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

Rodriguez-Galindo C, Krailo MD, Pinto EM, Pashankar F, Weldon CB, Huang L, Caran EM, Hicks J, McCarville MB, Malkin D, Wasserman JD, de Oliveira Filho AG, LaQuaglia MP, Ward DA, Zambetti G, Mastellaro MJ, Pappo AS, Ribeiro RC. Treatment of Pediatric Adrenocortical Carcinoma With Surgery, Retroperitoneal Lymph Node Dissection, and Chemotherapy: The Children’s Oncology Group ARAR0332 Protocol. J Clin Oncol. 2021 Aug 1;39(22):2463-2473. doi: 10.1200/JCO.20.02871. Epub 2021 Apr 6. PubMed PMID: 33822640; PubMed Central PMCID: PMC8462560.

Adrenocortical carcinoma (ACC) is a rare aggressive pediatric malignancy with distinct biology. Its treatment follows the principles developed for adults; pediatric-specific studies are scarce. Prospective single-arm risk-stratified interventional study. Study objectives were (1) to describe the outcome of patients with stage I ACC treated with adrenalectomy alone; (2) to describe the outcome of stage II patients (completely resected > 200 cc or > 100 g) treated with adrenalectomy and retroperitoneal lymph node dissection; and (3) to describe the outcome of patients with stage III or IV treated with mitotane and chemotherapy.