I first wrote
about pheo and heart 4 years ago (April 2009). At that time, my instinct was
that heart damage is a key issue for pheo. Over the years, after seeing more
patients with heart complications caused by pheo and discussing with colleagues
about the issue, I increasingly appreciate the intricacies of pheo-induced
cardiomyopathy.
Pheo-induced
cardiomyopathy is not a new finding. The first two cases of pheo known to medicine
were both about pheo-induced cardiovascular collapse in modern terms. The
correlation of pheo and cardiomyopathy was clearly and convincingly reported in
the 1960s. Hundreds of case reports of pheo-induced cardiomyopathy have been
published in the last 50 years. The problem is that pheo is very rare and
pheo-induced cardiomyopathy is even rarer. Many cardiologists and
endocrinologists simply are not aware of the concept of pheo-induced
cardiomyopathy. Part of the reason why pheo-induced cardiomyopathy is not well
known is that there are few, if any, systemic studies on pheo-induced
cardiomyopathy.
In the last year,
two very similar systemic studies were published on pheo-induced
cardiomyopathy. Both studies were retrospective review of experience of a large
single medical center (in Prague and Los Angeles, respectively). In both
studies, the authors focused on patients without known history of
pheochromocytoma but presenting with cardiovascular complications such as
arrhythmia, heart attack, heart failure, or stroke. Their results were
strikingly similar as well. The Prague study included 145 patients, 28 of them
(19%) presented with cardiovascular complications. The Los Angeles study was
about half the size with 76 patients, but still 9 of them (12%) presented with
heart complications. Because both medical centers are large tertiary referral
centers and tend to see sicker patients, the incidence of cardiovascular
complications may be overestimated but the 12-19% incidence is at least true in
large hospitals. Both studies showed that there are no good predictors on which
patients will get cardiovascular complications. Specifically, most patients
with cardiovascular complications do not have hypertension. There may be a
higher risk for cardiovascular complications if the pheo is biochemically very active
and large. I have mentioned in a previous post that pheos smaller than 3 cm do
not cause cardiovascular complications. Both studies showed that once correctly
diagnosed and managed, the pheo-induced cardiomyopathy is reversible. The moral
of the studies is that if a patient presents with cardiovascular complications,
pheo is a possibility, even if the patient has no history of hypertension. It
is also reassuring that the cardiomyopathy is reversible after pheo removal.
Dr. Pheo
Dr. Pheo,
ReplyDeleteA Unique Lung Carcinoid Conference to feature European and American Physicians is to be held on Saturday April 13, 2013 at the Vanderbilt-Ingram Cancer Center in Nashville, TN. Dr. Dan Granberg from Uppsala University in Sweden Joins Dr. Eric H. Liu and Dr. Ronald Walker both from Vanderbilt-Ingram for a day long program. More information may be found at: www.carcinoidawareness.org
Dear deborah,
DeleteThanks. Please say hi to Dr. Liu for me.
Dr. Pheo
Dr. Pheo,
ReplyDeleteI was wondering where to find useful,up to date information on survival rates in people with recurrent pheos/paras that have no genetic cause? Also what time is the time to start looking into treatment beyond surgery to get the best outcome?
Warm regards,
Dear Lori,
ReplyDeletePlease specify whether the pheo is recurrent (ie you have another pheo or para at an organ where pheo or para naturally occurs, for example adrenal gland) or metastasis (ie the pheo or para goes to an organ where usually there is no primary pheo or para, for example liver or bone)?
Dr. Pheo
Dr. Pheo,
ReplyDeleteThank you for a fast reply. Mine have spread to liver, bladder ( tumor recently removed from bladder wall) original was left adrenal many yrs ago,several surgeries left adrenal, lymph node behind heart removed, several small lymph nodes. Now on rt adrenal, lower rt pelvis, 3 liver and spinal lymph node.
Warm regards,
Dear Lori,
DeleteThe prognosis of malignant pheo is very variable. Generally speaking, the 5-year survival is about 50% but it varies a lot. The key clinical predictor is how fast the tumor grows.
Dr. Pheo
This comment has been removed by a blog administrator.
ReplyDeleteDear C,
DeletePlease post the email here. Make sure you remove any identifying information before you post. I will remove your current post to protect your privacy.
Dr. Pheo
Thanks for your response.
DeleteI have decided to ask a question instead.
Given that it does not have a medical cause, if diarrhea is persistantly present for a significant amount of time (occurring on every bowel movement) and is in addition to symptoms associated with abnormally elevated catecholamines, could this indicate the co-secretion of vasoactive intestinal peptide by a pheochromocytoma or could it simply be part of the flight or flight response?
