The moderators of this support board have created a Frequently Asked Questions so that important guidelines in the diagnosis and treatment of pheochromocytoma
are better understood. Because of the need for consistency in information and instructions, this topic will be 'read only'.
1. What is pheochromocytoma?
Pheochromocytoma is a neuroendocrine tumor of the medulla of the adrenal glands. It originates in the chromaffin cells and secretes excessive amounts of catecholamines, usually adrenaline and noradrenaline.
2 I have many of the symptoms of pheochromocytoma. Does this mean that
I have this illness?
No, it doesn't. The majority of persons who visit this site find that they do not have pheochromocytoma. There are a number of other illnesses which have the same symptoms as pheochromocytoma. You need to have your doctor order a Plasma Free Metanephrine blood test and/or urine tests as the first steps in obtaining a confirmed diagnosis.
Importantly, it is impossible to diagnose yourself. If tests confirm you do have pheochromocytoma, you need the medical attention of the physicians who are knowledgeable in treating this illness.
3. What are the actual symptoms of pheochromocytoma?
Symptoms vary greatly with each patient. Stereotype textbook cases of Pheochromocytoma are rare. Symptoms can include:
Headaches
Nausea
Vomiting
Weight loss or gain
Hypertension
Hyperglycemia
Diabetes
Diabetes-like symptoms
Palpitations
Angina Chest Pain
Clammy skin Cold skin
Anxiety
Nervousness
Panic
Feeling of impending doom
Rapid pulse
Rapid breathing
Breathing difficulty
Vision disturbance
Impaired vision
Orthostatic hypotension
Fainting
Sweating
Flushing
Abdominal pain
Flank pain
Constipation
Paresthesia (tingling, prickling, numbness or burning sensations)
4. Where are pheochromocytoma found?
Pheochromocytoma can basically be found wherever chromaffin tissue can be found. Pheochromocytoma can be located virtually anywhere in the human body.
5. I've read that some pheochromocytoma are inherited, but others
are spontaneous. How can this be possible?
According to data, from 10% to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1, SDHB and SDHD are all known to cause familial pheochromocytoma or extra-adrenal paraganglioma.
6. Is there any difference between a paraganglioma and a
pheochromocytoma?
Extra-adrenal paragangliomas, which are often described as extra-adrenal pheochromocytomas are closely related, though less common. These tumors originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin. Bilateral disease is present in approximately 10% of patients.
7. What are the advantages of the Plasma Free Metanephrine blood test
over the standard urine tests for pheochromocytoma?
There is considerable data which shows that the Plasma Free Metanephrines test is more reliable than urinary catecholamines (24-hour urine). Another consideration is practicality, in that a urinary collection can be difficult for some patients, for instance in the case of children.
8. Should the urine tests still be performed even though the Plasma
Free Metanephrine has been done?
Urine tests are conclusive for diagnosis in some cases of pheochromocytoma. Often, doctors prefer to do both the blood test and urine test for the purpose of thoroughness and comparison.
9. If I have pheochromocytoma, does this automatically mean that I have
cancer?
Most pheochromocytomas are benign. According to data, roughly 10% of pheochromocytoma cases are metastatic. It is considered cancerous when the diagnosis of metastatic is confirmed.
Updated Cited Sources:
http://www.endocrineweb.com/tenpercent.html
http://www.nichd.nih.gov/...ics/pheochromocytoma.cfm
http://www.cancerhelp.org...stions/phaeochromocytoma
http://www.pheochromocytoma.org/sys-tmpl/door/
This article dated 3/17/2010 states in about 30% of the cases the tumors are cancerous:
http://www.nichd.nih.gov/...ics/pheochromocytoma.cfm
10. Why are there so many different types of tests for
pheochromocytoma, and which ones should I have performed?
If your doctor is suspicious of pheochromocytoma, he can order the standard tests that are mentioned previously. The 24 hour urine tests for catecholamines, metanephrines and VMA are fairly standard in the beginning stages of a diagnosis. These must be performed correctly: the urine specimen must be kept refrigerated during collection, and an acid preservative added to the collection bottle. In some cases, unless an individual has an episode during collection, the urine test could be negative, even with a pheochromocytoma present. There is a blood test available which tests Plasma Metanephrines and Catecholamines, which has been approved for diagnosis of pheochromocytoma. Some pheochromocytoma researchers believe that because of the high sensitivity of the plasma free metanephrines test and the low incidence of the tumor, that plasma free metanephrines should be carried out as the first test. If that is not available, then the next best test is urinary fractionated metanephrines - but not urinary TOTAL metanephrines. Both tests may be combined with measurements of plasma or urinary catecholamines.
