OK. Now you are diagnosed with pheochromocytoma, carefully prepared before resection, and operated by a great surgeon. Are you out of the loop yet? What shall you do next?
For patients with truly sporadic pheochromocytoma, 90% is the chance that they are cured. The remaining 10% may have recurrence or metastasis in the future. The problem is that the doctors can NOT predict who will be the lucky 90% and who will be the unlucky 10%. Therefore no patients should think that they are absolutely cured and all patients should have follow-up.
My own approach to patients with apparently sporadic pheochromocytoma is to measure plasma metanephrines 1 month after surgical resection to make sure there is no hidden tumor somewhere. Too early measurements can be false positive because I suspect based on my experience that there is some kind of depot of metanephrines in the body that is only depleted in a month. If the metanephrines are normal, I then measure them yearly or whenever they have symptoms suggestive of pheochromocytoma or when an adrenal or other mass is identified incidentally.
If a patient has familial form of pheochromocytoma or a gene mutation is identified, then the patient will have a much higher chance of recurrence or metastasis. Yearly plasma metanephrines are definitely needed. Imaging studies may be indicated depending on which form of familial pheochromocytoma or which gene mutations. Other components of the syndromes need to be screened; patients' family members need to be tested and carriers need to be regularly screened.
Now I have finished the basic topics on pheochromocytoma. I will write more on my personal experience and more interesting topics. If the contents of my blog are mostly based on my own experience and there is not much existing study on the topic, I will clearly state that at the beginning of the post. I welcome colleagues and patients to critique and to suggest. I will also just use "pheo", instead of the full name.
Subscribe to:
Post Comments (Atom)
Do you, as an experienced pheo doctor, see a lot of patients with stories of people who are told they don't have a pheo by inexperienced doctors, when actually the patient does have a pheo?
ReplyDeleteI was told by one doctor two years into my hunt that I couldn't possibly have what I have because it's just too rare.. nine years later I got the final diagnosis that I in fact do have it. I was curious if that happens a lot.
I do have seen patients with typical pheo symptoms which are ignored by their doctors. One patient had classical symptoms and went on the internet for clues. He begged another doctor to order pheo markers and the results were very high. He showed the result to his original doctor who was only then convinced.
ReplyDeleteI also see the opposite of the above, which is similarly regrettable. Patients with symptoms resembling those of pheo only have borderline lab results without tumors on imaging. The adrenal glands may appear "fat" (slightly larger). Often one adrenal is resected but no tumor is found. Guess what, the symptoms will remain and the markers will stay borderline. The patients do not have pheo in the first place and getting rid of a nice adrenal gland won't help.
I will write more on these two situations as whole topics.
Dr. Pheo
first, I have to say I have good doctors.
DeleteI had pheo about a year ago, I was the textbook case. I had my left adrenal gland removed but that didn't get rid of some of symptoms: heart palpitations, shortness of breath,cold hands and feet, that go numb and that get clammy cold; (raynauds disease) but have low blood pressure that goes from 85/56 to 138/95..I also had a spinal tap done and found that I had high white blood count: 538, and high protein in my spinal fluid. I am hoping that someone out there can help me find what's wrong. I have also had cat scans,angiogram, pet scan.
my doctors have given me all the tests that they can think of..but it always comes back normal..
Doctors can tell me that I have viral meningitis, and I have an autoimmune disease which they don't know which one.
if anyone can help me, please do, as this has put my life on hold.
That's kind of what happened to my left adrenal gland except I did have a pheo (just not on the glands). When they couldn't locate it right away with the 131-MIBG scans they saw in a CT scan my left adrenal gland was "slightly enlarged" they told me it was their best bet (I was 17 and didn't think to get a second opinion) so out it came and no pheo. My levels went down slightly for a bit (still never went below ten times the normal though) so they started to question if I had bi-lateral pheos... I don't think it crossed their minds to think outside the adrenal gland. So nine years the little trouble maker sat on my heart without anyone seeing it. (until the 123-mibg came along and lit it up like a single christmas tree light in a dark room.)
ReplyDeleteWhich is exactly why I'm so happy more and more people are talking about pheos. I don't want people to go through what I went through. Diagnosed with Carney Triad, I'm just hoping I don't get a pheo on my remaining gland later in life. I'd be sorry then.
My Wife had a similar location of the pheo right next to the heart and lung. Luckily though before suspecting Pheo we saw this guy (3 cm) right there behind the left atrium of the heart and apparently(found out post surgery) that it was impinging on the left atrium and also the left pulmonary vien. This meant they had to do a lower lobectomy of the left lung and also a pericardial reconstruction on the left atrium wall. I think the surgery was the worst part for my wife. The post op complications were quite a bit. How did it go for you?
DeleteI just hope that there were no blood pressure fluctuations during the first surgery. It was a risky one in retrospect.
ReplyDeleteDr. Pheo
Dr. Pheo,
ReplyDeleteMyself and my family (with SDHB mutation) are being managed and advised by one of the physicians on your list. However, they recommend much more frequent screenings than once yearly. In fact, my brother who is a carrier and has had two tumors (pheo and paraganglioma) is being advised to be screened quarterly. Just thought I'd add my comment to the post.
Dear Katie,
ReplyDeleteHow frequent to follow a patient with familial pheo depends on quite a few factors. In most carriers, yearly screening is adequate. Most pheos do not grow very fast, so quarterly screening seems to me a littel too frequent. You may ask the doctor what her/his thoughts are on the quarter screening.