I would prefer to ask about my personal situation by email, although I understand that this may not be possible. Would it change anything if my GP were to send you the email and then you reply directly to her.
Are there any ways you know of that I could get an opinion from a pheo specialist about my situation without travelling long distances?
Thanks again
Dear C,
DeletePheo usually does not cause diarrhea. Loose stool has many causes. If it is a significant problem, then searching the causes is worthwhile and important. The diarrhea by VIP is usually very severe.
Dr. Pheo
Hi Dr Pheo,
ReplyDeleteI'm interested in your input please. I just had the results for a 24hr Urine after a ~1.1cm adrenal incidentaloma was found during CT scan for something else. My epinephrine was only slightly raised at 191nmoL (ref <190), but the metanephrine was 960nmoL (ref <350). Also the norepinephrine was normal, but the normetanephrine was a bit high at 864nmoL (ref <650). I think other parameters were normal. I suppose I'm just looking for reasons for this to be a false positive, but actually from reading your comments, it could be highly consistent with a 1.1cm pheo couldn't it? I have had some weird symptoms for a while that I've been dismissing... it is all adding up now. I'm a 42 yr old female. Thanks.
Hi again
ReplyDeleteSorry for double post, but I'm interested to know how common it is for pheo patients just to have raised metabolites? Is this soley down to the production of catechol o-methyltransferase by the pheos or longer half life of metabolites or a combination of the two?
I've been doing alot of reading of research papers and see that many centres have age/gender adjusted reference ranges, do you also adjust values for age/gender? Also have you seen many patients with raynaud's phenomenon or livedo reticularis as part of the clinical picture for pheo?
Sorry for the many questions :-)
Thanks and best wishes.
Dear Annie,
ReplyDeleteSorry for the late response. Please see my answers under your earlier similar posts.
Dr. Pheo
Hello again Dr Pheo, thanks for the replies. The plot thickens with me I'm afraid and I'm wondering if you could tll me what you think. My latest 24hrU showed all normal levels of catecholamines and metabolites, but this time the dopamine was slightly over the top of the reference range, which seems particularly weird as this was low normal last time when everything else was high. Is this type of pattern something you have encountered before? My endocrinologist is sending me for the MIBG scan anyway, but it all does seem a bit odd.
ReplyDeleteThanks and best wishes.
Dear Annie,
DeleteThe dopamine levels in the urine do not mean much if your blood dopamine levels are normal. Overall your risk of pheo is small.
Dr. Pheo
Hello Dr Pheo. A friend is suspected to have Pheo because of 1. Chronic high BP and other symptoms of on and off headaches, tensed and anxiety 2. 24hr urine shows semi high cathecholamines and metanephrines 3. CT Scan shows 5mm nodule in the left adrenal gland. Primary PCP wants patient to go to Johns Hopkins but not specific on which department or Dr to follow up with. 1. Could this really be Pheo 2. Any suggestions on the best way to approach a follow action 3. Since he is Maryland, which institution or group in the Maryland/Dc/Virginia area is best equipped in experience to handle this, that you may recommend.
ReplyDeleteDear Arodan,
DeleteThis small tumor unlikely causes the symptoms, even if it is a pheo. A clonidine suppression test should be done to see if the slight elevation of markers is really because of pheo. The NIH's Dr. Pacak is an expert on pheo (in Bethesda).
Dr. Pheo
Hi Dr. Pheo. I hope you are having a great summer. I just wanted to share my thoughts. I tested positive to SDHB mutation. I am getting ready to schedule my annual mri scan. I am thinking yearly scans are too often. It seems to me, if you are given 3 clean yearly scans, you could go to getting a mri every three to five years. I don't think a radiologist would even see a very small tumor. But annual blood work makes sense to me. I have two kids affected, so I am trying to figure out what would be appropriate follow up for the rest of our lives. Just thought I'd share my thoughts.
ReplyDeleteDear Team Burns,
DeleteI completely agree. Biochemical testing every year and imaging every 3 years are sufficient for most people with mutations.
Wish you and your family a great summer.
Dr. Pheo
Dr. Pheo, thanks for such an informative blog! I hear it is rare for someone to have pheochromocytoma and LOW blood pressure, but "my cousin Ronny" seems to. They've been searching for an answer to his debilitatingly-low blood pressure for 5 years and think this is it. His blood and urine tests have shown that pheochromocytoma is highly likely and a scan is being scheduled. My question to you is... how is the treatment different for patients like Ronny? Thanks!
