You may have heard the term “silent pheo”. The sole purpose of this post is to convince you that “silent pheo” is a dangerous term and should be avoided at all cost. So next time you hear someone mentioning “silent pheo”, you tell the person there is no such a thing. Every time I hear the term, I get goose bumps and try to correct the person.
The term “silent pheo” or “subclinical pheo” apparently refers to a pheo in a patient who does not have hypertension or paroxysmal attacks. There are quite a few situations where a patient indeed has a pheo or paraganglimoa but does not have classical symptoms of pheo. Most paragangliomas in the neck and chest do not produce significant amounts of catecholamines. I would rather call these tumors "nonfunctional" rather than "silent". Very small pheos produce only small amounts of catecholamines so that they do not cause clinical symptoms. In the above two situations, blood tests for pheo show normal results. Most pheos in adrenal glands, retroperitoneal space, and bladder do produce catecholamines and are therefore "functional". The majority of patients with pheo have at least subtle symptoms that can be elicited by experienced physicians or realized in retrospect after removal of the tumor.
Some patients indeed have no apparent hypertension in spite of very functional pheos. These patients tend to be mismanaged and are at the highest risk of complications around surgery. Here are the reasons. These patients tend to be young and healthy before they have pheo. Their bodies try very hard to accommodate the bombardment of catecholamines by shrinking their blood volume. My experience is that their blood pressure may be normal while sitting or standing but rises when they lie down. An inexperienced doctor may think they have so-called "desensitization" of catecholamine receptors and can go ahead with surgery without preparation. Disaster happens if they do. They will have very high blood pressure while the tumor is manipulated and profound hypotension after the tumor is resected. Then they will get a lot of intravenous fluid their hearts can not handle. It will be a mess!
The bottomline is that if a patient has normal blood test results, whatever tumor the patient has may not be sufficiently functional. If the patient has elevated blood markers for pheo, the patient should be treated as having a functional pheo and undergo careful preoperative preparation, regardless of having hypertension or not. A young, otherwise healthy patient with a large pheo and high levels of markers but without hypertension is the most vulnerable one. That patient particularly deserves careful preoperative preparation.
Dr. Pheo
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I had one of those pheos that didn't come with high BP. 110/80 was a typical reading in the doctor's office. It was higher during the tumor's monthly "squirts," but that never happened during a doctor's appointment. As a result, the endo who had followed me for diabetes never suspected a pheo, and even denied the possibility of it after it really erupted a few months ago and sent me to the ER with very high BP, crushing headaches, and a possible heart attack.
ReplyDeleteWhat a tricky little beast it was. Even the word benign seems a misnomer when it was so nasty.
Thank you for sharing. How big was your pheo?
ReplyDeleteDr. Pheo
I'm a 28 yr old male who has recently been diagnosed with a pheo on my adrenal gland following a 24 hr urine test and CT imaging. At the moment I feel the doctors I'm working with haven't been clear on some of my questions. I was wondering if you could add to my knowledge by answering the following;
ReplyDelete1. How urgent is it that I have the surgery done asap - Read: What is the prognosis for long-term affects on my body and general health if the surgery is done in the next week vs. three weeks from now (or, what risks are associated with a delayed surgery - with those risks being anything from stroke due to high blood pressure to the tumor growing in size to other cardiac arrhythmias). In line with this, what is the risk of something serious (fatal) happening to me while I await surgery?
2. What happens if my blood pressure disappears/increase dramatically during the surgery? How is this handled in the minute?
3. What is the chance of reoccurrence of the tumor, on the adrenal gland or somewhere else? What are the risks associated with it reoccuring?
4. How do I find out whether or not the tumor is malignant? So far all that my doctor has said is that it is a very rare thing. Is having the surgery the only way to tell, or should there not have been tests done already to rule this out?
5. Should there be an endocrinologist assigned to follow up with me post-surgery?
6. Who is the best surgeon/doctors in Canada for pheo's? And how do I weight whether or not I am getting the absolute best and speedy care available to deal with this disease?
8. While awaiting surgery, what activities should I omit from my daily routine (thinking, in particular, of those that might increase heart rate or blood pressure, ie: sex, weight training, cardio activit) – I’m typically an active person Can I still use this as my means of transportation?
9. The doctor as ordered me a specific medication to block the effects of the tumour. I was told by one doctor that I would be in the hospital closely monitered before and after the surgery for up to a week, while my doctor that is handling my case seems to think I should be in and out in a day or so, and that I will administer the medication myself. Which is the correct course of action?