Dr. Pheo
Do you know of any association between an adrenalectomy (post unilateral spontaneous pheochromocytoma) and infertility thereafter? We are having trouble conceiving since my adrenal gland was removed 10 months ago, whereas, we conceived within 2 months of trying with our first 2 children.
ReplyDeleteDear mjg323,
ReplyDeleteI am not aware of any association between adrenalectomy and infertility.
Dr. Pheo
My son, 34, had an adrenalectomy on the 10th of July. It was contained and not malignant. He since then has been crying or angry most of the day and has abdominal and back pain. He also has a neurofribromytisis lump along his spine.
ReplyDeleteA follow-up urine test came backslightly higher than Norman and cortisol was normal. What is the usual recovery like for an adrenalectomy of this type?
Lweiman
Dear Linda,
DeleteThe pheo markers are usually slightly elevated for longer than 2 weeks due to the surgical stress. I would retest the pheo markers at least one month after the operation.
Dr. Pheo
Hi Dr. Pheo,
ReplyDeleteI am so glad i found your blog, there is a lot of helpful information! But i still have a question, i had a right adrenalectomy and partial left adrenalectomy due to bilateral pheo in 2010, i have been doing check ups twice a year (24h urine and the usual blood work), everything is going well so far, i feel great too. I dont take any medication because the cortisol level is normal even though i only have half of my right adrenal. The doctors at the time said that only in emergencies or extreme stress situations the gland wouldn't be able to produce enough hormones. Well, i just got pregnant and was wondering if a normal pregnancy is considered a extreme stress for the adrenal. What are the symptoms of this adrenal insufficiency? I am a bit worried since this is my first pregnancy, i have no idea of what to watch for.
Also, it might sound like a silly question but i read somewhere that in adrenal fatigue cases the mother starts to draw upon the baby's adrenal to compensate the lack of hormone? Would that be the case if i can't produce enough?
Thank you,
Lilian
Dear Lumi,
ReplyDeleteYou probably do not need extra corticosteroid for your pregnancy. If you do, you probably will only need it in the 3rd trimester.
If you feel fine during the pregnancy, your baby is unlikely affected by that you only have half an adrenal left.
Dr. Pheo
This comment has been removed by the author.
ReplyDeleteDear Christina,
DeleteThe urine normetanephrine levels may not be as high as they sound. You need to see the lab report and find the normal reference range.
Dr. Pheo
This comment has been removed by the author.
DeleteThis comment has been removed by the author.
DeleteThis comment has been removed by the author.
DeleteDear Christina,
DeleteAgain we have to see the normal reference ranges. If all the values are within the normal reference range, pheo is ruled out.
Dr. Pheo
How can I show these results 2 u.
DeleteThw first urine test I took in bahamas was 847. Range was 82-500.the one hear for normetaneph is 257
DeleteRange is 82-500. Question is it possible for such a big drop.
This comment has been removed by the author.
ReplyDeleteWhat can mimic pheo
ReplyDeleteDear Christina,
ReplyDeletePlease read the post in July 2010.
Dr. Pheo
This comment has been removed by the author.
ReplyDeleteIs there a way to talk with you.or meet you
ReplyDeleteHi Doctor, I had my right adrenal surgically removed for a pheo. I am 30 years old and here are a few questions I have:
ReplyDelete1. After the surgery, the tests were redone in about 20 days and they came out fine. I have been monitoring my blood pressure and it seems to be going well too. Would you suggest I have another series of tests. My endocrinologist has only advised me to get the tests done again in a year.
2. Even before the surgery and for a long time now, I have this lump at the back of my neck. Knowing of the pheo, it now worries me if this could be an extra adrenal pheo. I can feel the lump but it doesn't hurt or cause me any discomfort.
3. For the last couple of days, I have been feeling slight pain on the left side of my stomach, just under the ribs. It is not a nagging pain, but just a slight pain that comes and goes occasionally. Is it something I should be worried about?
I am of the rare 10% who had reoccurring, but I underwent several genetic tests and all came back normal. I have had 3 reoccurrence within the past 10 years. If I were to become pregnant, being that I am only 28 years old, would I be considered a high risk pregnancy?
ReplyDeleteDear Holly,
DeleteIf there is no evidence of pheo recurrence at the time of conception, the pregnancy should have an average risk.
Dr. Pheo
I have been diagnosed with pheo, and am satisfied that the surgeon has put me through the correct tests, the last one being the MIBG scan. I meet with him tomorrow and will ask how many of these surgeries he has performed. Do you have any recommendations in the Little Rock, Arkansas area. Thank you
ReplyDeleteDear Anonymous,
DeleteYou may want to try Univ of Arkansas.
Dr. Pheo
Hello, I have had my pheo removed Nov 2017 and things were amazing for about 4 weeks. Now I am exhausted as in work at my desk and go home to bed sort of life, and dealing with huge depression issues. I have never had any depression before the tumor. Are there any post op effects of having a pheo removed that would be causing my troubles?
ReplyDeleteDear Joan,
DeleteAlthough most patients do excellently after pheo surgery, some have slow recovery and some others have new symptoms. The postoperative symptoms may be related to having a surgery in general, or may be specific to pheo surgery. You need to discuss with your doctors. It is important to make sure that you don't have adrenal insufficiency or residual pheo or other common causes of fatigue. You also should see a psychiatrist.
Dr. Pheo
This comment has been removed by the author.
ReplyDeleteThis comment has been removed by the author.
ReplyDelete