ReplyDeleteThis comment has been removed by the author.
ReplyDeleteHi Dr Pheo,
ReplyDeleteI would be interested to hear your thoughts on scanning techniques. As well as inter operator variation.
I have just had my annual MRI done which found my abdominal para, and one of my pelvic ones have grown 1.5 times larger in the last 12 months. (ie from 9mm to 14mm and 17 to 24mm). Gallium PET showed stable disease over the last 12 months. Could the extra mm be put down to differences in radiographers doing the reports?
A comparison between scans was not made so I have had to ask that they go back and compare with the previous MRI.
Also, could 'silent' paras cause night sweats. (I have one in neck, one in abdo and about 4 in pelvis.) The night sweats are becoming debilitation. Had TAH/BSO 3 years ago and am way past menopause and those symptoms, so I don't believe this is connected to female hormones. The night sweats are accompanied by drops in blood pressure. (Metanephrines are normal.)
Many thanks,
Sim from Australia
Dear Sim,
DeleteMRI is more accurate in measuring tumor size that PET. If metanephrines are normal, then the night sweats should not be caused by the paras.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteThank you for sharing the good advise, once again.
I wonder what ever happened with the theory that the SDHB gene mutation could hold some answers for why other tumors metastasis. I read an article by Dr. Charis Eng; and another by Dr. Karel Pacak, on the subject and then noticed that the idea kind of fizzled.
You might like to know that my family is doing well. My nephew, daughter, grandchildren, niece and myself are all being monitored due to the SDHB mutation and all scans have been clear for a while.
Thank you again for your kind help.
Frances
Dear Frances,
DeleteI have to admit that I know much less on the SDHB mutations than Dr. Eng and Dr. Pacak. I am glad that your family is doing well.
Dr. Pheo
Dr. Pheo,
ReplyDeleteThank you for this resource. I'm writing today to ask you for advice on finding an Adrenal tumor/Pheo expert in the Norfolk, Virginia Beach area of Virginia. I've read through older posts and I'm wondering if you would recommend seeking out Dr. Pacak in MD, using UVA or is there someone local you could recommend since those places are a few hours away? Thank you so very much for your time.
Dear Derek,
DeleteDr. Vinik at EVMS is an expert on neuroendocrine tumors in general.
Dr. Pheo
Dr. Pheo,
ReplyDeleteAfter a few borderline (but normal) test results, I did the 23 and me gene screen.
How likely is it for a symptomatic person who has not had astronomically abnormal blood tests to have pheo if their genes are suggestive.
The mutation was SDHD A/G rs11214077 at position (B36) 111463887. None of that means much to me but I'm still looking for an answer, and was wondering what you thought?
Thank you.
Dear Tetley,
DeleteIf the test results are normal or borderline, your high blood pressure is unlikely caused by a pheo. I don't know how the SDHD gene polymorphism should be interpreted.
Dr. Pheo
Hi Dr. Pheo! 7 years ago my mom presented with all the symptoms of a Pheo. Finally after a MiBG scan, Octreotide scan and 24 urine for VMA was all Postive. The MRI scan was negative. She finally had her Left adrenal removed, the surgeon stated it was in the medulla. well anyways my mom NOW still is having all the same issues plus..... she is on Toprol, Labetalol, verapamil, clonidine patch, Lasix, spironolactone and also Flecinade. with resently episodes of VT and feeling like she is going to pass out. Her Cardiac cath is clear and her EP study was ok could not be ablated.. she also has flushing after she eats and profuse sweating even if it 60 degrees out! LOL! what should she do if her CT scan comes back negative? Where we live there is not many MD's that know too much about MENS or Pheos.... Please help!!! We live in Syracuse,NY-concerned Daughter
ReplyDeleteDear Linda,
DeletePlease let me know the exact VMA values and what the MIBG and octreotide scan said. My overall suspicion of pheo is low. Please read my earlier post on adrenal medulla hyperplasia.
Dr. Pheo
Sorry I left out that she just had another MIBG scan the other day that stated, Right Adrenal and Pelvic area was positive...
ReplyDeleteHello Dr. Pheo
ReplyDeleteCan I ask your opinion of someone with SDHB showing "Bilateral cm T2 hyperintense enhancing adrenal masses suggestive of pheochromocytoma?"
I'm not sure what to think.
As always, thank you for your wonderful blog.
Frances
Hi Frances,
DeleteThese are likely pheos.