Flowchart for Diagnosis of Adrenal Mass
11. How is Pheochromocytoma treated?
Once a diagnosis of pheochromocytoma has been established and the tumor located, surgical removal of the tumor is standard treatment if possible. This is not always as simple as it sounds. Some patients with pheochromocytoma find that it is difficult for the location of the tumor to be discovered. There are also cases of "quiet" pheochromocytomas which take longer to find and remove. For pheochromocytoma surgery, it is vital to ask your surgeon how many patients he or she has operated on specifically for this type of tumor. An experienced surgeon will do no less than 3 to 4 pheochromocytoma surgeries in a year.
12. What makes the plasma metanephrine test different?
First, the free metanephrines are produced continuously within pheochromocytoma tumor cells from catecholamines leaking from storage vesicles within the cell cytoplasm. Thus, these metabolites are produced independently of catecholamine released by tumors. This has advantages since catecholamine release can be episodic or relatively non-existent in some pheochromocytomas. Second, metanephrines commonly measured in urine are different metabolites. They are actually sulfate conjugated, require an additional metabolic step for synthesis and are consequently produced in different parts of the body than the free metanephrines. Third, the advantages of a plasma test over a urine test outweigh the disadvantages. Most urine tests have a lesser accuracy rate, whereas the plasma metanephrine test has a much higher accuracy rate.
13. What is the accuracy of the plasma free metanephrines test? Where
can I have this test done?
Testing results indicate a sensitivity of 99% compared to 63% for VMA and 83% to 85% for urinary or plasma catecholamines.
Also see: Commercial Labs now offering Plasma Free Met Test
14. What substances interfere with the results of 24 hour urine
tests?
Many drugs can potentially interfere with assays of catecholamines and any other substances, but this often depends on the method and laboratory. One facility can have machines and equipment that vary from that being used by another facility. Also, whether it is a problem or not depends on the level of expertise and competency of the operator running the assay. Caffeine, vanilla, vanilla extract, and bananas are known problems for urine testing. Listing these interferences is best left up to the individual laboratory responsible for the test.
Also see: Substances that interfere with biochemical tests for pheo
15. What substances interfere with the plasma metanephrine
test?
Specifically, caffeine is a problem both directly and indirectly. Acetaminophen or any drugs containing acetaminophen can be a problem. No other medicinal source of interference has been identified. Clonidine or its metabolites show up in the chromatograms but they aren't a problem. Again, listing these interferences is best left up to the individual laboratory responsible for the test being performed. If the patient is not given a list of known interferences, be sure to insist on having a copy.
Also see: Substances that interfere with biochemical tests for pheo
Also see: What are the normal ranges for the 24 hr urine test?
16. What if the results are negative in the 24 hour catecholamine urine
test?
It is still recommended that the plasma metanephrine test be performed to confirm or eliminate the diagnosis of pheochromocytoma, particularly if the symptoms are episodic. The 24 hour urine tests are not always successful at picking up intermittently secreting tumors.
Again: Some pheochromocytoma researchers believe that because of the high sensitivity of the plasma free metanephrines test and the low incidence of the tumor, that plasma free metanephrines should be carried out as the first test. If that is not available, then the next best test is urinary fractionated metanephrines - but not urinary TOTAL metanephrines. Both tests may be combined with measurements of plasma or urinary catecholamines.
17. What if the results are negative in the Plasma Free Metanephrine
test?
If the test has been done properly, then it is unlikely that you have pheochromocytoma. You and your doctor should focus on trying to find what is actually causing your health problems. There are other serious conditions which have the same symptoms as pheochromocytoma.
18. What other illnesses have similar symptoms to
pheochromocytoma?
List of Conditions with Symptoms Similar to Pheochromocytoma
Also see: Other Diseases with Similar Symptoms As Pheo
Also see: Conditions That Increase Catecholamines
Also see: What Thyroid Imbalances Can Do
19. My doctor has ordered a Clonidine Suppression Test. Where can I
find information about this?
Clonidine Suppression Test
20. What are the possible treatments for pheochromocytoma and
metastatic pheochromocytoma?
