Adrenocortical tumours in children: a review of surgical management at a tertiary care centre

Background Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. Methods Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. Results The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I–III disease who had total excision without spillage (n = 17) are disease‐free for 2–170 months. Conclusions Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases. Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. Complete surgical excision is the only possible treatment of ACT and R0 resection is the treatment of choice if possible. Multicentre prospective studies with larger patient series are necessary to expand the knowledge about ACT of children and develop alternative therapies for stages III and IV adrenocortical carcionma.