Happy
Thanksgiving! This will be the last piece for 2013. I wish everyone a great
holiday season and a wonderful year of 2014!
When we, health
care professionals and lay people alike, talk about pheo, we seem to assume all
pheos are the same. In medical textbooks, pheo is mostly discussed in a way
that gives the reader the impression that if one has a pheo, one will have all
the classical signs and symptoms of pheo. In the lay communications, the heterogeneity
of pheo is even less appreciated. One pheo, however, can be very different from
another. I have written about clinically “silent” pheo, pheo associated with
normal blood pressure, pheo that causes frank cardiovascular problems, small
pheo, pheo caused by genetic mutations, etc. Understanding the heterogeneity of
pheo is critical to tailor pheo management to an individual patient with a
particular pheo.
Preoperative
management of pheo has been controversial since the very beginning. I myself
have advocated careful preoperative preparation for every patient with pheo. On
the other hand, there are many different ways of preoperative preparation used
by experts, all with similar excellent outcome. Recently, one review article
proposes risk stratification for pheo, which may help reconcile the paradox.
The article
divides pheos into 3 categories: low-, intermediate-, and high-risk, according
to the risk of heart complications a particular pheo can impose on a patient.
Low-risk pheos are those smaller than 3 cm in diameter. This article proposes
that specific preoperative preparation is not needed for low-risk pheos. High-risk
pheos are those that already cause obvious cardiovascular complications such as
arrhythmia, heart attack, heart failure, or stroke. Careful preparation is
needed for high-risk pheos and the doctors need to coordinate their care so
that the preparation regimen is safe and effective. Intermediate-risk pheos are
those that are none of the above. The preparation regimen should be
individualized. The higher the relative risk of cardiovascular complications a
pheo has, the more careful and longer the preparation it requires.
It needs to be
emphasized that this risk stratification is based on clinical observation and
just a proposal. It has not been prospectively tested. What am I doing? I
currently still carefully prepare everyone. If a patient does have a low-risk
pheo, I am more relaxed with the regimen.
Dr. Pheo
My nephew aged 14,has been operated twice for pheo at AIIMS,New Delhi. Now, after Dotanoc test it is detected that tumor spread in different parts of body. any specialist whom i can consuly ? is there any hope?
ReplyDeleteDear Anil,
DeleteHe should see a specialist on metastatic pheo or para. The outcome likely depends on the growth speed of the metastatic tumors.
Dr. Pheo
Same MEN2A patient who had borderline elevated labs, MRI confirms that a small (1.2 cm x 1.3cm) nodule is on my left adrenal and resembles pheo. They also found several small (largest .7cm) lesions throughout both lobes of my liver, radiologist is recommending further studies on those. Not sure if that should be concerning at this point or not, do you have opinion on that? Thank you for your opinion, my endo originally wasn't concerned about my labs until I pushed her on it and mentioned your opinion. She is referring me to NIH for further treatment.
ReplyDeleteDear Anonymous,
DeleteThanks for letting me know. MEN2A pheos are usually not aggressive. I thus doubt whether the liver nodules are metastatic pheos. The NIH has great expertise in pheos so you will be in good hands.
Dr. Pheo
Hello Dr. Pheo,
ReplyDeleteThis patient had bilateral pheo surgery recently at the NIH. The right adrenal was removed. It seems there were tiny pheo's in a row right behind the tiny one visible on scans, so that was removed. The left adrenal had a larger (although still very small) pheo and that pheo was removed leaving me with about 50% of that adrenal gland.
I'm grateful for screening of SDHB patients like myself and I hope to help in the fight to rid, especially children, of this disease.
Happy Holidays and much luck in the coming year.
Frances
Dear Frances,
DeleteThanks for sharing. Happy New Year!
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteOn November 8, 2013, I was rushed to the hospital in cardiac crisis. Thirteen days in critical care, I am blessed to still be here to write. Tests revealed a healthy heart, but a tumor on the right adrenal gland. At 56, I was in good health with NO prior symptoms. Now, I face surgery and trying to locate the best surgeon for this. I live in Southeast Florida. So far, have only found one surgeon who specializes in endocrine surgery and familiar with pheos. What questions should I ask on the initial visit? So glad my husband found your blog. Have discovered this is a rare condition to treat.
Dear 4amazinggrace,
DeleteThe most important questions are probably: 1) how many pheos have you removed? 2) Do you have a multidisciplinary team on pheo so that you work closely with an endocrinologist and cardiologist who both have experience in pheo cardiomyopathy?
Dr. Pheo
Hello, I recently had a metanephrine blood test done but the result was so abnormal that it seems like it must have been an lab error? (More than a hundred thousand.) How high can levels be in a person with pheo? Wouldn't all that adrenaline going on in my body had killed me already? Strange thing is I have many of the symptoms...
ReplyDeleteDear MH,
DeleteLikely this is a cleric error in the units (switching nmol and mmol) of the report. I have seen this kind error before.
Dr. Pheo
After a CT scan and blood and urine tests I have been diagnosed with a Pheo in my bladder. It is 1cm x 2cm at the front and top of the bladder. I am having an MRI and MIGB this week with surgery scheduled next week. The surgeon is a Urologist with 27 years experience and Mayo trained. However no one in Omaha, Ne has ever seen a pheo on the bladder. Should I be looking else where in the country for this surgery?
ReplyDeleteDear Tony,
ReplyDeleteIt depends on your comfort level and situation. If you only have this 1 x 2 cm bladder pheo, its surgical removal is not too complicated. You probably can have it done at Omaha.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteI recently started to get facial flushing and my bp went up (it was always normal). 24hr urine tests showed (at the same time) elevated all catecholamines:
VMA - 11 (ref range 0.0-7.5),
Epinephrine - 22 (ref range 0-20),
Norepinephrine - 176 (ref range 0-135),
Dopamine - 748 (ref range 0-510).
And markedly elevated cortisol: 226ug/24hr (reference range 0-50).
I want to mention that 6 years ago I was diagnosed with a kidney cyst (MRI report reads "simple benign kidney cyst"), which was not supposed to have any effect on the hormones. However, hormonal evaluation showed elevated cortisol (180ug/24hr), but catecholamines were normal.
What further evaluation would you recommend?
Thank you in advance for your opinion.
Regards,
Peter
Dear Anonymous,
DeleteThe pheo markers are not that impressive. The urine free cortisol levels need to be further followed. I recommend a low-dose dexamethasone suppression test. Another test can be midnight blood or saliva cortisol
Dr. Pheo
Thank you, doctor Pheo!
ReplyDeleteMy midnight salivary cortisol is <0.010 ug/dL (which is in the reference range <0.010-0.090).
Low-dose dexamethasone suppression test (1 mg of dexamethasone at 11 p.m., blood drawn at 8 a.m) returned 0.8 ug/dl (reference range 6.2-19.4). That appears to be a normal result.
What would you suggest as the next step?
Does it make sense to do a MBIG scan? My doctor claims it is helpful only when diagnosing pheochromocytoma, but not useful to investigate high cortisol levels.
Thanks & regards,
Peter
Dear Anonymous,
ReplyDeleteThe two normal test results rule out Cushing's syndrome. MIBG scan is unlikely helpful.
Dr. Pheo