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Paraganglioma (adrenaline-producing tumor outside the adrenal gland)

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Treatment

Not treating paragangliomaParaganglioma - a disease where too much adrenaline is being made by a tumor outside the adrenal glands is incredibly dangerous and life-threatening. The best treatment for paraganglioma is to surgically remove the tumor(s). This is true for almost all patients except for those with such severe other medical issues that they would not survive an operation. Medical therapy to block the effects of adrenalineAdrenaline - the hormone that helps control the "fight or flight" response and puts the body on "high alert" (also known as epinephrine) excess is available but is not nearly as effective as tumor removal. Patients can "breakthrough" the medication and have life-threatening problems.

Preparing for an operation

Before an operation, the patient must be appropriately prepared with medications such as alpha-blockers that will block the effects of adrenalineAdrenaline - the hormone that helps control the "fight or flight" response and puts the body on "high alert" (also known as epinephrine) during the start of anesthesia and during the operation. This preparation must be carried out under the supervision of a doctor (an endocrinologist, endocrine surgeon, or hypertension specialist) with expertise in managing patients with pheochromocytomaPheochromocytoma - a disease where too much adrenaline is being made by an adrenal tumor/paragangliomaParaganglioma - a disease where too much adrenaline is being made by a tumor outside the adrenal glands. Medication (alpha-blockers) will be given in increasing doses over 10 to 14 days prior to surgery to block the effects of excess adrenaline production. This preparation helps avoid wide swings in blood pressure during surgical handling of the tumor. These alpha blockers will slowly relax blood vessels that have clamped down (i.e. constricted) because of the excess adrenaline. As the alpha blocker dose is increased, the patient should start to have symptoms like a stuffy nose, fatigue, and mild orthostasis (i.e. feeling a little lightheaded when standing up quickly). In fact the specialist will look for these signs to make sure that the alpha blockade is working. Shortly before surgery (one to three days before), ß-blockers may be administered to slow a fast heart rate. Calcium channel blockers, which also relax blood vessels and slow the heart rate, may also be given either instead of or with alpha and beta blockers.

In addition to blocking the effects of adrenaline, it is equally important to fix the dehydration caused by adrenaline excess. As adrenaline causes the blood vessels to tighten, the body tries to keep the blood pressure normal by getting rid of fluid in the body through urine. This means that patients are actually dehydrated. Once the paraganglioma is removed, the blood pressure can be dangerously low if the dehydration is not fixed. As the alpha blockers relax the blood vessels, it is important to restore the lost fluid. During this time, it is important to drink plenty of extra fluid and take a little extra salt in the diet to fill up the relaxed blood vessels and replete the intravascular volume (i.e. overcome dehydration). Eating pretzels, potato chips, pickles and adding a little extra salt to meals will help hold on to the extra liquid the patient is drinking.

Surgery

Surgery is performed under general anesthesia with the patient completely asleep and relaxed. Even with appropriate pre-operative preparation, it is crucial that the anesthesia team and the surgeon are in constant communication. Blood pressure swings, abnormal heart rhythms, and bleeding can occur during adrenalectomyAdrenalectomy - an operation to remove the adrenal gland. Medications to lower and raise blood pressure must be available and given at a moment's notice as needed. For this reason, experienced surgeons often have a special group of anesthesiologists with whom they routinely work on paragangliomaParaganglioma - a disease where too much adrenaline is being made by a tumor outside the adrenal glands cases.

Most paragangliomas are removed using a standard open incision. However, several groups have described successful removal of paraganglioma using a minimally invasive or laparoscopic approach.13-15 The advantages of laparoscopic surgery may include a shorter length of hospitalization, less postoperative pain, and a faster recovery, similar to that for adrenal pheochromocytomaPheochromocytoma - a disease where too much adrenaline is being made by an adrenal tumor. However, if the tumor is locally invasive into surrounding structures or spread to adjacent lymph nodes, then the tumor should be removed through an open operation. See How is adrenalectomy performed?

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