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Milena Doroszko Institute of Biomedicine, University of Turku, Turku, Finland

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Marcin Chrusciel Institute of Biomedicine, University of Turku, Turku, Finland

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Joanna Stelmaszewska Department of Reproduction and Gynecological Endocrinology, Medical University of Bialystok, Bialystok, Poland

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Tomasz Slezak Department of Biochemistry and Molecular Biology, University of Chicago, Chicago, Illinois, USA

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Slawomir Anisimowicz Center of Gynecology and Reproductive Endocrinology Artemida, Bialystok, Poland

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Ursula Plöckinger Interdisciplinary Center of Metabolism: Endocrinology, Diabetes and Metabolism, Charité University Medicine Berlin, Berlin, Germany

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Marcus Quinkler Endocrinology in Charlottenburg, Berlin, Germany
Department of Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Berlin, Germany

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Marco Bonomi Department of Clinical Sciences & Community Health, University of Milan, Milan, Italy

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Slawomir Wolczynski Department of Reproduction and Gynecological Endocrinology, Medical University of Bialystok, Bialystok, Poland

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Ilpo Huhtaniemi Institute of Biomedicine, University of Turku, Turku, Finland
Department of Surgery and Cancer, Faculty of Medicine, Imperial College London, London, U.K.

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Jorma Toppari Institute of Biomedicine, University of Turku, Turku, Finland
Department of Pediatrics, Turku University Hospital, Turku, Finland

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Nafis A Rahman Institute of Biomedicine, University of Turku, Turku, Finland
Department of Reproduction and Gynecological Endocrinology, Medical University of Bialystok, Bialystok, Poland

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, Chrusciel et al. 2014 ). Intact inhα/Tag mice develop gonadal tumors, but when prepubertally gonadectomized, adrenocortical tumors appear with a hyperplasia-adenoma-adenocarcinoma sequence and abundant LHCGR expression ( Kananen et al. 1995 , 1996

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Ana Carolina Bueno Department of Pediatrics Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Rui M P da Silva Department of Internal Medicine Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Mônica F Stecchini Department of Pediatrics Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Junier Marrero-Gutiérrez Department of Pediatrics Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Danillo C de Almeida e Silva Department of Internal Medicine Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil
Department of Computation and Mathematics, Faculty of Philosophy, Sciences and Letters at Ribeirao Preto, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Izilda Cardinalli Boldrini Children’s Center, State University of Campinas, Campinas, Sao Paulo, Brazil

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Carlos Alberto Scrideli Department of Pediatrics Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Thais Junqueira Boldrini Children’s Center, State University of Campinas, Campinas, Sao Paulo, Brazil

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Carlos A F Molina Department of Surgery and Anatomy Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Fernando Silva Ramalho Department of Pathology Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Silvio Tucci Jr Department of Surgery and Anatomy Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Fernanda Borchers Coeli-Lacchini Department of Internal Medicine Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Ayrton Custodio Moreira Department of Internal Medicine Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Leandra N Z Ramalho Department of Pathology Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Silvia Brandalise Boldrini Children’s Center, State University of Campinas, Campinas, Sao Paulo, Brazil

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José Andres Yunes Boldrini Children’s Center, State University of Campinas, Campinas, Sao Paulo, Brazil

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Margaret de Castro Department of Internal Medicine Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Ricardo Zorzetto Nicoliello Vêncio Department of Computation and Mathematics, Faculty of Philosophy, Sciences and Letters at Ribeirao Preto, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Sonir R R Antonini Department of Pediatrics Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil

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Introduction Pediatric adrenocortical tumors (pACT) are rare and represent about 0.2% of all childhood cancers ( Gulack et al. 2016 ). pACT present distinct clinical and prognostic features from the disease in adults, usually presenting with

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Patricia de Cremoux Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Dan Rosenberg Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Jacques Goussard Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Catherine Brémont-Weil Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Frédérique Tissier Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Carine Tran-Perennou Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Lionnel Groussin Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Xavier Bertagna Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Jérôme Bertherat Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Marie-Laure Raffin-Sanson Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France
Unité de Pharmacologie, Ecole Nationale Vétérinaire d'Alfort, Laboratoire d'Oncobiologie, Department of Pathology, Department of Endocrinology, Department of Endocrinology, Faculté de Médecine Paris-Ile de France Ouest, INSERM, Département d'Endocrinologie Métabolisme and Cancer, CNRS, Faculté de Médecine René Descartes, Institut Curie, 26 rue d'Ulm, 75005 Paris, France

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Introduction Unilateral tumors account for most cases of adrenocorticotrophin (ACTH)-independent hypercortisolism and may be classified into benign adrenal adenomas (ACAs) and malignant adrenocortical carcinomas (ACCs). ACCs have an extremely poor

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Zsófia Tömböl
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Peter M Szabó
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Viktor Molnár Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Zoltán Wiener Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Gergely Tölgyesi Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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János Horányi Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Peter Riesz Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Peter Reismann
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Attila Patócs Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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István Likó Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Rolf-Christian Gaillard Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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András Falus Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary
Second Department of Medicine, Department of Genetics, First Department of Surgery, Department of Urology, Molecular Medicine Research Group, Gedeon Richter Ltd, Division of Endocrinology, Research Group of Inflammation Biology and Immunogenomics, Faculty of Medicine, Semmelweis University, Szentkirályi Street 46, H-1088 Budapest, Hungary

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Károly Rácz
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Peter Igaz
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Introduction Sporadic adrenocortical tumors are common, and their prevalence reaches up to 7–9% in pathological series ( Koch et al . 2002 ). Most of these are incidentally discovered, hormonally inactive benign tumors, whereas rare hormone

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Barbara Mariniello
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Antonio Rosato Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy
Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy

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Gaia Zuccolotto Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy

