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Treatment of Pediatric Adrenocortical Carcinoma With Surgery, Retroperitoneal Lymph Node Dissection, and Chemotherapy: The Children's Oncology Group ARAR0332 Protocol.

Carlos Rodriguez GalindoMark D KrailoEmilia Modolo PintoFarzana D PashankarChristopher B WeldonLi HuangEliana M CaranJohn HicksElizabeth B McCarvilleDavid MalkinJonathan D WassermanAntonio Gonçalves DE-Oliveira FilhoMichael P LaQuagliaDeborah A WardGerard ZambettiMaria J MastellaroAlberto S PappoRaul C Ribeiro
Published in: Journal of clinical oncology : official journal of the American Society of Clinical Oncology (2021)
Outcome for children with stage I ACC is excellent with surgery. Outcome for patients with stage II disease is inferior despite retroperitoneal lymph node dissection. Patients with stage III ACC have an excellent outcome combining surgery and chemotherapy. Patients with stage IV ACC are older and have a poor outcome; new treatments should be explored for this high-risk group. The combination of mitotane and chemotherapy as prescribed in ARAR0332 resulted in significant toxicity; one third of patients with advanced disease could not complete the scheduled treatment.
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