Any insight you can offer to the above questions would be greatly appreciated. I really appreciate your time as I've found this process a tad frustrating and feel that I lack some key information which the doctors should have supplied. Please note that I have done abundant research into pheo's and have a general understanding of the surgery and what they do, but I would really like to hear from someone such as yourself, who has experience treating them. Thanks again
I look forward to hearing from you
Brody
1. The most important factor to determine the timing of surgery is how well you are prepared (usually for at least 2 weeks). So long as you are prepared well, delaying surgery by 2 weeks does not carry significant risks.
ReplyDelete2. If you are prepared well, hopefully the blood pressure fluctuations won't happen. If they do, the anesthesiologist will use medications and intravenous fluid.
3. About 10%. Nothing you can do will prevent recurrence. If your pheo is familial, then recurrence is more likely.
4. Whether a pheo is malignant or not can not be reliably predicted by pathology. It is almost always a hindsight.
5. Yes. You should be followed life long.
6. See my doctor list for Canada.
8. Avoid vigorous exercise (especially those impact-prone) and emotional stress.
9. The preoperative preparation can be done safely and well as outpatient. Your doctor tells you want to do and have phone conversation every few days. If you are prepared well, you may stay in hospital for only a few days after surgery.
Best wishes.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteI am a 48 year old female who is being investigated for the third time in 15 years for pheo.
I also have RRMS which borders on benign and I am well. I started exercise in January, which was 1hour of vigourous cycling, up hills.
I do have hypertension which was being controlled with atenolol (to slow down my heart rate) losartan and amlopidine.
Each MS attack has always coincided with a massive spike in my BP which involves emergency measures.
This crisis usually disappears with the usual intravenous steroid protocol (1gm daily for 3 days).
I have not had a relapse since 2005, but have just come out of hospital after a pretty severe attack.
I took Rebif 44 for the MS for eight years and then stopped it for 6 months, as I had run out after moving to Spain from the UK.
Then I started on Low Dose Naltrexone as an experiment to see if the hype was true and that it can slow progression.
My relapse started in March and as usual my blood pressure spiked to dangerous levels.
I have restarted Rebif 44. My endocrinologist stopped the atenolol (after 15 years) and my heart rate is always over 100bpm.
My catecholomine levels on the second urine collection (without atenolol) were raised and I am about to have other scans and tests.
I have two questions.
1. Does the brain somehow 'know' that it is under attack at the start of a relapse, which then activates adrenal glands (and cause the pheo to switch on.
2. Do you think that the LDN effects plus the vigourous exercise, raised my endorphin levels and caused the relapse/pheo crisis?
I hope you can help. I appreciate that my condition is somewhat unusual!
kind regards,
K Roberts
I am not sure if you have pheo at the first place. Did your doctor think you have pheo? To see whether you have pheo is rather straightforward.
ReplyDeleteMultiple sclerosis can be associated with autonomic neuropathy whic causes blood pressure fluctuations. That does not mean you have pheo.
Dr. Pheo
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ReplyDeleteHi Dr Pheo
ReplyDeleteI have been in hospital for the past 2 and a half weeks. They have been searching for the pheo i mentioned prev. I had four attacks in one day in hospital with a geart trace on it showed psvt maximum heart rate of 186. BP at 190/118 they have done 3 24 hr urine tests which all came back normal. I had a ct of brian chest and abdomen all clear. ultrasound of kidney as i was suffering severelaft flank pain. Bloods all ok. They sent me home on a concoction of drugs. but still having attacks. any thoughts.
Martin
Dear Martin,
ReplyDeleteIn that case, it is unlikely that you have pheo. Many conditions mimic pheo (just as pheo mimics other conditions). It is not possible for me to suggest a specific condition, but in my experience, the most common pheo-mimicking conditions are poorly controlled essential hypertension, sleep apnea, and anxiety.
Dr. Pheo
A late response (my comments RSS stopped working). Mine was 5 cm. They think it was there between 6-12 years.
ReplyDeleteDr Pheo,
ReplyDeleteRelated question for you. I am someone who has already had one nonfunctional pheo successfully removed (small...1.5cm extra-adrenal near renal vein) and I'm also SDHB+. As a protective measure, I took phenoxybenzamine prior to surgery to be on the safe side. I worry that if I ever had to have an emergency surgery, the doctors may not know about the risk of excess catacholomines from a possible undetected low-functioning or non-functioning tumor.
Should I get some sort of Medic Alert bracelet? if so, how to summarize?
Dear Jennifer,
ReplyDeleteThere are several issues here.
1) It is a good idea to have a med alert bracelet. You can say "I may have pheochromocytoma" or "I have genetic risks for pheochromocytoma".
2) More important is to follow the screening test regimen. If you have a negative test, chances are small for you to have a clinically important pheo/para within a year after that test.
3) If you need emergent, life-saving surgery, you have to go ahead and do it with careful anesthesia management.
