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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Maria Riedmeier, Lester D R Thompson, Carlos Augusto Fernandes Molina, Boris Decarolis, Christoph Härtel, Paul-G Schlegel, Martin Fassnacht, and Verena Wiegering
Sara Jung, Zoltan Nagy, Martin Fassnacht, Gerard Zambetti, Max Weiss, Martin Reincke, Peter Igaz, Felix Beuschlein, and Constanze Hantel
monotherapy using different dosing regimens and higher drug concentration. In addition, topotecan was identified as a potentially effective agent for pediatric ACC in this study. As outlined previously, liposomal cisplatin is already investigated in clinical
J Crona, F Beuschlein, K Pacak, and B Skogseid
). Eighty-two patients with high-risk pediatric ACC were evaluated for outcome and prognostic factors: distant metastases and large tumor volume were associated with unfavorable prognosis ( Cecchetto et al . 2017 ). Improved classification and
Rossella Libè, Amato Fratticci, and Jérôme Bertherat
( Bell et al. 1999 ). Germline mutations in TP53 have been observed in 50–80% of children with apparently sporadic ACC in North America and Europe ( Wagner et al. 1994 , Varley et al. 1999 ). The incidence of pediatric ACC is about 10 times
Bruno Ragazzon, Guillaume Assié, and Jérôme Bertherat
have been identified ( Caron de Fromentel & Soussi 1992 ). In North America and Europe, 50–80% of children with sporadic ACC have a germline mutation of TP53 ( Wagner et al . 1994 , Varley et al . 1999 ). In Southern Brazil, pediatric ACCs are ten
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
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
alterations, detected in the cohort, were found within the subset of 12 pediatric cases. It is known that 50–80% of pediatric ACC are associated with Li Fraumeni syndrome caused by germline TP53 alterations ( Wagner et al. 1994 , Rodriguez-Galindo et al
Fidéline Bonnet-Serrano and Jérôme Bertherat
pediatric ACC is remarkably high (10–15 times the world-wide occurrence) in Southern Brazil because of the segregation of the TP53 hotspot mutation p.R337H. In this region, 90% of pediatric ACC cases harbor this mutation. A free screening of 171
S G Creemers, L J Hofland, E Korpershoek, G J H Franssen, F J van Kemenade, W W de Herder, and R A Feelders
found in pediatric ACC ( Figueiredo et al . 1999 , James et al . 1999 , Pianovski et al . 2006 ), in which it has also been suggested to be involved in tumorigenesis based on mRNA overexpression and strong SF1 staining ( Figueiredo et al . 2000
