ArticleThe Journal of clinical endocrinology and metabolism2008
Biochemically silent abdominal paragangliomas in patients with mutations in the succinate dehydrogenase subunit B gene.
Article in The Journal of clinical endocrinology and metabolism, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 68 papers, 2 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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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.
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Who cites it
68 citing papers in PubMed, 2 syntheses or guidelines pooled it, 121 citations in OpenAlex.
- Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025.Endocrine journal · 2026Guideline
- Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology.Frontiers in endocrinology · 2022Pooled it
- Double faces of pheochromocytoma and paraganglioma-a review of integrated pathologic diagnosis.Endocrine journal · 2026Review
- A Biochemically "Silent" Aortocaval Paraganglioma in a 19-Year-Old Female Causing Intraoperative Hypertensive Crisis: A Rare Case Report.Clinical case reports · 2026Article
- Fluctuating hypertension possibly leading to severe cardiomyopathy in SDHB-associated hereditary paraganglioma: from heart transplant candidacy to long-term lanreotide management: a case report.Frontiers in cardiovascular medicine · 2026Article
- Clinical significance, prognosis, and risk model of SDHB expression loss and ERBB2 expression in pheochromocytoma and paraganglioma.Endocrine connections · 2025Article
- Succinate dehydrogenase-related pheochromocytoma and paraganglioma (SDHx-PPGL): clinical and genetic insights from an Indian study.Endocrine connections · 2025Article
- Long-Term Persistence of Glycemic Dysregulation in Patients With a History of Pheochromocytoma/Paraganglioma.The Journal of clinical endocrinology and metabolism · 2025Article
- Article
- The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives.Pharmaceuticals (Basel, Switzerland) · 2024Review
- From Nonfunctioning Adrenocortical Cancer to Biochemically Silent Paraganglioma Associated withCase reports in endocrinology · 2024Article
- Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options.Cancer medicine · 2023Review
- Abdominal nonfunctional paraganglioma in which succinate dehydrogenase subunit B (SDHB) immunostaining was performed: a case report.Journal of medical case reports · 2023Article
- Co-occurrence of pheochromocytoma and paraganglioma of the organ of Zuckerkandl resected simultaneously by laparoscopy: a rare case report and literature review.The Journal of international medical research · 2023Review
- Primary Functioning Hepatic Paraganglioma Treated by Laparoscopy: A Case Report.Journal of clinical medicine · 2022Article
- Pre- and peri-operative characteristics, complications and outcomes of patients with biochemically silent pheochromocytomas; a case series.Endocrine · 2022Article
- The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines.Journal of clinical medicine · 2022Article
- Surveillance Improves Outcomes for Carriers of SDHB Pathogenic Variants: A Multicenter Study.The Journal of clinical endocrinology and metabolism · 2022Article
- Personalized Management of Pheochromocytoma and Paraganglioma.Endocrine reviews · 2022Review
- Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.Endocrine pathology · 2022Review
8 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
9 authors at 5 institutions in 3 countries.
Funding
Abstract
contextPatients with adrenal and extra-adrenal abdominal paraganglioma (PGL) almost invariably have increased plasma and urine concentrations of metanephrines, the O-methylated metabolites of catecholamines. We report four cases of biochemically silent abdominal PGL, in which metanephrines were normal despite extensive disease.
objectiveOur objective was to identify the mechanism underlying the lack of catecholamine hypersecretion and metabolism to metanephrines in biochemically silent PGL.
designThis is a descriptive study.
settingThe study was performed at a referral center. PATIENTS: One index case and three additional patients with large abdominal PGL and metastases but with the lack of evidence of catecholamine production, six patients with metastatic catecholamine-producing PGL and a mutation of the succinate dehydrogenase subunit B (SDHB) gene, and 136 random patients with catecholamine-producing PGL were included in the study.
main outcome measuresPlasma, urine, and tumor tissue concentrations of catecholamines and metabolites were calculated with electron microscopy and tyrosine hydroxylase immunohistochemistry.
resultsAll four patients with biochemically silent PGL had an underlying SDHB mutation. In the index case, the tumor tissue concentration of catecholamines (1.8 nmol/g) was less than 0.01% that of the median (20,410 nmol/g) for the 136 patients with catecholamine-producing tumors. Electron microscopy showed the presence of normal secretory granules in all four biochemically silent PGLs. Tyrosine hydroxylase immunoreactivity was negligible in the four biochemically silent PGLs but abundant in catecholamine-producing PGLs.
conclusionsPatients with SDHB mutations may present with biochemically silent abdominal PGLs due to defective catecholamine synthesis resulting from the absence of tyrosine hydroxylase. Screening for tumors in patients with SDHB mutations should not be limited to biochemical tests of catecholamine excess.
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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.