Abstract

Adrenal cortical carcinomas (ACCs) are rare adrenal tumors with a poor prognosis when diagnosed at an advanced stage. Early diagnosis is the key to assure prompt management and improve the prognosis. Surgery is the mainstay of therapy in patients with operable ACC, with R0 resection being the only curative intervention. Diagnosis of ACC is currently based on imaging phenotype and standard-of-care hormonal workup and may present a diagnostic dilemma when imaging characteristics and the hormonal workup are insufficient to make an accurate diagnosis. Noninvasive biomarkers that accurately distinguish ACC from other tumors could aid in making an earlier diagnosis and lead to a better overall prognosis. This review summarizes the current diagnostic approach to ACC including the emerging role of steroid biomarkers in the diagnosis of ACC.

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