Glomus Tumors (Paraganglioma)
Glomus tumors (paragangliomas) are mostly benign but locally invasive, highly vascular tumors arising from the glomus bodies of the head and neck. Types located in the temporal bone and skull base can present with pulsatile tinnitus and cranial nerve symptoms.
Ostatnia aktualizacja: 2026-06-07
Definition
Glomus tumors are highly vascular (hypervascular) tumors arising from the glomus bodies (chemoreceptor and neuroendocrine cells) associated with the autonomic nervous system. The vast majority are benign but grow locally invasively into surrounding bone and structures. By location there are types such as glomus tympanicum in the middle ear, glomus jugulare at the jugular foramen, and glomus vagale along the vagus nerve. They usually grow slowly.
Causes and Genetics
A significant proportion of cases are hereditary and are particularly associated with mutations in the SDH genes (SDHB, SDHC, SDHD). In hereditary cases tumors may appear at a younger age and may be multifocal or bilateral. Genetic counseling and testing are therefore advised in appropriate patients. A small proportion of these tumors can secrete hormones (catecholamines).
Symptoms
The earliest and most characteristic symptom of middle-ear glomus tympanicum is pulse-synchronous ringing in the ear (pulsatile tinnitus); this may be accompanied by conductive hearing loss. Otoscopy may reveal a reddish mass behind the eardrum. In larger, invasive glomus jugulare tumors there may be hearing loss, dizziness, and lower cranial nerve involvement causing swallowing difficulty, hoarseness and tongue/shoulder movement problems.
Diagnosis
Contrast-enhanced MRI is the gold standard for diagnosis; the tumor enhances intensely and, being highly vascular, gives a characteristic appearance. High-resolution temporal bone CT shows bone involvement and spread. Digital subtraction angiography (DSA) is used to assess vascular anatomy and to plan preoperative embolization when needed. DOTATATE PET/CT is useful for functional imaging. When hormone secretion is suspected, blood/urine catecholamine metabolites are measured; audiometry is performed for hearing.
Treatment
Treatment is individualized according to tumor size, location, symptoms and the patient's age/general condition. Active surveillance (watch-and-wait) with imaging may be appropriate for small, asymptomatic or slowly growing tumors and for older patients with comorbidities. Symptomatic or growing tumors are treated with surgical resection; as they are highly vascular, closing off the feeding vessels (embolization) shortly before surgery reduces bleeding. For tumors unsuitable for surgery or for residual tumor, stereotactic radiosurgery is an effective option.
Prognosis and Follow-up
As the vast majority of these tumors are benign, the long-term course is generally favorable; distant metastasis is rare. Because of their proximity to cranial nerves, both the tumor itself and treatment can affect functions such as hearing or swallowing/voice; this balance is important in choosing treatment. Long-term regular MRI follow-up is advised in hereditary cases and for residual tumor. Outcomes differ by patient and cannot be guaranteed.
Źródła
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:941.
- Winn HR, ed. Youmans Neurological Surgery. 6th ed. Saunders; 2011:1595.
- Lloyd RV, et al. WHO Classification of Tumours of Endocrine Organs — Paraganglioma. IARC. 2017.
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