This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
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. 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. 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. Treatment usually includes surgery.
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