Dr. Pheo
good advice, thank you! no worries, i am all over point #2 - yearly scans, excellent docs.
ReplyDeleteI have MEN2a and thus had a pheo on each adrenal gland back in 2004. I'm one of those people who has always run quite low blood pressure so it surprised me when the MEN2a diagnosis came right with the pheo diagnosis. I honestly had no clue and only started having the racing heartbeat symptom a few weeks before being diagnosed. :)
ReplyDeleteI lost my right adrenal gland with a 4cm pheo and part of my left gland with a 2.5cm pheo (I think, it's been awhile). Unfortunately I'm now steroid dependant but it just blew me away later to see others with pheo who ran really high blood pressure and here I never had a clue. lol
Dear Jo,
ReplyDeleteThank you for sharing the information. Some patients like you have no hypertension but other symptoms (palpitation in your case). And pheos are notorious for being unpredictable. It is always a good idea to treat pheo with great caution.
Dr. Pheo
I was a patient who only had elevated Blood Pressure and Heart Rate during a surge/flash. Initalially diffuicult to find because I had a surgical hysterectomy and symptoms similar to menopause. I had a surgical hysterectomy in 2005, for 1 year I suffered with surges/flashes, finally went on estrogen patch and got relief from the surges/flashes. Woke up one day with numbness on my left side, discontinued the estrogen, surges/flashes came back. Other findings brain Cavernous Angioma and Antiphosholipid Antibody Syndrome. Stopped the estrogen. Surges/flashes came back. Scan showed tumor on adrenal gland. Neurotransmitters levels, epinephrine: 3482, norapinephrine: 3353, metaepharine/creatine ratio: 3638. Had the pheo surgery last week. The day after the surgery had a tremendous spike in BP and HR. After 45 minutes they injected Lopressor. Stabilized. Sent home on Labatelol. Torso rash, discontinued Labetalol. Later identified rash to not be drug related. Blood Pressure ranged from 85-225 and Heart Rate ranged from 105-138. Flashes occurred hourly. Started another 24 hour test. Was put back on Labetalol 100mg BID. Was only able to complete 18 hours of urine collection, will this be sufficient? Had the blood test while on the Labetalol, will this impact the test results? Waiting for results. Endocrinologist thinks may have another pheo. Surgeon thinks it is unlikely. I am confused. Do you have any insight for me? Thanks in advance for you input. Annie
ReplyDelete24-hour urine test is important. It is hard to interpret an 18-hour test.
ReplyDeleteIdeally you should wait 2-4 weeks after surgery to repeat the pheo tests as it takes time for the old markers to be removed from the body.
Labetalo can interfere with pheo testing.
An undiagnosed pheo is a concern. Once that possibility is addressed, other diseases will be considered.
Dr. Pheo
Thank you. I will repeat the 24 urine test. It It was only a week after surgery that I did the follow-up test. How many days would you recommend for the Labetalol to be out of the system to retest? The 18 hour urine test came back, the norepinephrine plasma value was: 922, prior to surgery with the 24 hour urine test it was 3353. A metabolic Panel showed abnormal SGOT (AST):53 and SGPT (ALT): 81. Could this be from the pheo or surgery? Thank you so much for your input. I really appreciate it! Annie
ReplyDeleteI usually hold labetalol for at least 3 days. The abnormal liver enzymes have many possibilities. Pheo usually does not cause abnormal liver function. Anesthesia and surgery both may cause some transient liver damage. Viral hepatitis is also possible. I suggest that you talk to your surgeon about it.
ReplyDeleteDr. Pheo
So agree with your entry. Many doctors apparently go by 'the book' instead of using their own noggin and thinking/perceiving what they see in front of them. This is a problem in medicine. Doctors - do not be afraid to rely on your own experience/acumen/observation!If it walks like a dog, barks like a dog - it might be a dog, even tho it does not exactly fit the book/guidelines that you studied in medical school. Trust yourself and your talent - this is why you originally chose medicine! I am saddened by the number of potentially talented physicians that have become workhorses/grunts.
ReplyDeleteHello, for the past two years I have started having spikes in my blood pressure (178/124 pulse rate 144). I have been admitted to the hospital and they have run the 24hr urine and blood tests. My normetanephrines always come back in the high range but not high enough as they say to definately say I have a pheo. I think I am going crazy at times because I will be having a great day and then all of the sudden this surge just happens and I start sweating and having the palpitations. Usually only last about 30 minutes to an hour before my BP and heart rate start coming down. I have been on HCTZ for over a year now and it works perfectly until one of these attacks. Can you have a pheo when just your normetanephrines is high in both the plasma free and 24hr urine test? Any other suggestions? Thanks! Teresa
ReplyDelete