Clinical spectrum, imaging characteristics, and treatment outcomes of pediatric adrenocortical tumors: a 24-year experience from Western India
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Aaditya Daga
, Siddhant Dahale , Aditya Phadte , Vijay Sarathi , Saba Memon , Anurag Lila, Chethan Yamichannaiah
, Virendra Patil , Manjiri Karlekar , Rohit Barnabas , Nalini Shah , Amey Rojekar , Gwendolyn Fernandez , Pragati Sathe , Padma Badhe und Tushar Bandgar
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.
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Research ethics: Ethical approval was obtained from the Institutional Ethics Committee (EC/OA-01/2025).
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Informed consent: Waiver of consent was granted in view of retrospective nature of the study.
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Author contributions: All authors contributed to the study conception and design. Data collection and analysis were performed by Siddhant Dahale and Aaditya Daga. The first draft of the manuscript was written by Siddhant Dahale and Aaditya Daga. All authors commented on previous versions of the manuscript. All authors have accepted responsibility for the entire content of this manuscript and approved its submission.
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Use of Large Language Models, AI and Machine Learning Tools: None declared.
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Conflict of interest: The authors state no conflict of interest.
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Research funding: None declared.
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Data availability: Not applicable.
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Supplementary Material
This article contains supplementary material (https://doi.org/10.1515/jpem-2025-0162).
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Artikel in diesem Heft
- Frontmatter
- Review
- A multi-stakeholder perspective on medical devices for children and adolescents with type 1 diabetes: huge unmet needs for the smallest
- Original Articles
- Concerns for mood disorders in children presenting with early menarche is not an indication for pubertal suppression
- Testosterone in Duchenne muscular dystrophy: effects on puberty and growth
- First evaluation of fibroblast growth factor 21 levels in patients diagnosed with glycogen storage diseases with liver involvement
- Improving cardiometabolic health in children and adolescents with obesity: a comparison between in-person and virtual supervised training
- Impact of high-dose vitamin D supplementation initiated shortly after diagnosis on residual beta cell function and partial remission rates in children with type 1 diabetes
- Clinical spectrum, imaging characteristics, and treatment outcomes of pediatric adrenocortical tumors: a 24-year experience from Western India
- Comparison study of the correlation between free and total 25(OH)D in maternal and umbilical blood and early-life physical development parameters
- Hashimoto’s thyroiditis in children and adolescents: analysis of long-term course
- Case Reports
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- Hormone-active ovarian steroid cell tumor in a 2-year-old girl
- Lipoatrophy following weekly growth hormone therapy: a case report
- Surgical treatment and somatostatin experience in growth hormone-secreting pituitary macroadenoma due to novel AIP mutation
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