Possible Treatments for Pheo and Metastatic Pheo
1. What is pheochromocytoma?
Pheochromocytoma is a neuroendocrine tumor of the medulla of the adrenal glands. It originates in the chromaffin cells and secretes excessive amounts of catecholamines, usually adrenaline and noradrenaline.
2 I have many of the symptoms of pheochromocytoma. Does this mean that
I have this illness?
No, it doesn't. The majority of persons who visit this site find that they do not have pheochromocytoma. There are a number of other illnesses which have the same symptoms as pheochromocytoma. You need to have your doctor order a Plasma Free Metanephrine blood test and/or urine tests as the first steps in obtaining a confirmed diagnosis.
Importantly, it is impossible to diagnose yourself. If tests confirm you do have pheochromocytoma, you need the medical attention of the physicians who are knowledgeable in treating this illness.
3. What are the actual symptoms of pheochromocytoma?
Symptoms vary greatly with each patient. Stereotype textbook cases of Pheochromocytoma are rare. Symptoms can include:
Headaches
Nausea
Vomiting
Weight loss or gain
Hypertension
Hyperglycemia
Diabetes
Diabetes-like symptoms
Palpitations
Angina Chest Pain
Clammy skin Cold skin
Anxiety
Nervousness
Panic
Feeling of impending doom
Rapid pulse
Rapid breathing
Breathing difficulty
Vision disturbance
Impaired vision
Orthostatic hypotension
Fainting
Sweating
Flushing
Abdominal pain
Flank pain
Constipation
Paresthesia (tingling, prickling, numbness or burning sensations)
4. Where are pheochromocytoma found?
Pheochromocytoma can basically be found wherever chromaffin tissue can be found. Pheochromocytoma can be located virtually anywhere in the human body.
5. I've read that some pheochromocytoma are inherited, but others
are spontaneous. How can this be possible?
According to data, from 10% to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1, SDHB and SDHD are all known to cause familial pheochromocytoma or extra-adrenal paraganglioma.
6. Is there any difference between a paraganglioma and a
pheochromocytoma?
Extra-adrenal paragangliomas, which are often described as extra-adrenal pheochromocytomas are closely related, though less common. These tumors originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin. Bilateral disease is present in approximately 10% of patients.
7. What are the advantages of the Plasma Free Metanephrine blood test
over the standard urine tests for pheochromocytoma?
There is considerable data which shows that the Plasma Free Metanephrines test is more reliable than urinary catecholamines (24-hour urine). Another consideration is practicality, in that a urinary collection can be difficult for some patients, for instance in the case of children.
8. Should the urine tests still be performed even though the Plasma
Free Metanephrine has been done?
Urine tests are conclusive for diagnosis in some cases of pheochromocytoma. Often, doctors prefer to do both the blood test and urine test for the purpose of thoroughness and comparison.
9. If I have pheochromocytoma, does this automatically mean that I have
cancer?
Most pheochromocytomas are benign. According to data, roughly 10% of pheochromocytoma cases are metastatic. It is considered cancerous when the diagnosis of metastatic is confirmed.
Updated Cited Sources:
http://www.endocrineweb.com/tenpercent.html
http://www.nichd.nih.gov/...ics/pheochromocytoma.cfm
http://www.cancerhelp.org...stions/phaeochromocytoma
http://www.pheochromocytoma.org/sys-tmpl/door/
This article dated 3/17/2010 states in about 30% of the cases the tumors are cancerous:
http://www.nichd.nih.gov/...ics/pheochromocytoma.cfm
10. Why are there so many different types of tests for
pheochromocytoma, and which ones should I have performed?
If your doctor is suspicious of pheochromocytoma, he can order the standard tests that are mentioned previously. The 24 hour urine tests for catecholamines, metanephrines and VMA are fairly standard in the beginning stages of a diagnosis. These must be performed correctly: the urine specimen must be kept refrigerated during collection, and an acid preservative added to the collection bottle. In some cases, unless an individual has an episode during collection, the urine test could be negative, even with a pheochromocytoma present. There is a blood test available which tests Plasma Metanephrines and Catecholamines, which has been approved for diagnosis of pheochromocytoma. Some pheochromocytoma researchers believe that because of the high sensitivity of the plasma free metanephrines test and the low incidence of the tumor, that plasma free metanephrines should be carried out as the first test. If that is not available, then the next best test is urinary fractionated metanephrines - but not urinary TOTAL metanephrines. Both tests may be combined with measurements of plasma or urinary catecholamines.
