ArticleThe Journal of clinical endocrinology and metabolism2015
Pheochromocytoma Screening Initiation and Frequency in von Hippel-Lindau Syndrome.
Article in The Journal of clinical endocrinology and metabolism, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 1 of them a synthesis that pooled it.
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Who cites it
36 citing papers in PubMed, 1 synthesis or guideline pooled it, 79 citations in OpenAlex.
- Total adrenalectomy versus subtotal adrenalectomy for bilateral pheochromocytoma: meta-analysis.BJS open · 2023Pooled it
- Article
- Bilateral pheochromocytoma: case series and review of treatment strategies based on genetic mutations.Translational andrology and urology · 2026Article
- Cancer predisposition syndromes: an imaging review.Cancer imaging : the official publication of the International Cancer Imaging Society · 2026Review
- Diagnosis and treatment of Von Hippel-Lindau syndrome: A case series.Experimental and therapeutic medicine · 2025Article
- Pediatric and adolescent von Hippel-Lindau disease: tumor profiles, genotype-phenotype correlation and comparison with adults.Journal of endocrinological investigation · 2025Article
- New Developments in VHL-Associated Neuroendocrine Neoplasms.Current oncology reports · 2025Review
- CLINICAL EVALUATION, DIAGNOSIS AND TREATMENT OF PHEOCHROMOCYTOMA.Acta clinica Croatica · 2024Review
- Computed tomography-guided percutaneous cryoablation of hereditary adrenal pheochromocytoma in three patients.Journal of pediatric endocrinology & metabolism : JPEM · 2024Article
- Overview of endocrine tumor syndromes manifesting as adrenal tumors.Ewha medical journal · 2024Review
- A complementary comment on primary hepatic angiosarcoma: A case report.World journal of clinical cases · 2023Article
- Clinical, Biochemical, Tumoural and Mutation Profile of VHL- and MEN2A-Associated Pheochromocytoma: A Comparative Study.World journal of surgery · 2022Article
- Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: not a needle in a haystack.Endocrine connections · 2021Review
- A Critical Appraisal of Contemporary and Novel Biomarkers in Pheochromocytomas and Adrenocortical Tumors.Biology · 2021Review
- Long term outcomes for patients with von Hippel-Lindau and Pheochromocytoma: defining the role of active surveillance.Urologic oncology · 2021Article
- Phakomatoses and Endocrine Gland Tumors: Noteworthy and (Not so) Rare Associations.Frontiers in endocrinology · 2021Review
- Clinical decision making in small non-functioning VHL-related incidentalomas.Endocrine connections · 2020Article
- Hereditary Syndromes in Neuroendocrine Tumors.Current treatment options in oncology · 2020Review
- Clinical and Biochemical Features of Pheochromocytoma Characteristic of Von Hippel-Lindau Syndrome.World journal of surgery · 2020Article
- Von Hippel-Lindau Disease: Current Challenges and Future Prospects.OncoTargets and therapy · 2020Review
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextPatients with von Hippel-Lindau (VHL) syndrome have a 25-30% chance of developing pheochromocytoma. Although practice guidelines recommend biochemical and radiological screening every 1-2 years for pheochromocytoma in patients with VHL, there are limited data on the optimal age and frequency for screening.
objectiveOur objective was to determine the earliest age of onset and frequency of contralateral and recurrent pheochromocytomas in patients with VHL syndrome.
methodsThis is a retrospective analysis of a prospective cohort of patients with VHL enrolled in a natural history study.
resultsA total of 273 patients diagnosed with VHL were enrolled in a natural history clinical study. Thirty-one percent (84) were diagnosed with pheochromocytoma. The mean age of diagnosis was 28.8 ± 13.9 years. The earliest age at diagnosis was 5.5 years. Median follow-up for the cohort was 116.6 months (range, 0.1-613.2). Ninety-nine percent (83) of patients underwent adrenalectomy. Fifty-eight and 32% of patients had metanephrines and/or catecholamines elevated more than two times and more than four times the upper limit of normal, respectively. Twenty-five percent (21) of pheochromocytomas were diagnosed in pediatric patients younger than 19 years of age, and 86% and 57% of pediatric patients had an elevation more than two times and more than four times upper limit of normal, respectively. Eight patients had a total of nine recurrences. The median age at recurrence was 33.5 years (range, 8.8-51.9). Recurrences occurred as short as 0.5 years and as long as 39.7 years after the initial operation.
conclusionsOur findings among VHL pediatric patients supports the need for biochemical screening starting at age 5 with annual lifelong screening.
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