WOFAPS 2025 8th World Congress of Pediatric Surgery

PRIYA MATHEW, Sajid Qureshi

TATA MEMORIAL HOSPITAL, MUMBAI, INDIA

PURPOSE:

Adrenocortical tumours (ACTs) are rare in childhood, representing 0.2% of all paediatric tumours with an incidence of 0.2 new cases per 1 million children per year. Distinction between large benign adenoma (ACA) and adrenocortical carcinoma (ACC) may be difficult, and optimal treatment tricky. Complete surgical resection provides the best chance of cure, and in case of ACC is a prerequisite of cure. The aim of this study is to review our experience in the management and outcome of ACC at our institute.

METHODS:

Retrospective analysis of 17 patients with ACT, who underwent treatment, from January 2012 to June 2025 at our centre was done. Collected data included clinical details, chemotherapy, operative details, histopathology, complications, and outcomes, etc.

RESULTS:
Total 17 patients (09 females) were included. 14 patients had Cushing's syndrome (CS), 03 patients had virilizing tumours, 03 had systemic hypertension. Histopathological diagnoses were ACC in all patients of which 9 were left sided. Median age was 24 months. One received neoadjuvant chemotherapy. Tp53 germline mutations were found in 4 patients. Surgeries performed include Right adrenalectomy (06), Left adrenalectomy (06), Left adrenalectomy with left nephrectomy (01), Left adrenalectomy & left nephrectomy with IVC thrombectomy (01), Left adrenalectomy with IVC thrombectomy (01), Right adrenalectomy with right nephrectomy (01), Left adrenalectomy with IVC thrombectomy (01). Postoperative complications included postoperative ileus (03), Surgical site infection (02). Three were lost to follow up, two had remote (01 Liver, 01 Lung) metastasis. Rest other patients are under follow-up without local or remote recurrence with no sequelae of chemotherapy till date. Follow-up ranged from 12 - 155 months.

CONCLUSION:
Paediatric ACT are rare. Little information is available on the incidence, risk factors, prognostic factors, treatment, and overall survival. Therapeutic approaches are typically multimodal. Careful evaluation, multidisciplinary team approach, individualized surgical planning, appropriate chemotherapy are necessary for better outcome