Verlinde Liesbeth, Kinet Sam, Van Den Heede Klaas, Brusselaers Nele, Van Slycke Sam
Faculty of Medicine, KU Leuven, Leuven, Belgium.
Department of General and Endocrine Surgery, Onze-Lieve-Vrouw (OLV) Hospital Aalst-Asse-Ninove, Aalst, Belgium.
Acta Chir Belg. 2025 Aug;125(4):202-210. doi: 10.1080/00015458.2025.2506935. Epub 2025 May 17.
Adrenal cortical carcinoma (ACC) is a rare and aggressive endocrine malignancy. Clinical symptoms are mainly related to excess hormone secretion. Hypercortisolism and virilisation are among the most common presentations.
We report a case series of five patients with ACC, three of which presented as adrenal incidentalomas. Additionally, a literature review on current diagnosis and management of ACC was performed.
ACCs are often incidentally detected because of the liberal use of medical imaging. Management of ACC remains challenging, and the poor prognosis makes early diagnosis of crucial importance to increase chances of a better outcome. Biochemical evaluation should be performed to diagnose hormonally active tumours.
Surgery is the main and only potentially curative treatment option. Adjuvant treatment with mitotane may improve survival and is indicated for patients with a perceived high risk of recurrence. Aggressive cytotoxic therapy should be given to patients with an unfavourable prognosis.
肾上腺皮质癌（ACC）是一种罕见且侵袭性强的内分泌恶性肿瘤。临床症状主要与激素分泌过多有关。库欣综合征和男性化是最常见的表现。
我们报告了一组5例肾上腺皮质癌患者的病例系列，其中3例表现为肾上腺偶发瘤。此外，对肾上腺皮质癌的当前诊断和治疗进行了文献综述。
由于医学成像的广泛应用，肾上腺皮质癌常被偶然发现。肾上腺皮质癌的治疗仍然具有挑战性，预后较差使得早期诊断对于提高获得更好结局的机会至关重要。应进行生化评估以诊断激素活性肿瘤。
手术是主要且唯一可能治愈的治疗选择。米托坦辅助治疗可能改善生存，适用于复发风险高的患者。对于预后不良的患者应给予积极的细胞毒性治疗。
