Prolonged symptom duration and the potential for gradual progression in pediatric adrenocortical tumors: observations from the MET studies
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Michaela Kuhlen
,
Stefan A. Wudy
Abstract
Objectives
To explore the clinical spectrum and symptom duration in pediatric adrenocortical tumors (pACTs), with a focus on identifying cases that may reflect gradual tumor progression.
Methods
We retrospectively analyzed data from 110 pediatric patients with pACTs enrolled in the German Pediatric Oncology Hematology-Malignant Endocrine Tumor (GPOH-MET) studies (1997–2022). Endocrine symptom duration, histopathological classification, and clinical outcomes were assessed. Patients with symptom durations ≥2 standard deviations (SDs) from the mean were defined as outliers and evaluated for potential progression.
Results
The cohort included 31 patients with adrenocortical adenomas (ACAs), 12 with tumors of uncertain malignant potential (ACx), and 67 with adrenocortical carcinomas (ACCs). Seven patients (6.4 %) showed markedly prolonged symptom duration, including four with ACC. One representative case demonstrated a nearly 5-year course from initial androgen excess to metastatic ACC, with evolving biochemical features and a diagnostic urinary steroid profile indicative of adrenal tumor activity.
Conclusions
A small subset of pACTs may present with prolonged endocrine symptoms, possibly reflecting gradual tumor evolution. While molecular validation is lacking, these findings support the need for early recognition and further research into the natural history of pACTs.
Funding source: Mitteldeutschen Kinderkrebsforschung
Award Identifier / Grant number: not applicable
Funding source: German Childhood Cancer Foundation
Award Identifier / Grant number: DKS 2014.06, DKS 2017.16, DKS 2021.11, DKS 2024.16
Funding source: Faculty of Medicine, University of Augsburg
Award Identifier / Grant number: not applicable
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Research ethics: The MET studies were approved by the Ethics Committee of the University of Luebeck (IRB 97125) and Otto-von-Guericke University Magdeburg (IRB 174/12 and 52/22), Germany.
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Informed consent: Informed consent for participation in the study was obtained from all participants or, where applicable, from their parents or legal guardians. Informed consent for the publication of data was included as part of the informed consent process.
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Author contributions: MiKu: Conceptualization, Methodology, Investigation, Resources, Writing-original draft, Writing-review & editing, Supervision, Project administration, Funding acquisition. SAW: Investigation, Writing-review & editing. CB: Investigation, Writing-review & editing. MFH: Investigation, Writing-review & editing. MaKu: Investigation, Writing-review & editing, Project administration. CV: Investigation, Writing-review & editing. RC: Methodology, Writing-original draft, Writing-review & editing. AR: Formal analysis, Resources, Data Curation, Visualization, Writing-review & editing, Project administration, Funding acquisition. 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: To improve language ChatGPT was used.
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Conflict of interest: The authors state no conflict of interest.
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Research funding: The German MET studies were funded by Deutsche Kinderkrebsstiftung, grant number DKS 2014.06, DKS 2017.16, DKS 2021.11, DKS 2024.16, Mitteldeutsche Kinderkrebsforschung, and Magdeburger Förderkreis krebskranker Kinder e.V.
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Data availability: Not applicable.
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Articles in the same Issue
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- Mini Review
- Transition of care from childhood/adolescence to adulthood in familial hypercholesterolemia
- Original Articles
- Performance of adult height prediction methods in 6 to 8-year-old girls with GnRH-dependent precocious puberty
- Height velocity in the detection of growth disorders reconsidered: a retrospective analysis of the DONALD study
- Differentiating transient and permanent congenital hypothyroidism: predictive clues from Istanbul, Türkiye
- Evaluation of thyrotrophic and lactotrophic reserves in patients with pituitary dwarfism with and without empty sella turcica
- Exploring the impact of androgen levels on depression and anxiety in adolescent females: a clinical perspective
- The diagnostic value of stimulated androgen ratios in 5-alpha reductase type 2 (SRD5A2) deficiency: a case series and review of the literature
- Prolonged symptom duration and the potential for gradual progression in pediatric adrenocortical tumors: observations from the MET studies
- Distinguishing organic from idiopathic central precocious puberty: clinical characteristics and predictive factors for organic etiology in a multicenter Italian cohort study
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- Short Communications
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- Letter to the Editor
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- Case Reports
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- Caudal epidural steroid injection as a novel therapy for treatment-induced neuropathy of diabetes in children: report of two cases
- A novel homozygous missense DNAJC3 variant in syndromic juvenile-onset diabetes
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Articles in the same Issue
- Frontmatter
- Mini Review
- Transition of care from childhood/adolescence to adulthood in familial hypercholesterolemia
- Original Articles
- Performance of adult height prediction methods in 6 to 8-year-old girls with GnRH-dependent precocious puberty
- Height velocity in the detection of growth disorders reconsidered: a retrospective analysis of the DONALD study
- Differentiating transient and permanent congenital hypothyroidism: predictive clues from Istanbul, Türkiye
- Evaluation of thyrotrophic and lactotrophic reserves in patients with pituitary dwarfism with and without empty sella turcica
- Exploring the impact of androgen levels on depression and anxiety in adolescent females: a clinical perspective
- The diagnostic value of stimulated androgen ratios in 5-alpha reductase type 2 (SRD5A2) deficiency: a case series and review of the literature
- Prolonged symptom duration and the potential for gradual progression in pediatric adrenocortical tumors: observations from the MET studies
- Distinguishing organic from idiopathic central precocious puberty: clinical characteristics and predictive factors for organic etiology in a multicenter Italian cohort study
- Endocrinopathies associated with pediatric common variable immunodeficiency
- Genetic, neuroimaging, and clinical characteristics of a cohort of individuals with L-2-hydroxyglutaric aciduria from Türkiye
- Short Communications
- Stress hyperglycemia in pediatric patients as a risk factor for type 1 diabetes – a single center experience
- Observations of the effect of gonadotropin-releasing hormone analog treatment on psychosocial well-being in transgender youth and their caregivers – a pilot study
- Letter to the Editor
- GNB1 haploinsufficiency presents as monogenic obesity syndrome
- Case Reports
- Rare pediatric insulinoma case diagnosed by endoscopic ultrasonography: insights into endogenous hyperinsulinemic hypoglycemia
- Caudal epidural steroid injection as a novel therapy for treatment-induced neuropathy of diabetes in children: report of two cases
- A novel homozygous missense DNAJC3 variant in syndromic juvenile-onset diabetes
- 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: case report of a child with rare HMGCL gene variants
