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Information about Pheos on the net
If you knew to look for "pheochromocytoma", you are already ahead of the game! Pheos are a neuroendocrine tumor that emits hormones that will eventually cause damage to your heart and cardiovascular system, so they need attention quickly. 80% of pheos occur in or near the adrenal gland. Depending where they are located they may be know by other names: extra-adrenal pheochromocytoma (outside the adrenals), paraganglioma (usually closer to head and neck), carotid body tumor, etc. On this page, we tend to use "pheo" as the umbrella term for all of the above.
30% of pheos are caused by an underlying genetic flaw which might also be putting you at risk for additional health issues, so at some point it will be important to determine if that is the case. Meanwhile, the first order of business is to deal with the pheo.
Pheos may occur sporadically, or they may be associated with genetic
predisposition conditions like VHL or Multiple Endocrine Neoplasia (MEN), Neurofibromatosis (NF) SDHB, SDHC, or SDHD. The leading genetic cause of pheos is von Hippel-Lindau (VHL).
The pheos found in VHL are almost never malignant (less than 3%).
Pheos of other origins have different risks of malignancy.
We are here to help you, no matter what the origin of your pheo. Please feel free to take advantage of our online services, and to request materials. And of course if it turns out that you have VHL, there is a lot more here for you than our pheo information.
Welcome!
It's best to take this one step at a time:
- Is there a pheo somewhere? (chemical tests, usually beginning with plasma free metanephrines and/or 24-hour urine test)
- Where is it? (imaging studies, MIBG, or F-DOPA PET scan)
- Careful planning of the surgery to remove the pheo, including "blocking" the effects of the pheo so that the surgery will go well.
- Quiet recovery to give your body time to "withdraw" from the hormones the pheo has been pumping into your body. Your heart needs time to adjust to the new state of normal.
- Follow-ups with your doctors to assess the genetics of your pheo, and any possible risk that it might have spread. (In VHL, the risk of metastasis is low).
Feel free to ask questions at http://vhl.inspire.com, or to call our hotline.
Here is a list of some of the resources for pheo that you will find here:
- Basic advice in our Handbook. While this is aimed primarily at people with the VHL genetic alteration, it is a good starting point for anyone with a pheo. Determination of whether one should or should not remove the adrenal gland may be different for people with other genetic causes of pheo.
- Radio show on diagnosing pheos, The Powerful Patient (Podcast or listen on net)
- Diagnosing Pheochromocytoma, three presentations at the Boston meeting of the VHL Family Alliance, June 2007 (DVD available, or view on net)
- Learning
about Pheochromocytomas - Report of the First International Symposium
on Pheochromocytoma, Bethesda, Maryland, October 20-23, 2005
- Finding Elusive Pheochromocytomas,
by Graeme Eisenhofer
- Plasma Free Metanephrine and the Laboratory Evaluation for Pheochromocytoma, by David F. Keren, M.D. ** Note that this article stresses the importance of testing people with high blood pressure. Please be aware that in cases of inherited pheos, blood pressure may not be elevated. It may even be low. Any two or more symptoms should indicate the need for this test. Pheo surges may be interpreted as panic attacks, palpitations, or in some cases anger or rage. *** Note too that the use of psychotropic drugs or other drugs, including marijuana and cocaine, may cause a false positive. Be sure to disclose all drug usage to the physician.
- Diagnostic
Efficacy of Unconjugated Plasma Metanephrines for the Detection of Pheochromocytoma
- Pheo
Test Confirmed by a separate research team in Austria
- Establishing
Plasma Metanephrines Testing in your Clinical Laboratory
- Utility of Plasma Free Metanephrines for Detecting Childhood Pheochromocytomas, by M. Weise et al
Looking for a Doctor:
- If you are looking for a doctor who understands pheos, you might want to check out the membership list on the PRESSOR website. There are members all over the world.
- Or contact one of the VHL Clinical Care Centers, all of which treat pheos in connection with VHL. Or contact the VHL Family Alliance to request a referral, 1-800-767-4845, info@vhl.org
Family Stories
- some important meetings are scheduled
- covers all aspects of VHL, including pheochromocytoma. Anyone with a pheo of any origin is welcome here.
- Pheo paratroopers: www.pheoparatroopers.org - is now live
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