ArticleGMS current topics in otorhinolaryngology, head and neck surgery2011
Paragangliomas and paraganglioma syndromes.
Article in GMS current topics in otorhinolaryngology, head and neck surgery, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 63 citations in OpenAlex.
- Immunohistochemistry and Mutation Analysis ofInternational journal of molecular sciences · 2020Trial
- Treatment Outcomes of Radiation Therapy of Paragangliomas of the Head and Neck at a Single Institution.International journal of particle therapy · 2026Article
- Malignant Carotid Body Tumor in a Young Female Patient: Case Report with Review of Literature.Cureus · 2025Article
- Pros and cons of surgical versus conservative management for head and neck paraganglioma: a real-world data analysis.Endocrine · 2025Article
- Gruppo Otologico's Experience in Managing the So-Called Inoperable Tympanojugular Paraganglioma.Brain sciences · 2024Article
- Malignant Carotid Paraganglioma: A Case Report.Cureus · 2023Article
- Midline mandibulotomy for large carotid body tumors: a valuable approach.Journal of vascular surgery cases and innovative techniques · 2023Article
- Somatic Mutation Profiling in Head and Neck Paragangliomas.The Journal of clinical endocrinology and metabolism · 2022Article
- A Rare Case of a Right Atrial Paraganglioma in an Individual with the SHDB Mutation.Case reports in cardiology · 2022Article
- Head and Neck Paragangliomas in the Czech Republic: Management at the Otorhinolaryngology Department.Diagnostics (Basel, Switzerland) · 2021Article
- Head and neck paragangliomas: Ten years of experience in a third health center. A cohort study.Annals of medicine and surgery (2012) · 2021Article
- Jugular foramen paragangliomas: preoperative transcatheter particle embolization.Journal of cerebrovascular and endovascular neurosurgery · 2020Article
- Head and Neck Paragangliomas-A Genetic Overview.International journal of molecular sciences · 2020Review
- Mutation profiling in eight cases of vagal paragangliomas.BMC medical genomics · 2020Article
- Evolving management strategies in head and neck paragangliomas: A single-centre experience with 147 patients over a 60-year period.Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery · 2019Article
- Mathematical Models for Tumor Growth and the Reduction of Overtreatment.Journal of neurological surgery. Part B, Skull base · 2019Article
- Nationwide study of patients with head and neck paragangliomas carrying SDHB germline mutations.BJS open · 2018Article
- Bilateral carotid body tumor resection in a female patient.International journal of surgery case reports · 2017Article
- Familial paraganglioma syndrome: a rare cause of carotid artery occlusion.Journal of neurology · 2016Article
- Growth Rate Analysis of an Untreated Glomus Vagale on MRI.Case reports in otolaryngology · 2016Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Paragangliomas are rare tumors of neural crest origin. They are benign in the majority of cases and are characterized by a strong vascularisation.In the head and neck region they most commonly occur as carotid body tumors. Jugulotympanic and especially vagal paragangliomas are seen less frequently. Complete surgical resection represents the only curative treatment option even though resection of locally advanced tumors regularly results in lesions of the lower cranial nerves and major vessels. Appoximately 30% of all head and neck paragangliomas (HNPs) are hereditary and associated with different tumor syndromes. The paraganglioma syndromes 1, 3 and 4 (PGL 1, 3 and 4) make up the majority of those familial cases. PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC and PGL 4 by SDHB gene mutations. Multiple HNPs and the occurance of HNPs together with pheochromocytomas are seen in SDHD as well as SDHB mutation carriers. In patients with SDHB mutations the risk for the development of malignant paraganglial tumors is significantly higher compared to SDHC and SDHD patients as well as patients with sporadic tumors. SDHC mutation carriers almost exclusively present with benign HNP that are unifocal in the majority of cases. The role of transmission is autosomal dominant for all three symptoms. Interestingly, there is a "parent-of-origin-dependent-inheritance" in subjects with SDHD gene mutations. This means that the disease phenotype may only become present if the mutation is inherited through the paternal line. We recommend screening for mutations of the genes SDHB, SDHC and SDHD in patients with HNPs. Certain clinical parameters can help to set up the order in which the three genes should be tested.
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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.