Paraganglioma

A paraganglioma is a tumor that originates from cells called paraganglia found in the middle ear:
*Glomus tympanicum tumors are small sized tumors originating in the middle ear.
*Glomus jugulare arise from paraganglia in or around the jugular bulb, and as they grow they occlude this venous structure.

Treatment for Paraganglioma

Treatment of Glomus Tympanicum Tumors

There are two options in terms of following or treating these lesions. If the symptoms are not too troubling, these lesions can be followed by periodic examinations to determine the growth rate. These tumors may not follow a linear growth pattern. A period of little growth can be followed by rapid growth and vice versa.

SURGERY

The more desirable option is to remove the lesion. The smaller the tumor, the easier it is to remove. Depending on the size of the tumor, an angiogram may be needed prior to surgery. This is a procedure done by a radiologist to place particles within the arterial blood vessels that supply blood to the tumor. This allows for less blood loss during the surgery.

The surgical procedure can be done entirely through the ear canal. Click on the next diagram to see how the surgery is done. The ear canal skin along with the eardrum is elevated, exposing the middle ear. Occasionally, some bone around the chorda tympani nerve needs to be removed to allow better visualization of the tumor. Temporary taste disturbance may occur following surgery.

After tumor removal, the eardrum is laid back down and packing is placed in the ear canal

Step 1: Surgery for removal of a glomus tympanicum is illustrated. For orientation, the ear, ear canal, and eardrum are shown. The next diagram is an enlarged view of the square.

Step 2: The tumor can be seen behind the eardrum. Incisions are made in the ear canal adjacent to the eardrum.

Step 3: The ear canal skin and eardrum are elevated, exposing the middle ear. The chorda tympani nerve can be seen, and the glomus tumor is removed.

Treatment of Glomus Jugulare Tumors

There are three options for management of these lesions. The first involves watching the tumor and using MRI scans to help determine the growth rate. These tumors may not follow a linear growth pattern. A period of little growth can be followed by rapid growth and vice versa.

RADIATION

Radiation therapy is another way to manage these lesions. Radiation is not known to cause actual killing of tumor cells, but rather induces fibrous tissue proliferation around tumor cells in hopes of preventing further growth. There are a number of disadvantages to radiation therapy. First, tumor growth can occur even after having radiation treatment. Surgery becomes more complicated if previous radiation has been used. Radiation causes the loss of the dissection plane between the tumor and the surrounding nerves. Consequently, dissection within this plane may increase the chances of nerve injury. Healing from surgery is also impaired when previous radiation has been given. Radiation has also been known to change a benign glomus tumor into a malignant tumor. Radiation therapy may also cause other tumors to occur within the radiated site. For these reasons, I only recommend radiation therapy when a patient has an actively growing tumor and cannot undergo general anesthesia due to medical reasons.

SURGERY

The third option is to have the tumor surgically removed. The day prior to surgery, the patient undergoes angiography with embolization of the tumor. This is a procedure done by a radiologist to place particles within the arterial blood vessels that supply blood to the tumor. This reduces blood loss during the surgery. Clicking on the adjacent diagram shows the steps of the surgery.

Step 1: Surgery to remove a glomus jugulare tumor is illustrated. The dotted lines indicate some of the incisions around the ear that can be used.

Step 2: The parotid gland, mastoid bone, and surrounding muscles are exposed.

Step 3: A mastoidectomy is performed exposing the semicircular canals, venous sinus, and facial nerve. The tumor is deep to the facial nerve and extends into the middle ear.

Step 4: Here, the adjacent bone is removed. The entire tumor can be visualized.

Step 5: The venous sinus is opened, the tumor is removed, and the internal jugular vein is tied off.

Step 6: Following tumor removal, packing is placed within the venous sinus.

Step 7: Fat harvested from the abdomen is frequently placed to fill the defect and to help prevent spinal fluid leakage.

The surgical approach involves an incision behind the ear extending into the neck. The major blood vessels in the neck are identified, and smaller arterial blood vessels feeding the tumor can also be tied off. A mastoidectomy is performed, exposing the sigmoid sinus and facial nerve. When exposing the jugular bulb, the facial nerve is usually in the way. I have usually been successful in working around the facial nerve without mobilization. Occasionally, mobilization of the nerve is necessary to allow better visualization of the tumor. When the nerve is mobilized, patients usually have weakness of the face immediately following surgery, which recovers over the next several months. With smaller sized tumors, it may not be necessary to mobilize the facial nerve; therefore, the patient will not have any facial nerve weakness following surgery.

The internal jugular vein is tied off in the neck and the sigmoid sinus is packed off in the mastoid. This allows for tumor removal and wide opening of the jugular bulb. The tumor is then dissected from the nerves that control the vocal cord and swallowing mechanism. Occasionally, a spinal fluid leak can occur. This is usually controlled by placing fat from the abdomen into the area of leakage. Sometimes, a spinal drain is placed in the back to allow drainage of spinal fluid over several days. This is an effort to keep the pressure in the spinal fluid space low to allow the area of the jugular bulb to heal and seal off.

Weakness of the vocal cord and swallowing mechanism can occur after surgery. This usually recovers in time. Temporary diet modification might be required. In the extreme case, a tube may need to be placed in the stomach for a few months until the nerves recover.

Treatment of Carotid Body Tumors

Management options, like the other tumors discussed above, include periodic clinical examination, radiation treatment, or surgical excision. Surgery involves excision of the lesion after angiography and embolization. The tumor is then dissected away from the carotid artery.

Multiple tumors

Another reason for angiography is the detection of other glomus tumors in the opposite ear or in the neck that would otherwise go undetected.