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The feasibility of adrenal-sparing surgery in bilateral adrenal neuroblastoma
Chan Hon Chui1, Kenneth Tou En Chang2
1Surgery Centre for Children, Mount Elizabeth Medical Centre, Singapore.
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).
Insights
Adrenal-sparing surgery is feasible for children with bilateral adrenal neuroblastoma (BAN), preserving adrenal function. This approach avoids the need for adrenal replacement therapy in most cases.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Pediatric Surgery
Background:
- Bilateral adrenal neuroblastoma (BAN) poses a significant risk of adrenal insufficiency in pediatric patients.
- Preserving adrenal function is critical for long-term health and development in affected children.
Purpose of the Study:
- To evaluate the feasibility and outcomes of adrenal-sparing surgery in children diagnosed with BAN.
- To determine if surgical techniques can preserve adrenal function in this specific patient subgroup.
Main Methods:
- Retrospective review of a neuroblastoma database.
- Inclusion of patients with synchronous BAN confirmed surgically.
- Analysis of demographic data, clinical charts, and surgical outcomes.
Main Results:
- Five patients with BAN were identified, with a mean age of 0.89 years.
- Eight out of ten adrenal tumors underwent adrenal-sparing tumorectomy (AST); two underwent tumor adrenalectomy (TA).
- No patients required adrenal replacement therapy, and all were alive with no evidence of disease at a mean follow-up of 5.5 years.
Conclusions:
- Adrenal-sparing surgery is a feasible and effective approach for managing BAN.
- This surgical strategy can successfully preserve adrenal function in pediatric patients with BAN.
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