Happy Holidays!
For a serious diagnosis like pheo, repeat testing of pheo markers is generally a good idea. Pheo test results from the same patient even within a few days or weeks can be quite different at times. Patients having acute illness, anxiety attack, or any emotional or physical stress would have higher pheo marker levels than when they are healthy and restful. The position (sitting or lying) of patients getting blood draw matters. Random variations are always possible. Laboratory errors do happen as well. If the two test results are concordant (which fortunately are more common), we can confidently say whether the patient has normal or abnormal pheo marker levels. If, however, the two test results are discordant (one abnormal and one normal, or one very high and one only slightly high), what shall we do?
There are several factors to consider. First, we need to see the quantitative differences between the two results. If one result is in the high normal range and the other slightly abnormal, they are essentially similar and concordant results. Second, we need to delve into the details on the patient’s mental and physical condition when the tests are done. If the patient is seriously ill when one test is taken, the result is likely higher and does not necessarily suggest pheo. Third, we need to ask ourselves the likelihood of this patient’s having pheo is high or not, based on the grounds why we test for pheo in the first place. Of course the more suspicion we have, the more likely the abnormal result is true. Fourth, past research has shown that if there are no laboratory errors, a single normal test result rules out pheo in most patients. Thus a negative pheo test result should be given more weight in general. Last, when we are really not sure, imaging is needed to give us more information.
Dr. Pheo
Hello,
ReplyDeleteI have MEN2A. I'm always nervous and sometimes I have anxiety.
I have the following results with 24h urine test:
Normetanephrine: 407 , ref range 88-444
Metanephrine: 245, ref range 85-345
All the other values are normal, but I'm afraid because I did very low urine in the last spot (second morning) because I was nervous. I'm thinking that if I had expelled the urine normally, the normetanephine value would be above the uppercut value.
My doctor says its all ok but I'm worried about this
Dear Anonymous,
DeleteI agree with your doctor that you probably don't have an active pheo. In people with MEN2A or 2B, the metanephrine level (more important than normetaneprhine) is the one to watch for.
Dr. Pheo
Thank you Dr Pheo,
ReplyDeleteSometimes I have 135/85 (rarely 140/9) of blood pressure when I'm nervous because I'm studing and working at the same time. I don't know if this is an indicator or is only anxiety. Would you repeat the test?
Dear Anonymous,
DeleteThis does not indicate pheo or anxiety. It is normal to have higher blood pressure when stressed.
Dr. Pheo
Hello another time,
ReplyDeleteI'm the same person who wrote above that has MEN2A and:
Normetanephrine: 407 , ref range 88-444
Metanephrine: 245, ref range 85-345
But the last urine, in the second morning was too little, in example if we spend 30 seconds, i urine in 5 seconds due the anxiety of the test, I supose.
My NMN levels are in the high upper limit. If I had urine all the specimen the second morning, I had the NMN above the upper limit, moreover the second urine represents 8 hour (sleeping).
You said that you agree with my doctor, but having MEN2A and this values of NMN, I'm worried.
Would you repeet the test?
thanks.
Dear Anonymous,
DeleteThe urine metanephrines should be interpreted after correction by creatinine. If you do that, slightly less urine should not make a big difference. Of course the best way is to repeat it and do it right.
Dr. Pheo
Hi Dr Pheo
ReplyDeleteMy pathology report show paranganglioma after right adrenal gland removal w/ tumor. I am flagged as “high risk” due to primary Polycythemia on the report.
The plan states to do 3 monthly DOPA PET scan as some mutations are not seen on OG DOPA CT scan.
It mentions suspected hypoxia inducible factor pathway genetic mutation. They’ve requested referral to Genetic services clinic.
What does this mean exactly RE hypoxia pathway being suspected? Is this what they call HIF I would assume yes. Are Paras with polycythemia more prone to metastisis / reoccurrence? Thank you in advance for your time.
Oh P.S could not find any familial trail of Poly or gene mutation when I was tested back at age 8. I’m now 30.
Dear Anonymous,
DeletePheo and para can be associated with polycythemia. Most patients with this syndrome have somatic (not inherited) mutations in HIF2α. So the mutation test has to be done on the tumor specimen, not in blood. HIF2α is in the hypoxia pathway. I don't think there are data suggesting that the pheo and para are malignant they they can be multifocal. That's why additional scan is suggested.
Dr. Pheo
Due to COVID delays I have finally met with genetic services whom believe I may carry EPAS1 mutation due to the Poly diagnosis + Pheo.
DeleteDo you know much of this mutation?
I’m being screened for Pheo/Para panel genetic testing (AUS standard). I have no clue if the tumour was ever tested as such like you state above (?)
Dear Anonymous,
DeleteEPAS1 is the gene that directs the production of HIF2α. It is included in several pheo/para gene panels.
Dr. Pheo
Hi,
ReplyDeleteI'm the same person who had MEN2A and wrotte the last year.
The year 2020 results of plasma metanephrines are:
Metanephine 119 pg/mL - ref range max 65
Normetanephine 573 pg/mL - ref range max 196
What I have to do now?
Dear Anonymous,
DeleteThe results are quite concerning for pheo. You need to do imaging such as DOTATATE PET/CT as soon as possible.
Dr. Pheo
Hi, Finally I did a CT with contrast and I have a 33 mm nodule in the right gland, the left gland appears to be normal. It's necessary to do more imaging procedures like DOTATATE PET?
DeleteThanks
Dear Anonymous,
DeleteDOTATATE PET should still be done if covered by insurance. If not, FDG PET or MIBG scan can be used. The purpose of these scans is to see if there is another pheo somewhere else.
Dr. Pheo
Hey!
ReplyDeleteI have abit of a tricky situation, so I have had suspicion of a pheo from my doctor when I presented to her with bouts of pallor, sense of doom, increased heart rate and high blood pressure. I take Vyvanse for ADHD and my psychiatrist is worried because these symptoms are unusual for people who take vyvanse. We took me off the vyvanse for a little bit to see if symptoms settle, the didnt. So for the sake of my mental health I was put back on. When I have these spells my blood pressure can get as high as 180/103. My heart rate seems to always be over 100. My doctor did a battery of tests. 24 hour urine, plasma free metanephrines, full blood count, Doppler ultrasound of kidneys and a CT scan of pelvis area. All of it came back normal except my Plasma Free normetanephrines which were 794 pmol/L (Ref <570 pmol/l) and my 24 hour urine normetanephrines were high but we corrected with creatinine were borderline. My symptoms have persisted so my doctor sent me to and Endo who did another plasma free metanephrines test and it came back with my normetanephrine as 1030 pmol/L with the same reference range as before (<570) my symptoms aren’t responding to blood pressure medication, and these spells are becoming more frequent each couple of weeks. My Endo isn’t sure if it’s the Vyvanse causing the normetanephrine results and doesn’t want to do any further scans until we do a third plasma free metanephrines blood test. I guess what I want to know is, is my vyvanse causing this metanephrine result or is it indicative of a pheo?
Dear Cortex95,
DeleteIt is possible the higher normetanephrine level is related to Vyvance use. Your risk of pheo, however, is very low.
Dr. Pheo
This comment has been removed by a blog administrator.
ReplyDelete