Definition

Paragangliomas arise from neural crest tissue at locations of normal paraganglia. These vascular, typically benign tumors are most commonly located in the ear cavity, at the skull base, or in the upper neck. Also known as glomus tumors, chemodectomas or nonchromaffin paragangliomas, these slow growing, highly vascular tumors carry names based on their location.

  • Carotid paraganglioma is the most common head and neck paraganglioma.
  • Glomus tympanicum tumors originate in the middle ear.
  • Glomus jugulare arise from paraganglia in or around the jugular bulb. As they grow, they occlude this venous structure. These tumors as well as the glomus tympanicum affect the middle ear. They occur in women more than in men and are most common in women aged 40 to 60 years.
  • Vagal paraganglioma is least common of head and neck paragangliomas.

Left: Lateral image of a large, left-sided paraganglioma involving the skull base
Center: Lateral image of the same paraganglioma after embolization; note the decreased blood supply
Right: Axial MRI image of a large, left-sided glomus tumor, post-embolization

Causes

  • Inherited condition: Familial paragangliomas account for approximately 25 percent of cases.
  • Mutation within the genetic makeup of a paraganglia cell causes uncontrolled growth that eventually leads to a tumor. The cause for the mutation remains unclear.
  • No underlying inherited condition: the tumor is considered “sporadic” or random.
  • Prolonged living at high altitude is among the known causes.

Risk Factors

Risk factors associated with an increased chance of developing paraganglioma are:

  • Having other family members with a paraganglioma
  • Living at high altitudes
  • Female gender

Paragangliomas grow slowly and rarely metastasize, but their local growth may result in marked neurological deficits. Sometimes symptoms do not appear until the mass is too large for excision.

Symptoms

Paragangliomas are not painful. They usually manifest with an effect on an adjacent structure. For example, hearing loss is an early common complaint. They are usually nonsecretory but occasionally produce and secrete proteins into the bloodstream that can cause symptoms such as high blood pressure, rapid heart rate, tremor, sweating and headache.

Glomus jugulare and glomus tympanicum tumors

  • Middle ear mass
  • Pulsatile tinnitus or ringing of the ears that is heard with each heartbeat
  • Conductive hearing loss if tumor impairs normal vibration of ossicles or bones behind the ears
  • Ear pain
  • Dizziness
  • Swallowing difficulty or vocal problem (hoarseness)
  • Partial tongue or facial weakness or paralysis\
  • Neural hearing loss and dizziness only if the tumor has invaded the inner ear

Carotid paraganglioma

  • Painless neck mass
  • Cranial nerve palsies, usually of the vagus nerve and hypoglossal nerve

Diagnosis

  • Careful examination of the ear drum
  • Hearing tests
  • CT, MR, angiography
  • Blood and urine tests to determine if the tumor is secreting adrenaline-like hormones

Treatment

The patient and physician consider a number of factors when deciding on the treatment that include severity of symptoms, size and location of the tumor, presence of cranial nerve deficits, age and health of the patient. No medication or chemotherapy is effective against these tumors.

  • Observation: watching the tumor and using MRI and/or CT scans to monitor growth rate
  • Tumors are embolized (selective occlusion of blood vessels that feed the tumor) preoperatively to decrease blood loss
  • Surgical excision after embolization of the tumor with the goal of complete removal
  • Radiation therapy which does not remove or destroy the tumor but aims to stop growth

Prevention

There is no known way to prevent paragangliomas.

If you want to learn more about treatment for paragangliomas, call the Mount Sinai Department of Neurosurgery at 212-241-2377.

Disclaimer

Content generated and provided by Joshua B. Bederson, MD, Professor and Chairman of the Department of Neurosurgery at The Mount Sinai Health System. Some of the information contained in this article was adapted from the National Institutes of Health. Last reviewed June 2011 by the Department of Neurosurgery at The Mount Sinai Health System.

Images used with permission from Joshua B. Bederson and Aman Patel, MD.