ArticleThe Journal of clinical endocrinology and metabolism2023
Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma.
Article in The Journal of clinical endocrinology and metabolism, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025.Endocrine journal · 2026Guideline
- Recurrent paraganglioma of the urinary bladder 26 years after initial treatment: a case report.Annals of medicine and surgery (2012) · 2026Article
- Metachronous contralateral pheochromocytoma detected 62 years after initial surgery.JCEM case reports · 2026Article
- Genetic alterations of telomere maintenance pathways in paragangliomas.Archives of endocrinology and metabolism · 2026Article
- The GAPP Aggressivity Score Correlates with Total Enriched Somatic Variant Burden in Sporadic Pheochromocytoma-A Pilot Study.Cancers · 2026Article
- Who can safely discontinue lifelong follow-up among patients with sporadic pheochromocytoma and paraganglioma?Journal of internal medicine · 2026Article
- Bilateral pheochromocytoma: case series and review of treatment strategies based on genetic mutations.Translational andrology and urology · 2026Article
- Belzutifan for HIF2A-Related Pheochromocytoma and Paraganglioma: A Retrospective Study of Real-World Data.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2026Article
- Clinical and genetic features of older patients with pheochromocytomas and paragangliomas: A multicenter retrospective study.Journal of endocrinological investigation · 2026Article
- Massive Pheochromocytomatosis Treated With Cytoreductive Surgery.JCEM case reports · 2026Article
- Bladder paraganglioma presenting with atypical abdominal pain: A diagnostic challenge.Urology case reports · 2025Article
- Approach to the Patient With Metastatic Pheochromocytoma and Paraganglioma.The Journal of clinical endocrinology and metabolism · 2025Article
- Late-Onset Bone Metastasis 46 Years After Initial Surgery for Pheochromocytoma: A Case Report.IJU case reports · 2025Article
- Genetic Testing Referral Rates for Pheochromocytoma and Paraganglioma in an Academic Tertiary Centre.Clinical endocrinology · 2025Article
- CT-based radiomics deep learning signatures for non-invasive prediction of metastatic potential in pheochromocytoma and paraganglioma: a multicohort study.Insights into imaging · 2025Article
- International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.Nature reviews. Endocrinology · 2024Review
- A Rare Case of a Middle Mediastinal Mass With Intense and Diffuse Contrast Enhancement.Cureus · 2024Article
- Management and follow-up strategies for patients with head and neck paraganglioma.European journal of endocrinology · 2024Article
- The Immune Landscape of Pheochromocytoma and Paraganglioma: Current Advances and Perspectives.Endocrine reviews · 2024Review
- Imaging of Pheochromocytomas and Paragangliomas.Endocrine reviews · 2024Review
Corrections and comments
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Authors and funding
17 authors at 9 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextLong-term follow-up has been recommended for patients with pheochromocytoma or paraganglioma (PPGL) due to potential for recurrent disease. However, the need to follow patients with sporadic PPGL has recently become controversial.
objectiveTo investigate the prevalence of recurrence among patients with sporadic compared with hereditary PPGL and to identify predictors of recurrence for sporadic disease.
methodsThis multicenter study included retrospective data from 1127 patients with PPGL. In addition to sex and age at primary tumor diagnosis, clinical information included location, size, and catecholamine phenotype of primary tumors, genetic test results, and subsequent development of recurrent and/or metastatic disease. Patients with sporadic PPGL were defined as those with negative genetic test results.
resultsPrevalence of recurrence among patients with sporadic PPGL (14.7%) was lower (P < 0.001) than for patients with pathogenic variants that activate pseudohypoxia pathways (47.5%), but similar to those with variants that activate kinase pathways (14.9%). Among patients with sporadic recurrent PPGL, 29.1% and 17.7% were respectively diagnosed at least 10 and 15 years after first diagnosis. Multivariable regression analysis showed that a noradrenergic/dopaminergic phenotype (HR 2.73; 95% CI, 1.553-4.802; P < 0.001), larger size (HR 1.82; 95% CI, 1.113-2.962; P = 0.017) and extra-adrenal location (HR 1.79; 95% CI, 1.002-3.187; P = 0.049) of primary tumors were independent predictors of recurrence in sporadic PPGL.
conclusionPatients with sporadic PPGL require long-term follow-up, as supported by the 14.7% prevalence of recurrent disease, including recurrences at more than 10 years after first diagnosis. The nature of follow-up could be individualized according to tumor size, location, and biochemical phenotype.
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