ArticleEndocrine-related cancer2022
Head/neck paragangliomas: focus on tumor location, mutational status and plasma methoxytyramine.
Article in Endocrine-related cancer, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 4 of them syntheses that pooled it.
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Who cites it
35 citing papers in PubMed, 4 syntheses or guidelines pooled it, 38 citations in OpenAlex.
- Metastatic Disease in Phaeochromocytomas and Paragangliomas in Various Genotypes-A Systematic Review and Meta-analysis.The Journal of clinical endocrinology and metabolism · 2026Pooled it
- Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025.Endocrine journal · 2026Guideline
- Algorithm of genetic diagnosis for patients with head and neck paraganglioma-update.Frontiers in neurology · 2024Pooled it
- Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology.Frontiers in endocrinology · 2022Pooled it
- PPGLomics: an interactive platform for pheochromocytoma and paraganglioma transcriptomics.Endocrine-related cancer · 2026Article
- Synchronous Bilateral Carotid Body and Bilateral Vagal Paragangliomas: A Case Report and Literature Review.Journal of clinical medicine · 2026Article
- Pheochromocytomas and Paragangliomas.Endocrinology and metabolism clinics of North America · 2026Review
- Predominant catecholaminergic differentiation in tympanic paragangliomas: report of three consecutive cases.Virchows Archiv : an international journal of pathology · 2026Article
- Clinical and genetic characteristics of head and neck paragangliomas from China featuring Sino-European comparisons.Journal of the Endocrine Society · 2026Article
- Article
- Precision imaging and evolving therapies in paragangliomas and pheochromocytomas: from molecular diagnostics to imaging-guided management.Insights into imaging · 2026Article
- PPGLomics: An Interactive Platform for Pheochromocytoma and Paraganglioma Transcriptomics.bioRxiv : the preprint server for biology · 2026Article
- Surgical resection versus stereotactic radiosurgery for succinate dehydrogenase-related hereditary head and neck paragangliomas.Journal of neuro-oncology · 2026Article
- Biochemical Diagnosis of Phaeochromocytoma and Paraganglioma in Children and Adolescents: A Retrospective Cohort Study.Clinical endocrinology · 2025Article
- Approach to the Patient With Metastatic Pheochromocytoma and Paraganglioma.The Journal of clinical endocrinology and metabolism · 2025Article
- The Sensitivity of Arterial Spin-Labeling Imaging for Detection of Head and Neck Paragangliomas.AJNR. American journal of neuroradiology · 2025Article
- DecipheringJournal of endocrinological investigation · 2025Article
- Multi-omic analysis of SDHB-deficient pheochromocytomas and paragangliomas identifies metastasis and treatment-related molecular profiles.Nature communications · 2025Article
- Head and neck paraganglioma in Pacak-Zhuang syndrome.JNCI cancer spectrum · 2025Article
- Management and follow-up strategies for patients with head and neck paraganglioma.European journal of endocrinology · 2024Article
Corrections and comments
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Authors and funding
19 authors at 8 institutions in 6 countries.
Funding
Abstract
Head and neck paragangliomas (HNPGLs) are tumors of parasympathetic origin that occur at variable locations and are often secondary to germline mutations in succinate dehydrogenase (SDH) subunit genes. Occasionally, these tumors produce catecholamines. Here, we assessed whether different locations of HNPGLs relate to the presence of SDHx mutations, catecholamine production and other presentations. In this multicenter study, we collected clinical and biochemical data from 244 patients with HNPGLs and 71 patients without HNPGLs. We clarified that jugulotympanic HNPGLs have distinct features. In particular, 88% of jugulotympanic HNPGLs arose in women, among whom only 24% occurred due to SDHx mutations compared to 55% in men. Jugulotympanic HNPGLs were also rarely bilateral, were of a smaller size and were less often metastatic compared to carotid body and vagal HNPGLs. Furthermore, we showed that plasma concentrations of methoxytyramine (MTY) were higher (P < 0.0001) in patients with HNPGL than without HNPGL, whereas plasma normetanephrine did not differ. Only 3.7% of patients showed strong increases in plasma normetanephrine. Plasma MTY was positively related to tumor size but did not relate to the presence of SDHx mutations or tumor location. Our findings confirm that increases in plasma MTY represent the main catecholamine-related biochemical feature of patients with HNPGLs. We expect that more sensitive analytical methods will make biochemical testing of HNPGLs more practical in the future and enable more than the current 30% of patients to be identified with dopamine-producing HNPGLs. The sex-dependent differences in the development of HNPGLs may have relevance to the diagnosis, management and outcomes of these tumors.
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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.