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Exp Clin Endocrinol Diabetes 2023; 131(10): 515-522
DOI: 10.1055/a-2127-9292
Article

Clinical Characteristics and Long-Term Outcomes of Adrenal Tumors in Children and Adolescents

Ja Hye Kim
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Yunha Choi
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Soojin Hwang
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Ji-Hee Yoon
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Gu-Hwan Kim
2   Medical Genetics Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Han-Wook Yoo
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
,
Jin-Ho Choi
1   Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
› Author Affiliations Fundings This research was supported by the National Research Foundation of Korea (NRF) grant funded by the Korean government (Ministry of Science and ICT) (No. NRF2021R1F1A104593011). National Research Foundation of Korea (NRF) — NRF2021R1F1A104593011
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Abstract

Objective Adrenal tumors are generally rare in children and can be a part of familial cancer syndrome. This research was conducted to examine the clinical outcomes, histopathological results, and genetic etiologies of adrenal tumors in children and adolescents.

Methods Thirty-one children and adolescents with adrenal tumors were included. Data on clinical outcomes and endocrine and radiologic results were retrospectively analyzed. Molecular analysis was conducted in select patients according to their phenotype and family history.

Results The median age at diagnosis was 7.9 years (range: 0.8−17.8 years) with 5.1±1.8 cm of maximum tumor diameter. Adrenal adenoma (n=7), carcinoma (n=5), borderline (n=2), isolated micronodular adrenocortical disease (n=2), pheochromocytoma (n=8), paraganglioma (n=3), and ganglioneuroma (n=4) are all pathological diagnoses. The most common presenting symptom was excess production of adrenocortical hormones (n=15), including virilization and Cushing syndrome. Non-functioning adrenocortical tumors were found in a patient with congenital adrenal hyperplasia. Genetic etiologies were identified in TP53 (n=5), VHL (n=4), and PRKACA (n=1). Patients with mutations in TP53 were young (1.5±0.5 years) and had large masses (6.1±2.3 cm).

Conclusions This study describes clinical outcomes and the pathological spectrum of adrenal tumors in children and adolescents. Adrenocortical tumors mostly presented with an excess of the adrenocortical hormone. Patients with genetic defects presented at a young age and large size of tumors, necessitating genetic testing in patients at a young age.

Key words

adrenal tumor - Li Fraumeni syndrome - von Hippel-Lindau disease - pheochromocytoma

Supplementary Material



Publication History

Received: 24 February 2023
Received: 08 June 2023

Accepted: 06 July 2023

Accepted Manuscript online:
12 July 2023

Article published online:
06 September 2023

© 2023. Thieme. All rights reserved.

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