Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study

Background: Pediatric adrenocortical carcinoma (ACC) is a rare and aggressive cancer. The appropriateness of minimally invasive surgery (MIS), role of nodal dissection, and aggressiveness of surgery in patients with metastatic disease remain poorly understood. Patients And Methods: We performed a retrospective review of patients < 18 years with ACC treated from 2012 to 2022 at 22 institutions participating in the Pediatric Surgical Oncology Research Collaborative. Data collected included demographics, clinical presentation, genetic predisposition, tumor characteristics, surgical approach, adjuvant treatment, and survival outcome. Survival probabilities were estimated with Kaplan-Meier methods. Results: In all, 69 patients were included (median age: 8.4 years; 58% female), and 28% had Li-Fraumeni syndrome. At diagnosis, 36% had Stage IV disease and 28% had Stage I disease. A total of 55 (83%) patients underwent open adrenalectomy, and 11 (17%) underwent MIS. MIS approaches were employed for significantly smaller tumors (median: 4.4 cm, 38 g) compared with open cases (median: 9.6 cm, 246 g; p < 0.001), and none had tumor spillage (versus 22% in open cases; p = 0.087). Lymph node sampling was performed in 44% of open cases (mean yield: 2.96); no lymph nodes were sampled in MIS cases (p = 0.008). The 5 year overall survival (OS) for the entire cohort was 65.9%, and 5-year event-free survival (EFS) was 54.1%. Patients without metastases had significantly better survival than those