30-40% of pheos and paras are here-ditary. There are more than a
dozengenes that have been linked to pheopara. The most common are
SDHA,SDHB, SDHC, SDHD, VHL, NF1 & RET.More information about
each of thesegenes can be found in the geneticsbrochure and at
A GUIDE TO
All educational information is vetted by our MedicalAdvisory
Board, We also utilize current guidelines andrelevant research. For
more detailed info go topheopara.org. Printed 3/2020.
There is no test to determine if a pheoor para is malignant. The
disease iscancerous if it has spread to other partsof the body.
This happens in 15-25% ofcases. It is more common for parasthan for
pheos to become malignant,except for paras in the head and neck.The
likelihood that a pheo or parabecome malignant also depends onthe
type of genetic mutation, if one ispresent.
A pheochromocytoma (fee-o- kroe-moe-sy-TOE- muh) is a rare,
usually non-cancerous (benign), slow-growingneuroendocrine tumor
that develops incells in the center of an adrenal
gland, called the adrenal medulla. These twoadrenal glands,
one located above eachkidney, produce hormones
calledcatecholamines. Pheochromocytoma(pheo) typically produce an
excessamount of catecholamine hormones,which include
norepinephrine(noradrenaline), epinephrine(adrenaline), &
dopamine. Thesehormones produce the "fight or flight"response.
PHEOCHROMOCYTOMA & PARAGANGLIOMA
If detected early, pheo
para can be successfully
treated in the majority
The illness can be referred toas pheochromocytoma
(pheo), paraganglioma (para), pheo para or PPGL
Special Thanks to...
Pheo Para Alliance
pheopara.org / [email protected] / @pheopara
Pheo ParaAlliance MedicalAdvisory Board
what is pheo?
Pheos form in theadrenal glandslocated above
30-40% of pheos and parasare hereditary. If you are
diagnosed talk to your doctorabout genetic testing.
about 2 to 8 per 1 millionpeople are diagnosed each
year with pheo para
If you have a genetic mutation, yourchildren have a 50% chance
of inher-iting it. Even if your children inherit thegene, that
doesn’t mean that they willdevelop a tumor, but regular monitor-ing
is important. Regular screening cancatch tumors before you
connecting with others who
have pheo para can be
what is para?Paraganglioma (păr′ə-găng′glē-ō′mə) orpara is a
rare, usually noncancerous(benign), slow-growing tumor that
isclosely related to pheo, but even morerare. Paras are
extra-adrenal whichmeans they form outside of the adrenalglands, in
the sympathetic andparasympathetic nervous system.
Although it is rare, some para do notproduce catecholamines so
symptomsmay not occur. These non-secretingparas are often found
incidentally fromimaging for another reason.
Symptoms of pheo para:High blood pressureRapid or forceful
heartbeatProfound sweating Severe headacheTremorsPaleness in the
faceShortness of breathSensation of panic or doom AnxietyAbdominal
pain / ConstipationWeight loss
Pheo para can occur at any age, butmost commonly affect people
betweenthe ages of 20 and 50. If you experiencethe symptoms below,
you should see adoctor to get an accurate diagnosis, asmany of
these symptoms can becaused by other conditions. The release of
catecholamines frompheos and paras can cause persistent orepisodic
high blood pressure, head-ache, sweating and other symptoms. Ifleft
untreated, life-threatening damage,especially to the cardiovascular
DIAGNOSISIf pheo para is suspected, you should seean
endocrinologist, (a doctor whospecializes in hormonal disorders)
whohas pheo para experience. The twenty-four hour urine and blood
(plasma) testsare used if pheo or para is suspected.
Imaging, taking pictures of the inside ofthe body, is often used
once bio-chemical tests indicate a pheo or para.Imaging will help
to identify where, howmany, and size of the tumor(s).
CT/MRIare often used, but functional imaginginvolving the use of a
radioactivesubstance to take pictures of the bodymay also be
TREATMENTIf possible, the treatment of choice issurgery to
remove the tumor(s).Surgical treatment usually returnsblood
pressure to normal andalleviates symptoms. Alternatively,
anexperienced doctor may suggest onlyregular monitoring of the
tumor(s) ifthey are not secreting catecholamines,there are no
symptoms and thetumor(s) are stable (not growing). Radiation,
chemotherapy orradionuclide therapy may be used ifsurgery is not
possible. More informat-ion about these treatments can befound at
pheopara.org. Before undergoing surgery, patientsmust be
adequately “blocked” withmedication. Anesthesia andmanipulation of
the tumor(s) cancause the release of catecholaminesduring surgery,
which may result in ahypertensive crisis and even death. Thisis why
it is usually not recommendedfor patients to have a biopsy of
thetumor if pheo para is suspected.
Para commonly format the base of theskull, neck, chest,
abdomen, and pelvis.
It is critical that patients are
"blocked" with medication
before undergoing surgery.
A urine test or plasma test should berepeated 4-8 weeks after
surgery tocheck for any remaining disease. Urineor plasma tests
should be performedannually to detect remaining disease,return of
the disease, or thedevelopment of metastases.
Pheo is often referred to as the “greatmimic”. Receiving a
diagnosis anddistinguishing pheo para from otherconditions can be a
challenge. In arecent survey, 35% of respondentsindicated it
took 4+ years to receive adiagnosis.