Future directions in the diagnosis and medical treatment of adrenocortical carcinoma

摘要: Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. Discrimination between ACCs and adrenocortical adenomas (ACAs) remains challenging, the current gold standard being Weiss score, consisting of several histopathological characteristics. However, new markers like Ki67, marker for proliferation, staining reticulins are promising not only as it comes to identifying malignancy but also prognostic in patients ACC. Currently, surgery still curative treatment Mitotane, an adrenolytic drug, used adjuvant setting case metastatic or advanced disease. Patients progressive frequently treated mitotane, alone combination etoposide, doxorubicine cisplatin. Radiotherapy indicated selected cases. The low response rates high toxicity systemic therapies emphasize need that enable identification responders non-responders. Consequently, research focusing on predictive factors varying from expression DNA repair genes clinical patient Subgroups ACC different prognosis have been identified based transcriptome As conclusion large molecular studies, appear harbor many abnormalities compared ACAs. Altered pathways driving pathogenesis include IGF, TP53 Wnt signaling pathway, allowing these potential targets medical therapy. despite efforts preclinical studies investigating efficacy targeting pathways, most novel be effective subset New concepts therefore urgently needed.