The feasibility of adrenal-sparing surgery in bilateral adrenal neuroblastoma.

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 center'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 preoperative 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 preoperative 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).