グロームス腫瘍／パラガングリオーマ

Tympanojugular Glomus Tumor/Tympanojugular Paraganglioma

Point

・褐色細胞腫・パラガングリオーマは近年のWHO（World Health Organization）分類により，悪性腫瘍と定義されるようになった．

・鼓室型グロームス腫瘍は手術による摘出が原則である．

・頚静脈球型グロームス腫瘍は年齢，進展範囲，神経症状に応じて個々に治療方針を検討するべきである．

＊本論文中、［Video］マークのある図につきましては、関連する動画を見ることができます（公開期間：2028年8月まで）。

　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.