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Disease Profile

Glomus vagale tumor

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.



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Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Vagal paraganglioma


Rare Cancers


Glomus vagale tumor is a type of paraganglioma, which is a tumor that develops in the paraganglia (a group of cells that are found near nerve cell bunches called ganglia). Glomus vagale tumors, specifically, grow in the paraganglia located near the vagus nerve, an important cranial nerve which serves many functions including empowering the vocal cords and the muscles of swallowing. In most cases, the tumors are benign, although rare cancerous cases have been reported. Signs and symptoms vary based on the size and location of the tumor but may include a neck mass, tinnitus (ringing or buzzing in the ears), difficulty swallowing, hoarseness, pain, cough, and/or cranial nerve paralysis. Some tumors may not be associated with any concerning features and are diagnosed by chance during imaging studies performed to investigate other conditions.[1][2] In many cases, the underlying cause of glomus vagale tumors is poorly understood. Approximately 40-50% of affected people report a family history of the condition.[3][1] Glomus vagale tumors can be associated with inherited syndromes caused by genetic changes (mutations) in succinate dehydrogenase subunits (i.e. SDHB, SDHD, SDHA, SDHAF2, SDHC) or the MAX gene.[4][1] Treatment usually includes surgery.[1][2]


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Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Glomus vagale tumor. Click on the link to view a sample search on this topic.


        1. Salman Kirmani, MBBS and William F Young, MD, MSc. Hereditary Paraganglioma-Pheochromocytoma Syndromes. GeneReviews. November 2014; https://www.ncbi.nlm.nih.gov/books/NBK1548/.
        2. Righi S, Boffano P, Malvè L, Pateras D, Chiodo D, Boson M. Malignant vagal paraganglioma. J Craniofac Surg. September 2014; 25(5):e460-461.
        3. Zanoletti E, Mazzoni A. Vagal paraganglioma. Skull Base. August 2006; 16(3):161-167.
        4. Jennings AW, Preskitt JT, Vallera RD. Extraadrenal pheochromocytoma and vagal paraganglioma. Proc (Bayl Univ Med Cent). April 2012; 25(2):152-154.