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Maria Riedmeier, Lester D R Thompson, Carlos Augusto Fernandes Molina, Boris Decarolis, Christoph Härtel, Paul-G Schlegel, Martin Fassnacht, and Verena Wiegering

systems for pediatric ACC: Wieneke score (AFIP) and Weiss score. AFIP score (Wieneke) Weiss score Criteria Tumor weight > 400 g High nuclear grade (Führman G3/G4) Tumor size > 10.5 cm >5 mitosis/50 HPF

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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

( Rainey et al . 2004 ). Other putative ACC cell lines have not gained use due to the uncertainty of the source or lack of availability ( Wang & Rainey 2012 ). Pinto and coworkers reported a pediatric ACC PDX (SJ-ACC3) but were unable to derive a

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P S H Soon, A J Gill, D E Benn, A Clarkson, B G Robinson, K L McDonald, and S B Sidhu

pediatric tumors ( West et al . 2007 ). Across all these studies including in our study, the only consistent finding has been the overexpression of IGF2 in ACCs compared with ACAs. We selected nine genes from our microarray analysis for further study – five

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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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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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Maria Cristina De Martino, Richard A Feelders, Wouter W de Herder, Peter M van Koetsveld, Fadime Dogan, Joseph A M J L Janssen, A Marlijn Waaijers, Claudia Pivonello, Steven W J Lamberts, Annamaria Colao, Ronald R de Krijger, Rosario Pivonello, and Leo J Hofland

Introduction Adrenocortical cancer (ACC) is a rare and aggressive cancer with a 5-year survival at the metastatic stage below 15% ( Bilimoria et al . 2008 , Baudin et al . 2011 , Fassnacht et al . 2011 ). The insulin-like growth factor (IGF

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Bruno Ragazzon, Guillaume Assié, and Jérôme Bertherat

adrenals. ACC, adrenocortical carcinoma; ACA, adrenocortical adenoma. a Study compares expression profiles of pediatric tumors. b Meta-analysis. Molecular landmarks of malignancy in ACTs The malignancy signature is the strongest transcriptome feature of

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Enzo Lalli and Michaela Luconi

characterizes pediatric ACT, but with no association with malignancy ( West et al. 2007 , Rosati et al. 2008 ). Further molecular studies showed that gene expression profiles can stratify histologically confirmed adult ACC in 2 subgroups with very different

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S G Creemers, R A Feelders, N Valdes, C L Ronchi, M Volante, B M van Hemel, M Luconi, M H T Ettaieb, M Mannelli, M D Chiara, M Fassnacht, M Papotti, M N Kerstens, G Nesi, H R Haak, F J van Kemenade, and L J Hofland

2010 ). Only in rare cases, the adrenal tumor has malignant potential. Adrenocortical carcinoma (ACC) is a highly malignant tumor with 5-year-survival ranging from 16 to 38% ( Kebebew et al. 2006 , Fassnacht et al. 2013 ). The Weiss score (WS

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Tiantian Liu, Taylor C Brown, C Christofer Juhlin, Adam Andreasson, Na Wang, Martin Bäckdahl, James M Healy, Manju L Prasad, Reju Korah, Tobias Carling, Dawei Xu, and Catharina Larsson

pheochromocytoma (PCC) and an adrenocortical carcinoma (ACC). Pheochromocytomas (PCC) and abdominal paragangliomas (PGL) are typically catecholamine-producing tumors, originating from chromaffin cells in the paraganglia inside or outside the adrenal medulla ( Elder