Pheochromocytoma is an important cause of secondary Hypertension. We have adnenal gland located at upper portion of each kidney. It is divided into adrenal cortex and adrenal medulla. Pheochromocytoma is a tumor of Adrenal Medulla. Normal function of adrenal medulla is to produce epinephrine or adrenaline. Which is responsible for controlling blood pressure and to help cope with stressful situations.
So majority of symptoms of pheochromocytoma are due to excess secretion of adrenaline from adrenal medulla. Most patients of pheochromocytoma have recurrent episodes of headache, sweating and a feeling of high anxiety. The following symptoms are listed from the most common to the least common
sweating Episodes (generalized)
heart palpitations (tachycardia and palpitations)
nervousness (feelings of impending death)
Pain in the lower chest or upper abdomen
Nausea (with or without nausea)
These symptoms may come by situations which causes pressure over tumor like physical activity, exercise, defecation, anesthesia, or change in body position.
Who should be examined for Pheochromocytoma
Those who have uncontrolled hypertension
Those who have age between 40 and 60
Those who are already taking 4 antihypertensive drugs but blood pressure is not controllable.
Those who have episodes of above symptoms
Cause of pheochromocytoma is unknown, however some forms of this tumor runs in families like,
a-Multiple endocrine neoplasia, type II (MEN-II). In addition to a pheochromocytoma, people with MEN-II also have thyroid cancer. Other forms of MEN-II include pheochromocytoma with thyroid cancer and hyperparathyroidism (MEN-IIA), and pheochromocytoma with thyroid cancer and tumors of nerves in the eyes lips, mouth and digestive tract (MEN-IIB).
b-Neurofibromatosis 1 (NF1). Pheochromocytomas can occur in a small percentage of people with NF1, a syndrome that includes multiple tumors in the skin (neurofibromas), pigmented skin spots, tumors of the optic nerve of the eye, and bone diseases.
c-Von Hippel-Lindau (VHL) disease. People with this rare multi system disorder are at high risk of pheochromocytoma brain eye and kidney tumors.
Most pheochromocytomas are benign tumors and they don’t spread to other parts of body however some forms do show metastasis (spread) to other parts of body like lungs, bones and brains. Usually only one gland is involved however this tumor can be present in both adrenal glands.
Blood and Urine tests: We perform blood and urine tests to diagnose pheochromocytoma. 24 hr urine collection is tested for epinephrine, norepinephrine and dopamine. This test is called VMA. Idea is just to check level of epinephrine and its metabolites in urine. If these are detected in urine in excess quantity, then tumor is diagnosed.
Now you have diagnosed tumor by blood and urine tests, after that to find out location of tumor, do Ultrasound, CT scan of abdomen.
If high blood pressure remained uncontrolled it may lead to complications of heart failure, infarction, cerebrovascular accident, vision damage and kidney failure.
First treatment is to control high blood pressure with medication like Alpha Blockers and Beta Blockers. When epinephrine is secreted by this tumor it acts on alpha and beta receptors present on heart and blood vessels, result is vasoconstriction and increase heart rate. Now if if we take drugs like alpha blocker and beta blocker, epinephrine will be blocked by these drugs to act on these receptors so result is vasodilation and slow heart rate. Common Alpha blockers are Prazosin (Minipress), Terazosin (Hytrin) Phenoxybenzamine( Dibenzaline). Common Beta Blockers are Atenolol(Tenormin), Carvedilol(Carveda), Metoprolol(Mepressor), Inderal.
Treatment of choice for this tumor is Surgery. After removal of this gland by surgery, blood pressure becomes normal with a day. There are two types of surgeries. General and Laproscopic surgery. However Surgery is not an option for those tumors which show metastasis to other parts of body. For that we use chemotherapy and radiations.
Whenever you have high blood pressure with any symptom, don’t ignore it, you may be the patient of Pheochromocytoma. Always contact your Doctor.
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