Glomus Jugulare Tumors
Glomus jugulare tumors are rare growths that arise in the jugular foramen of the temporal bone near the skull base. Common surgical treatments for glomus jugulare tumors are craniotomy and endoscopic endonasal surgery, and nonsurgical treatment is stereotactic radiosurgery. At Columbia’s Skull Base Tumor Center, our neurosurgeons specialize in providing care for patients with these tumors.
Usually slow-growing and benign, glomus jugulare tumors are a type of paraganglioma. Glomus jugulare tumors arise from glomus cells located within the outermost wall of the jugular bulb. These highly vascular tumors obtain blood supply from the external carotid artery and internal carotid artery and can extend into the middle ear and brain. In rare instances, these tumors can secrete catecholamines, and in four percent of cases, the tumors metastasize.
Often, glomus jugulare tumors remain asymptomatic until they are quite large. Symptoms of a glomus jugulare tumor include:
- Hearing loss
- Ear fullness
- Ear pain
- Pulsatile tinnitus
- Facial paralysis
Diagnosis of glomus jugulare tumors typically begins with a series of exams to establish symptoms.
- Neurological examination involves evaluating eye movements, sense of smell, hearing, motor function, sensation, swallowing, balance, and coordination.
- Otoscopic examination involves examining the ear canal, tympanic membrane, and middle ear. If present, the tumor may appear reddish-blue behind the tympanic membrane.
- Audiologic examination involves measuring the sensitivity of a person’s hearing and can identify hearing loss.
The key method to diagnosing glomus jugulare tumors is imaging. Available tests include plain X-ray, magnetic resonance imaging (MRI) scan, and computed tomography (CT) scan; these scans provide pictures of the tumor. MRI is usually the method of choice because it provides superior detail of the tumor and other neural structures. To enhance the tumor image, MRI or CT scans may be conducted with or without contrast enhancement.
An angiogram may also be conducted if preoperative embolization is planned to minimize risk of bleeding during surgery to remove the tumor.
In addition, to detect the presence of a catecholamine-secreting tumor, blood, and urine may be collected for laboratory testing.
Biopsy is needed to confirm a diagnosis of a glomus jugulare tumor and can be conducted before or during surgery to remove the tumor.
The cause of a glomus jugulare tumor is usually unclear.
Most instances are sporadic; however, some cases may be familial and are associated with the genetic condition neurofibromatosis type 1 or mutations in the succinate dehydrogenase gene.
Glomus jugulare tumors are rare, occurring in one in every 1.3 million people each year. These tumors are much more common in women than men and tend to arise between the ages of 40 and 70.
At Columbia, our neurosurgeons use the latest surgical techniques to treat glomus jugulare tumors successfully, resulting in the best possible outcomes. Because these tumors involve the complex structures of the ear, brain, and neck, surgery may be performed by a team that includes a neurosurgeon, head and neck surgeon, and neurotologist.
Treatment involves a combination of medical therapy, tumor resection, and radiotherapy. Some tumors that are not causing problems may not require immediate treatment and instead are observed carefully.
For tumors that actively secrete catecholamines, medical therapy is useful for palliation or as adjuvant treatment before surgery. Alpha and beta blockers are given before surgery to prevent blood pressure lability and arrhythmias of the heart.
Glomus jugulare tumors are highly vascular; therefore, preoperative embolization of the blood supply to the tumor is often performed. Tumor resection can be performed by craniotomy usually using specialized skull base surgery techniques. Craniotomy is a procedure in which a piece of skull is removed, exposing the tumor and allowing tumor removal. Endoscopic endonasal surgery is a minimally invasive technique in which the surgeon operates through the natural corridors of the nose to excise the tumor.
Radiotherapy may be used as an adjuvant treatment or primary treatment and can be administered by stereotactic radiosurgery, a noninvasive method that delivers a highly precise beam of radiation to tumor cells, leaving the surrounding brain tissue, nerves, and blood vessels unaffected.
Even though the tumor resection and/or radiotherapy is often successful, glomus jugulare tumors have a high recurrence rate and may require multiple operations.