Therapeutic options of advanced pediatric adrenocortical carcinoma (pACC) are limited, and achieving valuable risk stratification remains challenging. We refined the value of prognostic factors with an emphasis on resection status. Retrospective international data from 106 patients with advanced pACC from various collaborating centers of the international pACC working groups ENSAT-PACT, IC-PACT and/or from individual international collaboration diagnosed were collected. One hundred six patients aged 0.1–18.1 (median 7.6) years were diagnosed with pACC, with 42 tumor stage III and 64 stage IV, respectively. Eighty percent (85/106) of the tumors were hormone-producing, with a mean Ki67 index for both stage groups of 29%. Patient survival was 45% (48/106) with a mean follow-up of 17.7 months. Higher age, tumor stage IV and increased Ki67 index worsened the prognosis on overall survival. Resection status had an essential impact on survival, as the patients with R0 resection (n = 32) had a better overall survival (71% for stage III patients; 80% for stage IV patients) than patients with R1 (n = 24) (45% for stage III; 69% for stage IV), R2 (n = 33) (17% for stage III; 15% for stage IV) and Rx (n = 7) (0% for stage III; 17% for stage IV). Of the ten patients with tumor spillage, only a few (57% of stage III; 0% of stage IV patients) survived. The resection status has a significant impact on overall survival in pACC. Therefore, tumor surgery should only be undertaken by experienced surgeons proficient in adrenalectomy and oncology, ideally within specialized pediatric oncological centers with a multidisciplinary team setting.
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