What are pheochromocytoma & paraganglioma?

An adrenal tumor can make excess amounts of adrenaline (epinephrine, norepinephrine, and in very rare cases dopamine). These substances are referred to as catecholamines. Patients with adrenal nodules producing excess amounts of catecholamines often present with new/worsening hypertension, headaches, sweating, palpitations (racing heart), flushing, and panic attacks. Blood pressure elevations can be so severe that they can lead to heart attacks, stroke and sudden death. Patients with pheochromocytomas may have a genetic syndrome. All patients diagnosed with a pheochromocytoma who are seen in the University of Michigan Multidisciplinary Endocrine Oncology clinic are also seen by one of our genetic counselors and may be referred for further genetic testing.

Malignant (cancerous) pheochromocytomas occur in approximately 10% of patients. Those with benign pheochromocytomas are usually offered laparoscopic surgery performed through several small incisions, while those with malignant appearing or very large tumors are offered surgery using an open approach performed through a larger incision. Malignant tumors are diagnosed when there is evidence of tumor invasion into surrounding tissue or organs, involvement of lymph nodes, or evidence of distant metastatic disease.

Paragangliomas

Paragangliomas are tumors which are very similar to pheochromocytomas. They produce excess norepinephrine and occur outside the adrenal gland. They may occur anywhere along the side of the spine from the neck to the pelvis. Some catecholamine producing tumors are found inside the bladder and may cause symptoms when patients urinate as the bladder contracts.

Prior to surgery, it is extremely important that the blood pressure be well controlled using certain medications called alpha blockers, beta blockers, and on occasion calcium channel blockers. Alpha blockers (such as dibenzyline) should always be started before beta blockers (such as metoprolol, propranolol, or other medications in the same class) in order to prevent cardiac collapse. Medications to control the blood pressure and heart rate are increased until patients achieve blood pressure goals and also if they have symptoms of nasal stuffiness and slight dizziness upon initially standing. If these goals are not met prior to surgery, the blood pressure may become dangerously high during surgery as the tumor is manipulated and lead to heart attacks, strokes and increased bleeding.