ArticleThe Journal of clinical endocrinology and metabolism2017
Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas.
Article in The Journal of clinical endocrinology and metabolism, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 74 papers, 3 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
74 citing papers in PubMed, 3 syntheses or guidelines pooled it, 177 citations in OpenAlex.
- Clinical characteristics and prognosis ofJournal of medical genetics · 2026Pooled it
- Metastatic Disease in Phaeochromocytomas and Paragangliomas in Various Genotypes-A Systematic Review and Meta-analysis.The Journal of clinical endocrinology and metabolism · 2026Pooled it
- Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2021Guideline
- Safety and dosimetry of [European journal of nuclear medicine and molecular imaging · 2025Trial
- Pediatric endocrine disorders: a review of intra-abdominal findings and appropriate imaging.Pediatric radiology · 2026Review
- Surgical management of para-aortic paragangliomas: case series of functional and non-functional tumors.International journal of surgery case reports · 2026Article
- Two Cases of Hereditary Paraganglioma-Pheochromocytoma Syndrome in Siblings, Caused by a Novel Succinate Dehydrogenase Complex Subunit D Variant.IJU case reports · 2026Article
- Multicenter evaluation of the PASS score as a negative predictive tool and the impact of inter-observer variability in pheochromocytoma and paraganglioma risk stratification.Journal of pathology and translational medicine · 2026Article
- Artificial Intelligence and Machine Learning in Pediatric Endocrine Tumors: Opportunities, Pitfalls, and a Roadmap for Trustworthy Clinical Translation.Biomedicines · 2026Review
- Biochemical Diagnosis of Phaeochromocytoma and Paraganglioma in Children and Adolescents: A Retrospective Cohort Study.Clinical endocrinology · 2025Article
- Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025Review
- [European journal of nuclear medicine and molecular imaging · 2025Article
- Takotsubo syndrome as initial presentation of pheochromocytoma in young children: case report and literature review.BMC pediatrics · 2025Review
- Prediction of Metastasis in Paragangliomas and Pheochromocytomas Using Machine Learning Models: Explainability Challenges.Sensors (Basel, Switzerland) · 2025Article
- Metastatic Malignant Phaeochromocytoma in Ascitic Fluid: Cytological Diagnosis of a Rare Entity.Cytopathology : official journal of the British Society for Clinical Cytology · 2025Article
- Adrenal Tumors in Children and Adolescents in Sweden: A Register-Based Study Covering 15 Years.Journal of the Endocrine Society · 2025Article
- Article
- Current Strategies for Managing Pheochromocytoma and Paraganglioma in Children and Adolescents.Medical science monitor : international medical journal of experimental and clinical research · 2025Review
- Genetics of urinary bladder paragangliomas: a multi-center study of a Chinese cohort.Journal of endocrinological investigation · 2025Article
- Composite Pheochromocytoma-Paraganglioma With Ganglioneuroma: A Dual-Center Clinical Experience.Journal of the Endocrine Society · 2025Article
14 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
17 authors at 13 institutions in 6 countries.
Funding
Abstract
contextPheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively.
objectiveTo identify differences in presentation of PPGLs between children and adults.
designA retrospective cross-sectional clinical study.
settingSeven tertiary medical centers. PATIENTS: The study included 748 patients with PPGLs, including 95 with a first presentation during childhood. Genetic testing was available in 611 patients. Other data included locations of primary tumors, presence of recurrent or metastatic disease, and plasma concentrations of metanephrines and 3-methoxytyramine.
resultsChildren showed higher (P < 0.0001) prevalence than adults of hereditary (80.4% vs 52.6%), extra-adrenal (66.3% vs 35.1%), multifocal (32.6% vs 13.5%), metastatic (49.5% vs 29.1%), and recurrent (29.5% vs 14.2%) PPGLs. Tumors due to cluster 1 mutations were more prevalent among children than adults (76.1% vs 39.3%; P < 0.0001), and this paralleled a higher prevalence of noradrenergic tumors, characterized by relative lack of increased plasma metanephrine, in children than in adults (93.2% vs 57.3%; P < 0.0001).
conclusionsThe higher prevalence of hereditary, extra-adrenal, multifocal, and metastatic PPGLs in children than adults represents interrelated features that, in part, reflect the lower age of disease presentation of noradrenergic cluster 1 than adrenergic cluster 2 tumors. The differences in disease presentation are important to consider in children at risk for PPGLs due to a known mutation or previous history of tumor.
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Registered trials
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 OpenQuestion graph.