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Enzo Lalli and Michaela Luconi

in several types of endocrine cancers through a variety of mechanisms ( Lima et al . 2017 ). Metastasis in adrenocortical carcinoma (ACC): do paradigms apply? ACC is a rare endocrine malignancy with a high risk of relapse and

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S G Creemers, P M van Koetsveld, F J van Kemenade, T G Papathomas, G J H Franssen, F Dogan, E M W Eekhoff, P van der Valk, W W de Herder, J A M J L Janssen, R A Feelders, and L J Hofland

Introduction Adrenocortical carcinoma (ACC) is a rare malignancy, with an estimated incidence of 0.7–2.0 cases per million population each year ( Kebebew et al . 2006 , Golden et al . 2009 , Fassnacht et al . 2013 , Kerkhofs et al

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Isobel C Mouat, Kei Omata, Andrew S McDaniel, Namita G Hattangady, Debnita Talapatra, Andi K Cani, Daniel H Hovelson, Scott A Tomlins, William E Rainey, Gary D Hammer, Thomas J Giordano, and Tobias Else

with PA have a benign adrenal adenoma or bilateral hyperplasia. Adrenocortical carcinoma (ACC), however, is a very rare cause of increased aldosterone levels, and a recent literature review identified only 58 reported patients ( Seccia et al . 2005

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Yunhui Cheng, Raili Emilia Kerppola, and Tom Klaus Kerppola

order to develop such compounds for therapeutic use, it is essential to determine their efficacy in cell and animal models of cancer and to characterize their molecular mechanisms of action. Adrenocortical carcinoma (ACC) is an aggressive disease with

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Meenu Jain, Lisa Zhang, Mei He, Ya-Qin Zhang, Min Shen, and Electron Kebebew

Introduction Adrenocortical carcinoma (ACC) is a rare but aggressive malignancy of the adrenal cortex ( Favia et al . 1995 , Wajchenberg et al . 2000 , Icard et al . 2001 , Bilimoria et al . 2008 ). Prognosis is poor in patients with locally

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Constanze Hantel, Sara Jung, Thomas Mussack, Martin Reincke, and Felix Beuschlein

Introduction Adrenocortical carcinoma (ACC) represents a rare but highly malignant tumor entity. In a large proportion of cases, the high growth rate of this endocrine neoplasm results in late diagnosis not amendable to surgical resection. However

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Peter M van Koetsveld, Giovanni Vitale, Richard A Feelders, Marlijn Waaijers, Diana M Sprij-Mooij, Ronald R de Krijger, Ernst-Jan M Speel, Johannes Hofland, Steven W J Lamberts, Wouter W de Herder, and Leo J Hofland

Introduction Adrenocortical carcinoma (ACC) is a rare but highly malignant endocrine tumor. Surgery is the primary choice of treatment but recurrence rates after surgery are high ( Schteingart et al . 2005 , Fassnacht et al . 2011 ). In case of

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Sara Jung, Zoltan Nagy, Martin Fassnacht, Gerard Zambetti, Max Weiss, Martin Reincke, Peter Igaz, Felix Beuschlein, and Constanze Hantel

Introduction In recent years, genetic and molecular profiling of surgical tumor specimens has led to the identification of novel biomarkers with prognostic relevance for patients with adrenocortical carcinoma (ACC) ( Assie et al . 2014

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Katja Kiseljak-Vassiliades, Yu Zhang, Stacey M Bagby, Adwitiya Kar, Nikita Pozdeyev, Mei Xu, Katherine Gowan, Vibha Sharma, Christopher D Raeburn, Maria Albuja-Cruz, Kenneth L Jones, Lauren Fishbein, Rebecca E Schweppe, Hilary Somerset, Todd M Pitts, Stephen Leong, and Margaret E Wierman

incidental adrenal adenomas are common, occurring in up to 4–7% of patients, adrenocortical carcinoma (ACC) is an uncommon, aggressive cancer occurring in 0.7–2 per million people ( Allolio & Fassnacht 2006 , Else 2014 ). Adrenocortical carcinoma has a

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Nikita Pozdeyev, Lauren Fishbein, Laurie M Gay, Ethan S Sokol, Ryan Hartmaier, Jeffrey S Ross, Sourat Darabi, Michael J Demeure, Adwitiya Kar, Lindsey J Foust, Katrina Koc, Daniel W Bowles, Stephen Leong, Margaret E Wierman, and Katja Kiseljak-Vassiliades

Introduction Adrenocortical carcinoma (ACC) is an orphan malignancy affecting individuals across a broad age spectrum with bimodal distribution from pediatric to adults. The rarity of the disease, limited preclinical models and lack of