J Pediatr Endocrinol Metab. 2025 Aug 25. doi: 10.1515/jpem-2025-0162. Online ahead of print.
ABSTRACT
OBJECTIVES: This study aims to characterize the clinical spectrum of pediatric adrenocortical tumors (P-ACTs) in individuals aged <20 years and to compare the distinguishing features of pediatric adrenocortical adenomas (P-ACAs) and carcinomas (P-ACCs).
METHODS: This retrospective study consisted of P-ACT patients who presented to our institute over the past 24 years (2001-2024). They were categorized as P-ACC or P-ACA based on Wieneke score. Data on clinical presentation, hormonal evaluation, imaging, management, and outcomes were recorded.
RESULTS: A total of 30 (22 females) P-ACT patients (7 P-ACA and 23 P-ACC) were identified. The median age at presentation was 7 (range, 1.0-20) years, with a median symptom duration of 6.0 months (range 0.6-42 months). All patients, except one 16-year-old having P-ACC, had hormonal hypersecretion. Cortisol and androgen co-secretion was most common (56.6 %), followed by isolated cortisol (26.6 %) and androgen hypersecretion (13.3 %). On steroid profiling by LCMS/MS, P-ACC patients had raised levels of multiple steroid metabolites (6-9) as compared to P-ACA (2-5). P-ACA patients were relatively older (17.0 vs. 6.0 years, p=0.045), more often had isolated cortisol secretion (57.1 vs. 17.3 %, p=0.060), had smaller tumors (2.8 vs. 8.5 cm, p=0.002), a lower incidence of calcification (0 vs. 80 %) and a lower percentage venous enhancement (PVE 109.6 vs. 187.8 %, p=0.008) and achieved higher disease remission rates (100 vs. 21.4 %, p=0.005).
CONCLUSIONS: CECT features, particularly PVE, may aid in differentiating P-ACT from P-ACC. Serum steroid panels may have a role in preoperative assessment, but their utility remains to be validated.
PMID:40847542 | DOI:10.1515/jpem-2025-0162
