Tuesday, May 29, 2012

Small and very small pheos


As the readers may have guessed, I have been extremely busy (due to increased workload) in the last few months. That’s why I haven’t written anything in the last 4 months. I found some time at the Memorial Day weekend to write this post.  

An interesting study was published in May about small pheos. Small pheos are more commonly seen in these days. In the last year alone, at least 5 physician colleagues discussed with me about small pheos. There is no official definition of a “small” pheo but the authors call anything less than 3 cm in diameter as small. I actually agree with the authors in the 3 cm cutoff. In the past, I reviewed the literature on pheo-induced cardiomyopathy and noted all the tumors that cause cardiomyopathy are equal or larger than 3 cm. An inquisitive reader may wonder why doctors only use one dimension in describing tumor size. On one hand, doctors are lazy and one dimension is easier to remember; on the other hand, most pheos are nice and round. I have yet to see an odd-shaped pheo, say one resembling the shape of a cucumber. Of course you never know.

There are 3 main findings in the paper. First, about 1/3 of all pheos in the last 15 years are “small” (<3 cm). Second, small pheos do not usually cause hypertension or other classical pheo symptoms but some small pheos ironically cause hypertensive crisis during an unrelated surgical procedure. So if one has a small pheo and hypertension, the hypertension unlikely gets better after pheo removal. I recently had a patient with small pheo whose blood pressure actually got worse after pheo removal. Third, small pheos have typical appearance on CT/MRI but the biochemical test results can be borderline or in the cases of very small pheos (i.e. <1 cm), even normal. In my earlier posts, I emphasized that most borderline test results are false positive. That statement is still correct but I need to add some qualifications now. If an adrenal tumor is small but has typical pheo appearance on CT/MRI, and the test results are borderline, it has a good chance to be a real (small) pheo!

Pheo is a humbling tumor. The more I know about it, the more careful I become when I make a diagnosis. I begin to question myself whether I was absolutely right when I told my patients with normal biochemical test results “You don’t have pheo.” They might still have a very small pheo. Now I use a buzz phrase “clinically significant pheo.” I tell my patients if they likely or unlikely have a “clinically significant pheo” rather than if they have pheo or not. Nobody can say for sure whether a patient has a very small pheo. The good news is that very small pheos are clinically insignificant.

Dr. Pheo

49 comments:

  1. Thanks for the update Dr. Pheo. We are still reading and learning. I am going for another scan in July. The only question I have now are about cysts. It seems that cysts are showing up in some of my families MRIs (pancreas, kidney, spine). Any insight on those? You have given great info on this blog. I understand what we are facing much better now. Have a great summer.

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    1. Dear Team Burns,

      Thanks for the compliments. Cysts in visceral organs (liver, pancreas, kidneys, etc) are pretty common. They may be sporadic or genetic. If a person has cysts in multiple organs, then the chance of genetic disease is much higher. For example, in von Hippel-Lindau disease, cysts can be seen in the kidney, pancreas, and spinal cord. Management of these cysts in von Hippel-Lindau disease depends on the individual clinical situation. Cysts in pancreas are usually benign while those in the kidneys can be pre-malignant.

      You too have a great summer.

      Dr. Pheo

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  2. Hi Dr Pheo, Sim from Australia again. Just got back some results from a Ga tate scan. Shows multiple (5 plus a small cluster) paras in adbo, pelvis and one in my neck. I have sdhb mutation. Is there any information about multifocal disease turning up in neck and abdo simultaneously? How likely is it to have paras show up in both places?
    Thanks again.

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  3. Dear Sim,

    About 30% of SDHB carriers can have head and neck paras (SDHD carriers have more such tumors). So it is not so uncommon for SDHB carriers to have tumors in neck and abdomen at the same time.

    Dr. Pheo

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  4. Thank you. I appreciate all your input. I was interested in your comments about pheos/paras being round(ish). I have had 2 long pheos. First one I think was caused as it filled all available space on one side of my abdomen. It was shaped like a mini football. Now I have one that is 1.5x3.6 in pelvis. I guess it might also be constricted so is filling available space. I would be fascinated if I weren't so fed up with the things :-)
    Thanks again.
    Sim

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    1. Dear Sim,

      Thank you very much for letting us know. I am very happy to have learned from your experience.