Dr. Pheo
Hi Dr. Pheo,
ReplyDeleteA relative of mine has pulomary stenosis and hypoxia. When she first started seeing pheo symptoms - bouts of fast heart rate and high BP - cardiologists were looking for a cardiac cure. After many inconclusive cardiac tests a valvuloplasty was performed. The valve is now leaking/insufficient in lieu of stenotic.
Approximately a month post op, a pheo has been located and determined to be the cause of the HR and BP problems.
Are there any special precautions that should be taken due to the congenital heart defect and now leaking valve?
Thanks.
Hello,
ReplyDeleteMy husband who is 33 has just been diagnosed with a pheo on the top of his left adrenal measuring 3.8cm.
He has many of the classic symptoms for 4 years. I was just wondering if it is still a pheo considering it is on top of his gland and not inside? Also I was wondering if we should have the genetic testing? I am worried for our 4 daughters ages 5,8,11,12.
Thank you so much for your help.
Dear Darylynn,
DeleteThere is only semantic difference between "on top of" or "inside" the adrenal gland. As he is so young, it is a good idea to do genetic testing.
Dr. Pheo
Ok. Thanks:)
ReplyDeleteDear Dr. Pheo,
ReplyDeleteMay I ask for citations for the Prague and Los Angeles studies please? If there are other references that you may suggest, I would be very appreciative for those as well.
The situation is: I wish to present evidence to my key doctors in hope that I will finally be granted surgery to remove my pheo. Since it is only 1.7cm, it is assumed that it cannot possibly cause problems. Bio-chem and imaging tend to flip-flop complicating the diagnosis. I am just not a text-book case.
After 9 years of severe typical pheo symptoms (extreme hypertension, drenching sweats, anxiety, horrendous headaches, etc), I have entered the TIA, heart attack, heart failure, and cardiomyopathy phase of the disease.I am fairly certain death is the next phase if this pheo is not removed. Meds are helping with the symptoms but not with the deeper pathology.
Any suggestions that may help with my presentation to my doctors would be greatly appreciated.
Thank you,
Kit
P.S. My close cousin (maternally and paternally-related) recently died of a pheo-induced heart attack. The pheo was discovered only upon autopsy.
ReplyDeleteDear Kit,
DeleteHere is the newest and probably largest study:
Heart. 2013 Oct;99(19):1438-44. Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma.
On the other hand, I do agree with your doctors in that a small pheo unlikely causes cardiomyopathy. If you do have a pheo, it is still a good idea to remove it.
Dr. Pheo
Thank you!
ReplyDeleteKit
Dr
ReplyDeleteFor the last 18 months I have battled heart palps severe anxiety and sweating with other issues finally did 24 hour test and it showed 497 with max range of 300 dr ordered a ct and found 10 cm tumor. He said all other levels were normal and this might be a dopamine only producing tumor. He is not a 100 percent sue this is what is causing my problems but I have never suffered like this before. Please help based on that should I have it removed? I want it out because I can't live like this anymore. I am on many different things to calm the anxiety. I was sure this is what was causing my problems and now I don't know please respond
Dear Chad,
DeleteThe dopamine level and the size of the tumor cannot make or rule out pheo diagnosis. As the tumor is very big, it needs to be removed.
Dr. Pheo
I'm getting ready to have an adrenal gland removed on one of my kidneys. I have been told by an endocrinologist that I have a pheo. I have also been told by another endocrinologist that although he can't rule a pheo out, that it is too small and that I probably just need an estrogen patch. My hormones have been checked and are fine for my age. I have some doubts as to it being a pheo. I have been suffering with random surges of BP spikes for over 3 yrs. I get episodes where I first start feeling off balance, it's like a waive going through my head and then Sometimes I lose speech, become disoriented, close my eyes and basically become inert although I can hear everything going on around me. It gives an appearance of a TIA. Sometimes with these episode I'll get an overwhelming sense of emotions that can make me tear up. In the last week they have gotten worse, in that my BP is going over 200 with every episode, prior to this last week I could have episodes and my BP did not shoot up. The tumor on my kidney is only 1.7 cm and only once did it possibly show up in a urinalysis, never once on a blood test and last urinalysis came out good. Not sure, if I should go through surgery. Previous misdiagnosis was seizures and anxiety.
ReplyDeleteDear RA,
DeleteTwo issues here. Whether you have a pheo and whether removing the adrenal tumor will help you.
You need to ask your doctors whether the 1.7-cm adrenal tumor has imaging characteristics of pheo. If it doesn't, it is unlikely a pheo. If it does, it is probably a pheo.
If the imaging characteristics of the tumor are consistent with pheo, removing it is a good idea but you unlikely will have improvements in symptoms.
Dr. Pheo