Refractory and relapsed paediatric ACC in the MET studies – A challenging situation necessitating novel diagnostic and therapeutic concepts

Michaela Kuhlen,Marina Kunstreich,Lienhard Lessel,Stefan A. Wudy,Paul-Martin Holterhus,Christian Vokuhl,Eva Juettner,Christoph Roecken,Guido Seitz,Christoph Slavetinsky,Jörg Fuchs,Denis M. Schewe,Rainer Claus,Pascal D. Johann,Michael C. Frühwald,Peter Vorwerk,Antje Redlich

EJC Paediatric Oncology（2023）

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摘要

•Refractory ACC has a dismal prognosis with a 3-year OS and PFS of 0%.•Outcome of relapsed ACC depends on site of relapse and resectability.•OS after event is 28.6% in local, 59.6% in distant and 14.3% in combined relapse.•Only 4 of 17 relapsed patients with initial stage II were rescued.•Novel diagnostic and therapeutic concepts are urgently needed to improve outcomes.

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关键词

Adrenocortical carcinoma,Relapsed,Refractory,Children,Outcome

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