Pheochromocytoma
A pheochromocytoma is a tumor that occurs in the adrenal medulla, which produces catecholamines (adrenaline and noradrenaline), the so-called “stress hormones” that increase alertness, strength and speed in an emergency situation. In people with pheochromocytoma, the medulla produces excess catecholamines.
Symptoms
A pheochromocytoma may have no symptoms at all, or present with classic symptoms that resemble what one experiences in a “fight or flight” situation:
- High blood pressure, either persistent or intermittent
- Sweating
- Headache
- Rapid or irregular heartbeats
- Feelings of anxiety, panic, fear
- Pallor (paleness)
- Dizziness/lightheadedness with standing
- Tremor
- Weight loss
Approximately 15% of pheochromocytomas occur as part of a genetic syndrome. Pheochromocytoma can be related to Multiple Endocrine Neoplasia type 2 (MEN2), von Hippel-Lindau Syndrome (VHL) and Neurofibromatosis type 1(NF1) and familial pheochromocytoma/ paraganglioma. Most pheochromocytomas are sporadic, meaning they have no known genetic basis.
Treatment
Pheochromocytoma is usually treated with surgery, which may require removal of the entire affected adrenal gland. In some cases, the surgeon may be able to spare the cortex (outer layer) by removing only the tumor and the adrenal medulla. A laparoscopic approach using smaller incisions and miniature instruments may be possible with some carefully selected patients. If both adrenal glands are removed (bilateral pheochromocytoma), lifelong treatment with hormones is required. Screening for pheochromocytoma should be done prior to any elective surgery, pregnancy and childbirth.
Individuals who have had both adrenal glands removed are encouraged to wear a medical alert bracelet stating that they have had bilateral adrenalectomy and are at risk for adrenal crisis, which is caused by insufficient amounts of cortisol hormone. An adrenal crisis is life-threatening, typically occurring during times of stress or trauma. Medical alert bracelets can be purchased at most jewelers or through the MedicAlert® website.
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