Journal of pediatric endocrinology & metabolism : JPEM · 2025
Clinical spectrum, imaging characteristics, and treatment outcomes of pediatric adrenocortical tumors: a 24-year experience from Western India.
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Daga Aaditya, Dahale Siddhant, Phadte Aditya, Sarathi Vijay, Memon Saba, Lila Anurag, Yamichannaiah Chethan, Patil Virendra, Karlekar Manjiri, Barnabas Rohit, Shah Nalini, Rojekar Amey, Fernandez Gwendolyn, Sathe Pragati, Badhe Padma, Bandgar Tushar
Аннотация
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.
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.