title: [Pathogenesis of adrenocortical cancer].
abstract: Adrenocortical cancer is a rare tumor with poor prognosis. Whereas most cases occur in a sporadic setting, there are very rare hereditary forms that are important for the understanding of tumor pathogenesis. The hereditary syndromes associated with adrenocortical cancer are: Li-Fraumeni's syndrome, Beckwith-Wiedemann's syndrome and familial adenomatous polyposis, whereas multiple endocrine neoplasia type 1, Carney's complex and McCune-Albright's syndrome mostly predispose to benign adrenocortical tumors. Overexpression of insulin like growth factor 2, activation of Wnt/beta-catenin and cAMP-protein kinase A signaling, as well as mutations of p53 and MEN1 genes are regarded as major pathogenetic mechanisms. Options for medical treatment of adrenocortical cancer are rather limited. Recently published molecular-bioinformatical studies have revealed several previously unknown pathogenetic pathways that may even represent potential drug targets. In this study, the pathogenesis of hereditary tumor syndromes, the alterations in sporadic tumors and the most recent molecular-bioinformatical observations are discussed.
authors.affiliation: Semmelweis Egyetem, Altalános Orvostudományi Kar, II. Belgyógyászati Klinika, Budapest.
authors.forename: Diána
authors.initials: D
authors.lastname: Szabó
authors.forename: Adrienn
authors.initials: A
authors.lastname: Zsippai
authors.forename: Melinda
authors.initials: M
authors.lastname: Bendes
authors.forename: Zsófia
authors.initials: Z
authors.lastname: Tömböl
authors.forename: Péter M
authors.initials: PM
authors.lastname: Szabó
authors.forename: Károly
authors.initials: K
authors.lastname: Rácz
authors.forename: Péter
authors.initials: P
authors.lastname: Igaz
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000235:genetics; D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy / Q000201:enzymology / Q000235:genetics* / Q000378:metabolism*; D001506:Beckwith-Wiedemann Syndrome / Q000235:genetics; D056733:Carney Complex / Q000235:genetics; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / Q000378:metabolism; D005359:Fibrous Dysplasia, Polyostotic / Q000235:genetics; D015972:Gene Expression Regulation, Neoplastic; D016158:Genes, p53; D020022:Genetic Predisposition to Disease; D006801:Humans; D007335:Insulin-Like Growth Factor II / Q000378:metabolism; D016864:Li-Fraumeni Syndrome / Q000235:genetics; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics; D009154:Mutation; D011518:Proto-Oncogene Proteins / Q000235:genetics; D015398:Signal Transduction; D015854:Up-Regulation; D051153:Wnt Proteins / Q000378:metabolism; D051176:beta Catenin / Q000378:metabolism
chemical_list: C495270:CTNNB1 protein, human; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; C105373:MEN1 protein, human; D011518:Proto-Oncogene Proteins; D051153:Wnt Proteins; D051176:beta Catenin; D007335:Insulin-Like Growth Factor II