      Dr. Pheo

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  5. Dear Dr. Pheo,
    I am 42 years old male, Anaesthesia Technician suffering since 7 months with Paroxysmal attacks of Hypertension reaching sometimes (220/140)and Tachycardia (HR:180/bpm). However the frequency and intensity has been reduced now by itself. But I am also suffering from Excessive belching >100 per day. The Cardiologist suggested it is Labile Hypertension and Iam now on Atenolol 25mg O.D. I have consulted Endocrinologist, Gastroenterologist, Surgical Gastroenterologist, Medical Specialist, Cardiac Electrophysiologist but left with no diagnosis so far. Sir, I will present here my pheo markers results and scan result. Serum Metanephrines-82pg/ml(normal<90pg/ml); 24hrs. Urine metanephrines 0.218mg/24hrs.(normal <1.0mg/24hrs.); 24hrs.Urine VMA 7.18mg/day(normal 1-8mg/day); Serum Adrenaline 27.0pg/ml(normal <100pg/ml); Serum Nor-Adrenaline-526pg/ml(normal <600pg/ml); 24hrs. Urine 5HIAA-12.0(normal 0-15mg/24hrs.); hsCRP-1.23mg/L(1-3 Average risk); Ionized Calcium -1.21mmol/L(normal 1.10-1.35); 25 OH VitaminD 13.37ng/ml(Insufficiency 6-20ng/ml) Serum Calcium 7.9(normal 8.1-11); ALP 233mIu/ml(normal36-141); but repeated ALP result after 2 days was normal; MIBG Scan shows bilateral symmetrical uptake by both adrenal galnds and it could be due to Bilateral Adrenal Medulla Hyper plasia as per the radiologist otherwise there is no abnormal uptake of the tracer anywhere else. PET Scan with FDG is done with mildly increased FDG uptake is seen in small bilateral neck level II nodes-(likely reactive)and a 7mm sized nodule is seen in the body of left adrenal gland with mildly increased FDG uptake (max SUV 3.4)could be Adenoma as per the radiologist; CT abdomen with contrast media done earlier was also normal, Brain MRI was normal, Ultrasound ab-Pel was normal, ECG is normal,Pattern shows during attack is sinus Tachycardia, Echo cardio graphy is normal, CBC is normal, Electrolytes are normal, Lipid profile is normal, Cardaic Enzymes are normal, LFT is normal, KFT also normal, Creatinine clearance is normal, Renin is normal,Cortisol level is normal, Insulin level is normal. My major suffering is too much pain in the neck and sometimes vertigo and Excessive belching and occasional attacks of Hypertension and Tachycardia usually after food. Finally Iam referred to a Psychiatrist and he said I have panic disorder and he prescribed anti psychotic drugs. Please give me your precious advise, I will be ever grateful to you Sir.

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    1. Dear mr.baig,

      You unlikely have pheo with the normal pheo markers. The mild uptake of tracer in MIBG scan and PET is no evidence of pheo. Panic or anxiety is certainly possible after the unremarkable workup results. I suggest that you try the psychiatrist's medications and see whether you improve.

      Dr. Pheo

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  6. Dear Dr. Pheo,

    Thank you so much Sir for your kind suggestion. I am taking anxiolytics but I have a question to you. There is slight swelling at the back of my neck and there is pain too. my Serum Nor-Adrenaline level is in upper limit i.e; 526pg/ml(normal <600pg/ml). can it be paraganglioma in the neck which is causing sudden hypertension and tachycardia. there is bilateral increased FDG (tracer) uptake in the neck level II nodes. Please give your input. Thanx.

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    1. Dear mr.baig,

      The neck masses can be paras but they should not cause the hypertension. As at least one of them is painful, it may be a simple inflammatory lymph node.

      Dr. Pheo

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    2. Hello! I arrived at your site via a web search on Chromogranin A. I am a 55 yr old F with episodes of massive fluid filled diarrhea and waves of needle-like burning pain down and across both extremities. These episodes are also accompanied by HBP/LBP, headache, fear, neck pain, chest pain, cold intolerance and involuntary sustained toe contractures.

      Blood tests in chronological order: Normal urinary/plasma catecholamines/metanephrines except plasma norepi at 852pg/ml (range 80-520). VIP at 227pg/mL (range 0-60). VIP at 220.5pg/mL (range 0-60). Plasma norepi at 1725 pg/mL (range 80-520). Chromagranin A 7nmol/L (range 0-5). Chromogranin A 70ng/mL (range 0-90). I did/do NOT take PPI's.

