
decompensated heart failure, newly diagnosed hypertension, type 2 diabetes mellitus, and obesity, preceded
by a 3-year history of secondary amenorrhea and progressive weight gain. On admission, she exhibited florid
Cushingoid features. Biochemical evaluation confirmed
severe adrenocorticotropic hormone (ACTH)-independent
hypercortisolism: morning serum cortisol 1,950 nmol/L,
failure of suppression on overnight dexamethasone
suppression test (post-ODST cortisol 2022.9 nmol/L),
and elevated 24-hour urinary free cortisol (2,069 nmol/24
hours, 2.56 × upper limit of normal). Androgen excess was
evident, with elevated dehydroepiandrosterone sulfate
(DHEAS more than 27 µmol/L) and testosterone (9.91
nmol/L). ACTH was suppressed, supporting an adrenal
source, while aldosterone was normal. Contrast-enhanced
computed tomography demonstrated a large left adrenal
mass (12 cm) with tumor thrombus extending into the
inferior vena cava and renal veins, with extensive hepatic
and pulmonary metastases, consistent with advanced ACC.
Management was limited by disease severity and resource
constraints. Ketoconazole was contraindicated due to
transaminitis, and alternative steroidogenesis inhibitors
were unavailable, leaving metyrapone as the only feasible
option. Oncological therapy was deferred due to sepsis and
clinical instability. Her course was fulminant, complicated
by recurrent heart failure, sepsis, and metabolic derangements, culminating in refractory cardiopulmonary failure.
She died within 1 month of diagnosis, prior to definitive
oncological intervention.
Conclusion:Fulminant cortisol-secreting ACC may present catastrophically as acute heart failure and progress rapidly. Early
recognition and timely access to multimodal cortisollowering therapy are critical, particularly in resourcelimited settings. In fulminant hypercortisolism, the
challenge is not diagnosis—but timing.
- Full text:202607281513134261EP_A023.pdf
