The feasibility of adrenal-sparing surgery in bilateral adrenal Neuroblastoma

Abstract Purpose Loss of adrenal function is a major concern in the treatment of children with bilateral adrenal neuroblastoma (BAN). We aimed to evaluate the feasibility of adrenal-sparing surgery in this unique subgroup. Method Retrospective review of our centre's neuroblastoma database was conducted. Patients with synchronous BAN confirmed at surgery were included. Their demographic data and clinical charts were analyzed. Results Five patients were confirmed with BAN. Mean age at diagnosis was 0.89 (0.39–1.32) years, male:female ratio was 4:1. They were stratified as stages M(n = 2), MS(n = 1), L1(n = 1) and L2(n = 1). MYCN amplification was present in 1 patient (stage M) and was stratified as high-risk group. All patients except one received pre-operative chemotherapy. Among the 10 adrenal gland tumors, 2 underwent tumor adrenalectomy (TA) and 8 had adrenal-sparing tumorectomy (AST). After chemotherapy, 3 patients underwent single-stage bilateral AST and 1 patient underwent 2-stage TA-AST. One patient underwent upfront single-stage TA-AST, where lack of pre-operative chemotherapy rendered the adrenal gland indistinguishable. The high-risk patient received autologous hematopoietic cell transplantation for consolidation. No patients required adrenal replacement therapy. All patients were alive without evidence of disease with mean follow-up 5.5 (2.6–8.5) years. Conclusion Adrenal-sparing surgery is feasible in patients with BAN. Level-of-evidence Level IV (case series with no comparison group).