Adrenocortical carcinoma (ACC) is a rare but aggressive malignancy in children. A recent COG study, ARAR0332, provided the largest and most meaningful trial to date. However, several questions remain. First, surgery was recommended for all stages but the impact of surgery in the setting of metastatic disease is still unclear. Additionally, retroperitoneal lymph node dissection (RPLND) was recommended for all patients with stage two and higher but had no impact on survival; however, RPLND was likely not adequately performed in all patients. Third, the oncologic impact of minimally invasive surgery for ACCs is still debated. This study will investigate contemporary surgical approaches to ACC. We aim to:
Investigate the impact of surgery on oncologic outcomes in metastatic disease.
Define the utility of minimally invasive approaches to patients with ACC.
Define the incidence of compliance with RPLND and impact on oncologic outcomes.
Study PI: Sara Mansfield, MD, MS
Contact Chloé Boehmer (Chloe.Boehmer@cchmc.org) if you have any questions on how your site can participate in this study.