ESPE Abstracts

Purpose: Adrenal neoplasms (ANs) are rare endocrine neoplasms in children, and etiopathogenesis and prognosis of pediatric ANs remain obscure. This study investigated clinical features, histopathological features, genetic etiologies and prognosis of ANs.

Methods: This study included 33 ANs patients diagnosed from March 1997 to April 2021. Clinical features and endocrine findings were collected by retrospective medical chart review. The histopathological features were assessed using the Weiss scoring system. Molecular analysis was performed by Sanger sequencing according to the patients’ phenotype.

Results: Among 33 patients, adrenocortical carcinoma (n = 9, 27.3%) and pheochromocytoma (n = 9, 27.3%) were the most common, followed by adrenal adenoma (n = 5, 15.2%) and ganglioneuroma (n = 3, 9.1%). The median age at diagnosis was 7.8 years (range, 0.8–17.8 years). There was no metastasis at diagnosis, however, ANs recurred in 6 patients. All patients survived during the follow-up period. The most common presenting symptom was precocious adrenarche (n = 13, 39.4%), followed by hypertension (n = 11, 33.3%) in all patients with pheochromocytoma, adrenal incidentaloma (n = 6, 18.2%), and Cushing syndrome (n = 4, 12.1%). Molecular etiologies were identified in 12 patients: Li-Fraumeni syndrome (n = 5), 21-hydroxylase deficiency (n = 3), and von Hippel-Lindau syndrome (n = 4). The mean Weiss score was 1.5 ± 1.6. Weiss scores was not significantly different between adrenocortical carcinoma and pheochromocytoma (2.6 ± 1.9 vs. 1.1 ± 1.2, P=0.094), however, the score of ganglioneuroma was higher than that of phenochromocytoma (3.5 ± 0.7 vs. 1.1 ± 1.2, P=0.036). Weiss score of Li-Fraumeni syndrome was significantly higher than the other ANs (3.2 ± 1.6 vs. 1.1 ± 1.4, P=0.013). The maximum diameter of tumor was 5.3 ± 1.9 cm, which showed no significant correlation with the Weiss score (r = 0.141, P=0.464).

Conclusions: In this study, adrenal carcinoma and pheochromocytoma were most frequent in children and adolescents with ANs. In patients with genetic etiologies, tumor screening and for other organs and genetic counseling are necessary.