SynthesisThe Journal of clinical endocrinology and metabolism2026
Metastatic Disease in Phaeochromocytomas and Paragangliomas in Various Genotypes-A Systematic Review and Meta-analysis.
Synthesis in The Journal of clinical endocrinology and metabolism, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 1 of them a synthesis that pooled it.
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Who cites it
5 citing papers in PubMed, 1 synthesis or guideline pooled 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
- Biomarkers of metastatic disease in pheochromocytoma and paraganglioma.Endocrine connections · 2026Article
- Multi-omics insights into metastatic pheochromocytomas and paragangliomas: Mechanisms, signatures, and prediction models.Hormones (Athens, Greece) · 2026Review
- Bilateral pheochromocytoma: case series and review of treatment strategies based on genetic mutations.Translational andrology and urology · 2026Article
- Tumor risks and surveillance outcomes inEndocrine oncology (Bristol, England) · 2026Article
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Authors and funding
5 authors.
Funding
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Abstract
backgroundParagangliomas and phaeochromocytomas (PPGLs) are rare neuroendocrine tumours with 10% to 20% of patients developing metastatic disease. The tumors exhibit a high degree of heritability, and pathogenic genetic variants have been associated with metastases.
objectiveWe aimed to investigate the association between the genotype of PPGL patients and their risk of metastatic disease, adjusting for time.
methodsA systematic search was conducted in PubMed and Embase. The primary outcome was the rate of metastatic disease per 100 followed patient-years analyzed through meta-analyses.
resultsA significantly increased rate of metastatic disease per 100 followed patient-years was observed in all pathogenic germline variants included in the analyses, compared to the group with no identified variant. The group with no variant had a rate of 1.55 per 100 patient-years. SDHA, SDHC, SDHD, VHL, RET, NF1, and MAX had rates of 13.73, 6.27, 2.03, 2.34, 1.91, 4.11, and 9.66, respectively. The rate of SDHB is not presented as statistical heterogeneity exceeded 75%. The pathogenic somatic variant EPAS1 showed a rate of 3.82. Cluster-divided meta-analyses resulted in rates of 4.41 and 3.0 for cluster 1 and cluster 2, respectively. Meta-regression analysis revealed a 2.3-fold higher rate for the SDHB variant compared to the other cluster 1 variants.
conclusionWe present associations between genotype and metastatic disease in PPGL patients. Our results indicate that patients harboring a pathogenic genetic variant have a higher rate of metastases compared to patients with no identified variant. High heterogeneity in several analyses suggests that further large cohort studies are needed.
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