[Tympanojugular Glomus Tumor/Tympanojugular Paraganglioma].

Abstract

Glomus tumors, also known as paragangliomas, were previously classified as benign tumors; however, the WHO classification of endocrine and neuroendocrine tumors 4^th edition no longer classified paragangliomas as benign and malignant given any lesion can have metastatic potential. Temporal bone glomus tumors are classified into glomus tympanicum tumors and glomus jugulare tumors. Complete surgical resection is preferred for glomus tympanicum tumors whereas, for glomus jugulare tumors, it is necessary to evaluate age, tumor extension, hearing, and neurological symptoms to determine treatment strategy. In cases of catecholamine production, cranial nerve paralysis, young age, and SDHB gene mutations, surgery should be considered. The infratemporal fossa type A approach is one of the main surgical approaches for glomus jugulare tumors. Management of glomus jugulare tumors requires a thorough understanding of pathophysiology of the tumor including biochemistry, genetics, and metastasis. Surgery, radiotherapy, and active surveillance are treatment options, and should be individualized to patients to maintain quality of life.