Adrenal cortical carcinoma
Adrenal cortical carcinoma is a rare, aggressive adrenal tumor that frequently secretes cortisol, androgens, or both, presenting with rapid-onset Cushing syndrome, virilization, or an incidentally discovered large adrenal mass. First-line therapy for localized disease is open resection, with adjuvant mitotane to reduce recurrence, and combination chemotherapy (etoposide, doxorubicin, cisplatin) plus mitotane for advanced or metastatic disease; mitotane-induced adrenal insufficiency requires lifelong glucocorticoid, and often mineralocorticoid, replacement.
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What this article covers
- Overview
Adrenal cortical carcinoma (ACC) is a rare, aggressive malignant tumor of the adrenal cortex that often secretes excess hormone, most commonly cortisol, and frequently presents late because…
- Epidemiology
ACC is rare, in contrast to the far more common benign, nonfunctioning adrenal adenoma, which accounts for most adrenal masses discovered incidentally.
- Risk factors
A larger adrenal mass on imaging (usually greater than 4 cm) raises pretest probability of ACC compared with the typical benign adenoma, which is usually smaller than 4 cm.
- Pathophysiology
ACC arises from the adrenal cortex and, unlike most benign adrenal adenomas, frequently retains the capacity for autonomous, high-level steroid hormone production, particularly cortisol and adrenal androgens.
- Clinical features
Rapid onset of Cushing syndrome, with or without virilization, is a hallmark presentation and should raise concern for ACC (or an aggressive ectopic ACTH-secreting tumor) rather than a benign, slowly evolving cortisol-secreting adenoma.
- Differential diagnosis
Benign, nonfunctioning adrenal adenoma: typically under 4 cm with a CT density below 10 Hounsfield units, homogeneous enhancement, and contrast washout greater than 50% at 10 minutes, unlike the…
- Diagnosis
The diagnosis rests on a large or imaging-malignant adrenal mass with biochemical confirmation of autonomous cortisol and/or androgen excess, since neither size nor hormone secretion alone is…
- Management
First, for localized, resectable disease, perform open surgical resection, the first-line and only potentially curative treatment; laparoscopic resection is avoided when ACC is strongly suspected…
- Complications
Adrenal insufficiency from mitotane therapy, occurring in essentially all treated patients, requiring lifelong glucocorticoid (and often mineralocorticoid) replacement for as long as mitotane is used.
- Prognosis
Five-year survival is 62% to 82% for disease confined to the adrenal gland at diagnosis, reflecting the importance of early detection and complete resection.
- Prevention
There is no primary prevention for ACC; management instead focuses on early detection through structured evaluation of every incidentally discovered adrenal mass.
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Related articles
- Adrenal incidentalomas
An adrenal incidentaloma is an adrenal mass 1 cm or larger discovered on imaging obtained for an unrelated reason, most often a benign nonfunctioning adenoma but requiring evaluation for hormone hypersecretion and malignancy in every patient. Management is surgical excision for functioning tumors, masses 4-6 cm or larger, or those with malignant imaging features, and periodic monitoring for smaller nonfunctioning lesions with benign imaging characteristics.
- Cushing syndrome
Cushing syndrome is the clinical state of chronic glucocorticoid excess from any cause, most often exogenous glucocorticoid use, diagnosed by demonstrating loss of normal cortisol suppression or diurnal rhythm with at least two of three screening tests. Treatment targets the underlying source: withdrawal or tapering of exogenous steroid, transsphenoidal surgery for a pituitary cause, or adrenalectomy for an adrenal or ectopic tumor, with steroidogenesis inhibitors reserved for persistent or unresectable disease.
- Primary adrenal insufficiency
Primary adrenal insufficiency (Addison disease) is destruction or dysfunction of the adrenal cortex, most often autoimmune, causing combined cortisol and aldosterone deficiency with hyperpigmentation, hyponatremia, and hyperkalemia. Lifelong glucocorticoid replacement with hydrocortisone plus mineralocorticoid replacement with fludrocortisone is required, with sick-day dose increases to prevent adrenal crisis.