Flowchart for Diagnosis of Adrenal Mass
11. How is Pheochromocytoma treated?
Once a diagnosis of pheochromocytoma has been established and the tumor located, surgical removal of the tumor is standard treatment if possible. This is not always as simple as it sounds. Some patients with pheochromocytoma find that it is difficult for the location of the tumor to be discovered. There are also cases of "quiet" pheochromocytomas which take longer to find and remove. For pheochromocytoma surgery, it is vital to ask your surgeon how many patients he or she has operated on specifically for this type of tumor. An experienced surgeon will do no less than 3 to 4 pheochromocytoma surgeries in a year.
12. What makes the plasma metanephrine test different?
First, the free metanephrines are produced continuously within pheochromocytoma tumor cells from catecholamines leaking from storage vesicles within the cell cytoplasm. Thus, these metabolites are produced independently of catecholamine released by tumors. This has advantages since catecholamine release can be episodic or relatively non-existent in some pheochromocytomas. Second, metanephrines commonly measured in urine are different metabolites. They are actually sulfate conjugated, require an additional metabolic step for synthesis and are consequently produced in different parts of the body than the free metanephrines. Third, the advantages of a plasma test over a urine test outweigh the disadvantages. Most urine tests have a lesser accuracy rate, whereas the plasma metanephrine test has a much higher accuracy rate.
13. What is the accuracy of the plasma free metanephrines test? Where
can I have this test done?
Testing results indicate a sensitivity of 99% compared to 63% for VMA and 83% to 85% for urinary or plasma catecholamines.
Also see: Commercial Labs now offering Plasma Free Met Test
14. What substances interfere with the results of 24 hour urine
tests?
Many drugs can potentially interfere with assays of catecholamines and any other substances, but this often depends on the method and laboratory. One facility can have machines and equipment that vary from that being used by another facility. Also, whether it is a problem or not depends on the level of expertise and competency of the operator running the assay. Caffeine, vanilla, vanilla extract, and bananas are known problems for urine testing. Listing these interferences is best left up to the individual laboratory responsible for the test.
Also see: Substances that interfere with biochemical tests for pheo
15. What substances interfere with the plasma metanephrine
test?
Specifically, caffeine is a problem both directly and indirectly. Acetaminophen or any drugs containing acetaminophen can be a problem. No other medicinal source of interference has been identified. Clonidine or its metabolites show up in the chromatograms but they aren't a problem. Again, listing these interferences is best left up to the individual laboratory responsible for the test being performed. If the patient is not given a list of known interferences, be sure to insist on having a copy.
Also see: Substances that interfere with biochemical tests for pheo
Also see: What are the normal ranges for the 24 hr urine test?
16. What if the results are negative in the 24 hour catecholamine urine
test?
It is still recommended that the plasma metanephrine test be performed to confirm or eliminate the diagnosis of pheochromocytoma, particularly if the symptoms are episodic. The 24 hour urine tests are not always successful at picking up intermittently secreting tumors.
Again: Some pheochromocytoma researchers believe that because of the high sensitivity of the plasma free metanephrines test and the low incidence of the tumor, that plasma free metanephrines should be carried out as the first test. If that is not available, then the next best test is urinary fractionated metanephrines - but not urinary TOTAL metanephrines. Both tests may be combined with measurements of plasma or urinary catecholamines.
17. What if the results are negative in the Plasma Free Metanephrine
test?
If the test has been done properly, then it is unlikely that you have pheochromocytoma. You and your doctor should focus on trying to find what is actually causing your health problems. There are other serious conditions which have the same symptoms as pheochromocytoma.
18. What other illnesses have similar symptoms to
pheochromocytoma?
List of Conditions with Symptoms Similar to Pheochromocytoma
Also see: Other Diseases with Similar Symptoms As Pheo
Also see: Conditions That Increase Catecholamines
Also see: What Thyroid Imbalances Can Do
19. My doctor has ordered a Clonidine Suppression Test. Where can I
find information about this?
Clonidine Suppression Test
20. What are the possible treatments for pheochromocytoma and
metastatic pheochromocytoma?
Possible Treatments for Pheo and Metastatic Pheo