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Beatrice Rubin
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Maria Verena Cicala
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Isabella Finco
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Maurizio Iacobone Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy

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Anna Chiara Frigo Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy

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Ambrogio Fassina Endocrinology Unit, Department of Surgery, Istituto Oncologico Veneto IRCCS, Endocrine Surgery Unit, Department of Environmental Medicine and Public Health, Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, Via Ospedale Civile 105, 35128 Padua, Italy

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Raffaele Pezzani
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Franco Mantero
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Introduction Adrenocortical masses are common tumors, with a prevalence of at least 3% in the population over the age of 50 years; they are incidentally discovered up to 5% on abdominal imaging and 15% of patients at autopsy ( Grumbach et al . 2003

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Régia Caroline Peixoto Lira Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Paola Fernanda Fedatto Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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David Santos Marco Antonio Department of Research and Development, Fleury Group, Sao Paulo, São Paulo, Brazil

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Letícia Ferro Leal Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Carlos Eduardo Martinelli Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Margaret de Castro Department of Internal Medicine, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Silvio Tucci Department of Surgery, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Luciano Neder Department of Pathology, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Leandra Ramalho Department of Pathology, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Ana Luiza Seidinger Boldrini Children Center, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil

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Izilda Cardinalli Boldrini Children Center, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil

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Maria José Mastellaro Boldrini Children Center, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil

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José Andres Yunes Boldrini Children Center, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil
State University of Campinas (UNICAMP), CampinasSão Paulo, Brazil

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Silvia Regina Brandalise Boldrini Children Center, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil
State University of Campinas (UNICAMP), CampinasSão Paulo, Brazil

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Luiz Gonzaga Tone Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Sonir Roberto Rauber Antonini Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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Carlos Alberto Scrideli Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, São Paulo, Brazil

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clinical and pathological features of adrenocortical tumors in a large series of pediatric patients’ samples and investigated the effect of IGF1R inhibition by OSI-906 in the ACC cell line NCI-H295A as well as the altered downstream signaling ­pathways

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Susanna Vuorenoja Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland
Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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Adolfo Rivero-Müller Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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Adam J Ziecik Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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Ilpo Huhtaniemi Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland
Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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Jorma Toppari Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland
Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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Nafis A Rahman Department of Physiology, Department of Pediatrics, Institute of Animal Reproduction and Food Research, Department of Reproductive Biology, University of Turku, Kiinamyllynkatu 10, 20520 Turku, Finland

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-castration elevation of LH levels apparently induced the ectopic LHR expression, which together with the potent oncogene Tag expression triggered adrenocortical tumorigenesis ( Mikola et al . 2003 ). LH dependence of the tumors was proven by findings that they failed

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Maria Cristina De Martino
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Richard A Feelders
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Wouter W de Herder
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Peter M van Koetsveld
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Fadime Dogan
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Joseph A M J L Janssen
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A Marlijn Waaijers
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Claudia Pivonello Department of Internal Medicine Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

Dipartimento di Medicina Clinica e Chirurgia Sezione di Endocrinologia, Università Federico II, Naples, Italy

Department of Pathology Erasmus Medical Center, Rotterdam and Reinier de Graaf Gasthuis, Delft, The Netherlands

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Steven W J Lamberts
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Annamaria Colao Department of Internal Medicine Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

Dipartimento di Medicina Clinica e Chirurgia Sezione di Endocrinologia, Università Federico II, Naples, Italy

Department of Pathology Erasmus Medical Center, Rotterdam and Reinier de Graaf Gasthuis, Delft, The Netherlands

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Ronald R de Krijger Department of Internal Medicine Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

Dipartimento di Medicina Clinica e Chirurgia Sezione di Endocrinologia, Università Federico II, Naples, Italy

Department of Pathology Erasmus Medical Center, Rotterdam and Reinier de Graaf Gasthuis, Delft, The Netherlands

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Rosario Pivonello Department of Internal Medicine Division of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

Dipartimento di Medicina Clinica e Chirurgia Sezione di Endocrinologia, Università Federico II, Naples, Italy

Department of Pathology Erasmus Medical Center, Rotterdam and Reinier de Graaf Gasthuis, Delft, The Netherlands

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Leo J Hofland
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negative, 2–3 as low, 4–5 as intermediate, and 6 as high. Finally, adrenocortical tumors (ATs) were dichotomously grouped as having intermediate to high expression of the evaluated protein and phosphoproteins (IHC score ≥4) or not (IHC score <4). In AHs and

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P S H Soon Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia
Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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A J Gill Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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D E Benn Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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A Clarkson Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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B G Robinson Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia
Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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K L McDonald Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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S B Sidhu Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia
Cancer Genetics, Department of Surgery, Anatomical Pathology, Endocrinology, Endocrine and Oncology Surgery, Kolling Institute of Medical Research, Royal North Shore Hospital, University of Sydney, St Leonards, Sydney, New South Wales 2065, Australia

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) can be difficult. The Weiss score, a nine-point histopathological scoring system, is presently the most widely used system for classifying adrenocortical tumors (ACTs) as benign or malignant. A total score of ≤2 is typically associated with a benign

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Susanna Vuorenoja Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland
Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Bidut Prava Mohanty Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Johanna Arola Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Ilpo Huhtaniemi Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland
Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Jorma Toppari Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland
Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Nafis A Rahman Departments of, Physiology, Pediatrics, Department of Pathology, Institute of Reproductive and Developmental Biology, University of Turku, Kiinamyllynkatu 10, FIN-20520 Turku, Finland

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Introduction Adrenocortical tumors are rare and aggressive as they often are diagnosed late and have poor survival rate ( Schulick & Brennan 1999 a ). These tumors are 1.5-fold more common in females than males with peak incidences in the first and

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