Jugulotympanic Paraganglioma (Glomus Tumor): A Case Report

Introduction: Jugulotympanic paragangliomas (JTPs) are benign, very slow-growing vascular tumors arising from extra-adrenal paraganglia of the autonomic nervous system. The incidence of JTP is approximately 1 in 1,300,000 population, and they are the second most common temporal bone tumor, after acoustic neurinoma. The most common clinical evidence is pulsatile tinnitus and multiple cranial neuropathies, usually facial (VII), vagus (X), hypoglossal (XII), glossopharyngeal (IX), and spinal accessory (XI). Magnetic resonance imaging (MRI) of the skull base and MR angiography are the methods of choice for diagnosis and determination of tumor size. Surgery and radiotherapy are the 2 modalities of treatment available. We describe an interesting case of JTP and discuss the symptoms, diagnostic procedure, and treatment of the entity. Case Report: A 79-year-old woman with a previous medical history of hypothyroidism was admitted in our hospital because of hoarseness of voice for the last 6 months. Recently she noticed difficulty in abduction of the left arm. Direct laryngoscopy revealed paralysis of the left vocal cord (X). Neurological examination showed weakness and severe atrophy of the left sternocleidomastoid muscle and upper part of the trapezius (XI).The uvula was deviated to the right (IX). Magnetic resonance imaging of the skull base showed a “salt and pepper” appearance typical of paraganglioma. Magnetic resonance imaging (T1 images) showed a large vascular mass, 4.7 × 1.5 cm in size spreading into the jugular foramen and extending superiorly into the left middle ear cavity and sigmoid sinus and inferiorly into the left internal auditory meatus. The angiographic description was also characteristic. She was treated by preoperative embolization and gamma-knife radiosurgery. Conclusion: Despite their rarity, JTPs are of considerable interest because: (1) of their vascular nature, (2) of the recent genetic research on familiar JTPs, and (3) they often cause multiple cranial nerve abnormalities, and (4) surgery and/or radiotherapy are acceptably safe and effective in treating them.