overestimation of biological aggressiveness have been frequently reported for pediatric patients ( Wieneke et al. 2003 , Chatterjee et al. 2015 , Gupta et al. 2018 , Jehangir et al. 2019 ). Table 1 Criteria and classification of the two scoring
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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
Ana Carolina Bueno, Rui M P da Silva, Mônica F Stecchini, Junier Marrero-Gutiérrez, Danillo C de Almeida e Silva, Izilda Cardinalli, Carlos Alberto Scrideli, Thais Junqueira, Carlos A F Molina, Fernando Silva Ramalho, Silvio Tucci Jr, Fernanda Borchers Coeli-Lacchini, Ayrton Custodio Moreira, Leandra N Z Ramalho, Silvia Brandalise, José Andres Yunes, Margaret de Castro, Ricardo Zorzetto Nicoliello Vêncio, and Sonir R R Antonini
functional tumors and signs of excessive hormone secretion, mainly virilization ( Michalkiewicz et al. 2004 ). Most pACT are classified as adrenocortical carcinomas (ACC) based on histopathological findings ( Wieneke et al. 2003 , Michalkiewicz et al
Michaela Kuhlen, Pascal Mier, Marina Kunstreich, Lienhard Lessel, Dominik Schneider, Ines Brecht, Denis M Schewe, Michael C Frühwald, Peter Vorwerk, and Antje Redlich
features of the tumour to one of three scores of the Wieneke index ( Wieneke et al. 2003 ). Complete remission (CR) was achieved if patients had normalized hormone profiles and no structural evidence of disease. Partial remission (PR) was defined as a
Enzo Lalli and Michaela Luconi
Papotti et al. (2011) ), in children ( Wieneke et al . 2003 ) and in histological subtypes of ACT as the oncocytic, myxoid and sarcomatoid tumors ( Papotti et al . 2011 ). For these reasons, a new scoring system (Helsinki score) was recently introduced
