Ces Urol 2025, 29(2):69-75 | DOI: 10.48095/cccu2025012
Adrenocortical carcinoma (ACC) is a rare but highly aggressive malignancy of the adrenal cortex, accounting for less than 0.2% of all solid tumors. Despite its low incidence, ACC is associated with significant morbidity and mortality and requires a multidisciplinary diagnostic and therapeutic approach. This review summarizes current knowledge of the epidemiology, etiology, diagnostics, staging, treatment strategies, and follow-up of ACC, with an emphasis on the specifics of Czech clinical urological practice. Special attention is paid to the integration of hormonal diagnostics, imaging modalities (including advanced PET/MR and novel tracers), and genetic aspects relevant to ACC pathogenesis. Surgical resection remains the cornerstone of curative treatment, complemented by adjuvant therapy with mitotane and, in advanced cases, cytotoxic chemotherapy. Emerging therapeutic options, including immunotherapy and targeted agents, are also discussed. Given the rarity and complexity of ACC, centralization of care, adherence to international guidelines, and interprofessional collaboration are essential to improving patient outcomes in the Czech Republic.
Received: April 8, 2025; Revised: May 2, 2025; Accepted: May 4, 2025; Prepublished online: May 22, 2025; Published: June 24, 2025