Paragangliomas

Paragangliomas are tumors that originate from paraganglionic tissue which is found in various locations. They are more commonly found in women and are believed to occur at a rate of about 1 in 30,000. The majority of paraganglionic tissue in the human body is found in the neck, at an area called the carotid body. This is the point where the carotid artery divides. Paraganglionic tissue is also located in or around the middle ear or the base of the skull. Symptoms may vary, depending on where the tumor occurs.

When paragangliomas occur at the carotid body, the most common place for these types of tumors to develop, they are usually benign and become malignant in about 10% of the cases. Multiple tumors may also develop. The carotid body is responsible for sensing blood oxygen level and alerting the heart and lungs to increase heart and respiratory rates when the bloodstream contains insufficient oxygen. Patients with these types of tumors may develop a slow-growing, firm mass in the neck which can be moved from side to side but not vertically.

These types of tumors may result in pain and ringing in the ear, as well as dizziness or hearing loss.

Paragangliomas that result from the middle ear area are known as glomus tympanicum. These types of tumors may result in pain and ringing in the ear, as well as dizziness or hearing loss. Glomus jugulare tumors, those resulting from the jugular bulb at the base of the skull, can also result in ringing in the ear as well as deafness or facial numbness. Difficulty swallowing and hoarseness may also occur.

Other symptoms that may be noted due to paragangliomas include high blood pressure, anxiety, palpitations, pain in the face or tongue, and headache.

Symptoms generally develop when paraganglioma grow large enough to apply pressure on surrounding areas, including nerves. Surgery is often recommended to remove the tumors. However, because the tumors involved have a large blood supply, surgery can be difficult. The patient may be at risk of significant blood loss and injury to the cranial nerves.1 Embolization is often performed several days prior to surgery in an effort to block off the flow of blood to the tumor.

Embolization is a process that is similar to an angiogram. A catheter or tube is placed into the blood vessels that supply the tumor via the leg. Material is injected into the vessels to block off the supply of blood. The combination of embolization and surgery provides a highly effective method for treating paragangliomas.2

The embolization procedure from start to finish
Prior to undergoing embolization, patients are expected to sign a consent form and will have the opportunity to have questions answered. The anesthesiologist will administer medications and fluids through the use of an intravenous line (IV) into a vein in the hand or arm. He or she will also administer general anesthesia so that the patient will be completely asleep for the procedure. Blood pressure will be monitored through an A-Line, a thin flexible tube or catheter placed in an artery in your wrist. An electrocardiogram (EKG) will monitor heart rate and rhythm as well as oxygen levels. A specific type of catheter known as a Foley will be placed in the bladder to allow urine to drain.

Once the procedure is complete, the patient will be transferred from the operating room to either the Surgical Intensive Care Unit (SICU) or the Post-Anesthesia Care Unit (PACU). Here, a heart monitor will closely monitor vital signs. This machine is very sensitive and often sounds inadvertently; this is not cause for alarm. The nursing staff will assist in changing positions, but bed rest is required. Eventually, the patient can look forward to a normal diet and transfer to a room on a surgical floor. The Foley catheter will also be removed prior to leaving the bed for the first time.

Patients are expected to keep the leg that underwent surgery straight for several hours and a tube may remain in place at the puncture sight for several days. Once it is removed, the physician will apply pressure for about 20 minutes to prevent bleeding, and a device may be used to seal the puncture.