Phechromocytomas

Q: What is a pheochromocytomas?

  • A pheochromocytoma is an adrenaline-producing tumor found in center, or medulla, of the adrenal gland. The adrenaline hormones, epinephrine and norepinephrine, are your "flight or fight" hormones.

Q: What is a paraganglioma?

  • A paraganglioma is a rare adrenaline-producing tumor found outside the adrenal gland that grows in the peripheral nervous system. They can either be non-functioning or generally produce only norepinephrine (with one exception). Common places are in the abdomen along the aorta and in the neck along the carotid artery.

Q: What are the symptoms?

  • Common symptoms may include: high blood pressure, either continuous or episodic, palpitations, headaches and sweating.
  • There are many other less common, non-specific symptoms.
  • You may have a tumor that produces small amounts of adrenaline sas not to cause any obvious, overt symptoms.
  • A constellation of symptoms may occur after a procedure or eating certain foods. If this happens, you should be evaluated for a pheochromocytoma.

Q: How is it diagnosed?

  • Diagnosis is made through a series of blood tests, sometimes urine tests, and imaging. There is no role for needle biopsy, and it is contraindicated.

Q: Are there any risk factors?

  • The only known risk factors are certain genetic syndromes and mutations.
  • The most common of these are: Multiple Endocrine Neoplasia Syndrome types 2A and 2B, von Hippel-Lindau Syndrome, Neurofibromatosis 1, Familial Paraganglioma Syndromes (SDH mutations).

Q: What is the treatment?

  • Treatment is surgery. A laparoscopic, minimally-invasive surgery is used for all tumors confined to the adrenal gland.
  • Very large adrenal tumors and those that have extended to adjacent structures, as well as the majority of abdominal paragangliomas, require an open surgical procedure.

Q: Will I be off all my high blood pressure medication after the surgery?

  • Most likely, though not necessarily right away. Sometimes your body needs time tre-regulate itself and your medication may have tbe weaned off more slowly.

Q: Do I need regular follow-up visits?

  • Yes. Pheochromocytomas can come back either in the same area, the other adrenal gland, or other areas in the body.
  • You will have lifelong follow-up with your endocrinologist and possibly your surgeon and yearly blood tests to monitor for recurrence.

Q: Do I need to see a genetic counselor?

  • Current recommendations for referral ta genetic counselor include young age at diagnosis (in your 20s or 30s), bilateral pheochromocytomas, all paragangliomas, family history of one of these tumors, or genetic syndromes.