(en) [Adrenocortical carcinomas: The usefulness of a multidisciplinary approach] Adrenocortical carcinomas (ACC) are rare tumors with an incidence of 1 to 2 cases per million per year. These tumors are often aggressive, exhibiting a poor outcome. About two-third of these tumors secrete adrenal steroids and can, over time, lead to Cushing's syndrome or virilization, and less frequently to hyperaldosteronism or feminization. The diagnostic work-up must include a complete general and hormonal evaluation, as well as detailed imaging studies, including CT scan or MRI and 18fluorodeoxyglucose-PET. Tumor histopathology and immunohistochemistry are essential to confirm diagnosis, determine tumor grade, and assess prognosis. ACC treatment should be multidisciplinary. Complete surgical resection is the only curative treatment modality. Mitotane is an adrenolytic drug specifically used in advanced or recurrent disease, often in combination with other chemotherapeutic agents. The benefits of mitotane as an adjuvant therapy after surgical resection have been suggested in retrospective studies, but must still be confirmed in larger randomized trials. Prognosis has been shown to have improved over recent years due to earlier and more accurate diagnosis, early aggressive surgery, and better chemotherapy regimens. Promising new therapies, based on molecular tumor biology, are currently tested in clinical trials.
De La Kéthulle, C., Mourad, M., Cornelis, F., Humblet, Y., & Maiter, D. (2015). Les adénocarcinomes cortico-surrénaliens. Intérêt d’une prise en charge multidisciplinaire. Louvain médical. https://hdl.handle.net/2078.5/185977