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Adrenocortical carcinoma: towards genomics guided clinical care
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Tumor Biology.ORCID iD: 0000-0003-0677-4894
Klinikum Univ Munchen, Med Klin & Poliklin 4, Munich, Germany;Univ Spital Zurich, Klin Endokrinol Diabetol & Klin Ernahrung, Zurich, Switzerland.ORCID iD: 0000-0001-7826-3984
2019 (English)In: Nature Reviews Endocrinology, ISSN 1759-5029, E-ISSN 1759-5037, Vol. 15, no 9, p. 548-560Article, review/survey (Refereed) Published
Abstract [en]

Adrenocortical carcinoma (ACC) is an aggressive and rare neoplasm that originates in the cortex of the adrenal gland. The disease is associated with heterogeneous but mostly poor outcomes and lacks effective pharmaceutical treatment options. Multi-omics studies have defined the landscape of molecular alterations in ACC. Specific molecular signatures can be detected in body fluids, potentially enabling improved diagnostic applications for patients with adrenal tumours. Importantly, pan-molecular data sets further reveal a spectrum within ACC, with three major subgroups that have different disease outcomes. These new subgroups have value as prognostic biomarkers. Research has revealed that the p53-RB and the WNT-beta-catenin pathways are common disease drivers in ACC. However, these pathways remain difficult to target by therapeutic interventions. Instead, a unique characteristic of ACC is steroidogenic differentiation, which has emerged as a potential treatment target, with several agents undergoing preclinical or clinical investigations. Finally, a large proportion of ACC tumours have genetic profiles that are associated with promising therapeutic responsiveness in other cancers. All these opportunities now await translation from the laboratory into the clinical setting, thereby offering a real potential of improved survival outcomes and increased quality of life for patients with this serious condition.

Place, publisher, year, edition, pages
2019. Vol. 15, no 9, p. 548-560
National Category
Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-393685DOI: 10.1038/s41574-019-0221-7ISI: 000480419200011PubMedID: 31147626OAI: oai:DiVA.org:uu-393685DiVA, id: diva2:1354606
Funder
Swedish Cancer SocietyGerman Research Foundation (DFG), CRC/Transregio 205/1Wallenberg FoundationsTore Nilsons Stiftelse för medicinsk forskningAvailable from: 2019-09-25 Created: 2019-09-25 Last updated: 2019-09-25Bibliographically approved

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