Evidence map›Paper›PMID 40577853›Full record

ArticleJournal of pediatric endocrinology & metabolism : JPEM2025

Prolonged symptom duration and the potential for gradual progression in pediatric adrenocortical tumors: observations from the MET studies.

Michaela Kuhlen, Stefan A Wudy, Clara Baumann, Christian Vokuhl, Michaela F Hartmann, Marina Kunstreich, Rainer Claus, Antje Redlich

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Article in Journal of pediatric endocrinology & metabolism : JPEM, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Michaela KuhlenPediatrics and Adolescent Medicine, Faculty of Medicine, 531257 University of Augsburg , Augsburg, Germany.ORCID https://orcid.org/0000-0003-4577-0503
Stefan A WudySteroid Research & Mass Spectrometry Unit, Laboratory for Translational Hormone Analytics, Pediatric Endocrinology & Diabetology, Centre of Child and Adolescent Medicine, Justus Liebig University Giessen, Giessen, Germany.
Clara BaumannDepartment of Pediatric Kidney, Liver, Metabolic and Neurological Diseases, Children's Hospital, Hannover Medical School, Hannover, Germany.
Christian VokuhlSection of Pediatric Pathology, Institute of Pathology, University of Bonn, Bonn, Germany.ORCID https://orcid.org/0000-0002-4138-4536
Michaela F HartmannSteroid Research & Mass Spectrometry Unit, Laboratory for Translational Hormone Analytics, Pediatric Endocrinology & Diabetology, Centre of Child and Adolescent Medicine, Justus Liebig University Giessen, Giessen, Germany.ORCID https://orcid.org/0000-0001-7232-9532
Marina KunstreichPediatrics and Adolescent Medicine, Faculty of Medicine, 531257 University of Augsburg , Augsburg, Germany.ORCID https://orcid.org/0000-0002-2672-4045
Rainer ClausBavarian Cancer Research Center (BZKF), Augsburg, Germany.ORCID https://orcid.org/0000-0003-2617-8766
Antje RedlichDepartment of Pediatrics, Pediatric Hematology/Oncology, Otto-von-Guericke-University, Magdeburg, Germany.ORCID https://orcid.org/0000-0002-1732-1869

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo explore the clinical spectrum and symptom duration in pediatric adrenocortical tumors (pACTs), with a focus on identifying cases that may reflect gradual tumor progression.

methodsWe 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.

resultsThe 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.

conclusionsA 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.

Indexed as

Adrenal Cortex NeoplasmsAdrenocortical AdenomaAdrenocortical CarcinomaAdolescentChildChild, PreschoolDisease ProgressionFemaleFollow-Up StudiesHumansInfantMalePrognosisRetrospective Studiesadenomaadrenocortical tumorcarcinomachildren and adolescentssequence

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.