Pheochromocytoma- Monday Zebra

Condition- Pheochromocytoma

Pronunciation- fee-oh-crow-moe-sigh-tow-mah

Rarity- Very Rare

Difficulty in Diagnosis- mixed, while there is a test to diagnose this condition, finding a doctor that will know to test for it can be difficult.

     Pheochromocytoma is a rare endocrine tumor that is usually located on the adrenal gland (the adrenal glands are located on the kidneys) and are often referred to as "micro tumors" because they tend to be very small, though their impact can be startling because they release adrenal hormones and can cause dramatic symptoms.

     The symptoms of Pheochromocytoma can mimic many other conditions and for that reason can be very difficult to get a diagnoses because, as the saying goes, when looking for horses no one ever looks for a zebra.  Basically, this means that Physicians will often test for and treat the symptoms of the disease because often the most common cause is the correct one.

     It is only when symptoms intensify and do not respond to conventional treatment that a Physician might consider looking at the more rare conditions... but even then there are so many conditions that cause the same symptoms that it is like trying to find a needle in a haystack of possible ailments.

     The symptoms of Pheochromocytoma are-

• Excessive Sweating
• High blood pressure- uncontrollable high blood pressure and hypertensive crisis
• Rapid heart rate- also known as tachycardia
• Forceful heartbeat
• Profound sweating
• Abdominal pain
• Sudden-onset headaches - usually severe and of varying duration
• Anxiety
• Sudden uncontrollable rage
• Feeling of extreme fright
• Pale skin
• Weight loss

     If a pheochromocytoma tumor is suspected the first test that will be given is a 24 hour urine test which will check for certain hormones released by the adrenal glands.   If this test comes back elevated the next test to be done would be a specialized scan to locate the tumors.  The most common place for them is on the adrenal glands, but they are not ALWAYS located there and because of the size of them (as stated before, they are micro-tumors for the most part) they can be very hard to find.

     The only 100% reliable treatment for a pheochromocytoma tumor is to remove it, because it will constantly release hormones and the symptoms can be very hard to manage with traditional medications or can be impossible to manage.

     Surgery to remove these tumors must be done with great care by a specialist because anesthesia issues can be common and blood pressure can go into hypertensive crisis even under anesthesia.

     There are many good websites for more information about this condition such as the one by the Mayo Clinic where you can find out more... but here are some interesting facts about this disease.

• Most people are diagnosed with a pheochromocytoma between the ages of 30 and 50.  Though it can happen at any age it is even more rare in those younger than 30 years of age.
• Most pheochromocytoma tumors are actually found in autopsy!
• Eating foods or drinking beverages with vanilla in them can affect the 24 hour urine test!
• Most pheochromocytoma are benign
• It can be hereditary 
• There could be a single tumor on one adrenal gland, one on both or they could actually be anywhere in the body but will still release adrenal hormones no matter where they are.

     Thus ends the first of what I hope to be many "Monday Zebra" posts about Rare and/or Incurable Conditions.  Keep an eye out every Monday as I highlight another condition to help spread the word about the world of rare conditions out there.

     If you have or know someone that has a Rare and/or Incurable Condition that you would like to see featured, please contact me and I will add it to the list of conditions that will be brought forth into the Snarky light every Monday.