Endocrine Abstracts

Adrenal tumors (ATs) are uncommon in children and adolescents. The aim of this nation-wide retrospective registry-based cohort study was to characterise the various ATs in this population and demonstrate their differences from ATs in adults. All patients under the age of 21 in Sweden with verified adrenal lesions according to ICD-codes between 2005 and 2019 were identified in linked nation-wide registries. Out of the total 232 patients, 121 (52.2%) were boys, with boy predominance preserved even in benign ATs. Mean age was 8.7±7.9 years. Malignant ATs were found in 57% of patients, while 34% of patients had benign ATs with mean age at diagnosis of 4.4 and 14.5 years respectively. The most prevalent diagnosis was neuroblastoma (37.0%), followed by benign adenoma (29.7%) – of which 10% hormonally active, pheochromocytoma (8.6%) and adrenocortical carcinoma (7.3%). Hormone related prodromal symptoms were detected in 16% of patients. Endocrine commorbidities were observed in 46.6% of cases with the most common being hypertension (28%) and adrenal insufficiency (17.7%). In contrast to adults, 13 out of 20 cohort patients with pheochromocytoma had a concurent genetic syndrome and 9 out of 20 had another concomitant tumor, 7 of which were endocrine tumors. Patients were primarily treated and operated at tertiary hospital in 72.4% and 98.9% of cases respectively. 47.8% of individuals received chemotherapy and 42.2% were operated. The most common post-operative complication was adrenal insufficiency (23.5%), and was more prevalent in adrenocortical carcinomas (56.3%) and hormone-producing benign adenomas (57.1%). Unexpectedly 40% of patients with pheochromocytoma and 11.1% of benign adenomas without overt hormonal production were in need of corticosteroid replacement post-operatively. Mortality rate was 19,4%, rising to 33.3% in patients with malignant ATs and 100% within 2 years for the 5 patients with adrenal metastasis. In conclusion, ATs were found to be rare in children and adolescents in Sweden. Hormone producing or malignant ATs were the most common diagnoses with the latter having a poor prognosis.