      Imaging tests in chron order: "MRI of abdomen is inconclusive as unable to visualize R adrenal gland". CT scan of abdomen/pelvis showed "1.3cm complex cyst in head of pancreas, 1-2cm subpleural nodule L lower lobe and several small mm areas likely representing hepatic cysts".

      The biopsy via EUS states "smears are nondiagnostic consisting of blood and rare benign columnar epithelial cells compatible with ductal cells. Cystic lesion, head of pancreas".

      My wonderful young internist just died! He seemed to know what he was looking for and made a note in my chart that VIP and Chromo A are both tumor biomarkers. But, his partners, as well as other local MD's I visited, are unaware of the clinical significance, if any, of my recent medical results. However, my symptom episodes, particularly the fluid diarrhea, headaches, needle-like pain and toe contractures are increasing in frequency.

      I am confused as to any next steps. What are your thoughts????

      Very kindly yours,

      Delete
    3. Dear Presley,

      It is not clear at this stage whether you have a neuroendocrine tumor. I suggest testing urine 5-HIAA to rule out carcinoid. If it is negative, I would then consult a gastroenterologist to see if irritable bowel syndrome is possible. A trial of Sandostatin may also help.

      Dr. Pheo

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    4. Variable blood pressure/autonomic signs + leg pain/ pins & needles can be caused by small fiber neuropathy, no? Toe contractures wouldn't be due to small fiber neuropathy specifically but can be seen in other conditions that also include small fiber neuropathy. Maybe a work up by a neurologist is on order? (I'm not a doctor but I have small fiber neuropathy- spouse with SDHB/paragangliomas)

      Delete
  7. Dear Dr. Pheo,

    (I posted this under the previous subject but copied it here also)

    In regards to functioning vs. non-functioning pheos, I was wondering what causes some people in the same family to have non-functioning pheo's and others functioning ones if all are SDHB positive?

    You might remember my nephew, the fellow with Carney triad - his were not functioning. My twin sister and my grandson both had functioning tumors (my grandson had neuroblastoma) I take it my nephew's tumors would not show up on a urine test? How about the Blood Plamsa test? Would his tumors show on that test?

    Thanks again for all your help.

    Best regards,
    Frances

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  8. Dear Frances,

    I am sorry for my late response. I just returned from a vacation. Interestingly, whether a pheo or para is functioning or not is largely dependent on where it is. Adrenal pheos are almost always functioning. Bladder pheos are usually functioning. Retroperitoneal pheos are functioning in 50% of cases. Pheos from other places are usually not functioning. Pheo markers (urine or blood) in patients with non-functioning tumors are sometimes slightly elevated but they are not useful in clinical decision making.

    Best regards,

    Dr. Pheo

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  9. Dear Dr. Pheo,

    Thank you for the reply.

    I guess it just depends on where the darn thing happens to pop up. Since Eileen and I are/were identical twins, I often wonder when her gene went haywire and why I haven't presented with any tumors.

    I saw a paper where the NIH has found a way to basically fix the SDHB gene mutation using a drug cocktail. I'm not sure what their next step is, but I'm hoping they find the answer quickly.

    I hope you had a great vacation.

    Best regards,
    Frances

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  10. Dear Dr Pheo,
    Any thoughts please?
    I was previously a fit and healthy woman ( 39 when it started) but three years ago i started getting strange 'waves' with palpitations. They would come on without any warning and slowly taper off. Sometimes several a day ( up to 20), other times one a week. This was the start of the worse three years of my life. They would range from small waves to full blown attacks- with shaking and feeling like i was going to die. The heart thuds I experience as it starts and then the attack happens. I am left fatigued after. The other symptoms are weight loss despite eating which alternates, nausea,constant pallor, heart palpitations, anxiety, headache, to name a few. Whatever this is seems to be affecting my whole body in some way or another. I only have occasional night sweats, i never sweat during the attacks and never have sweaty palms. I have cold hands and feet and numbness and 6 months after the attacks started I have had awful right flank pain and now have left flank pain too which comes and goes. The only definite trigger I have found for the attacks is positional. If I am sitting with my feet up or lying down it happens. Sleep is near impossible as soon as i lie down i feel ill. I have now got awful rib pain on the right side if i lie on that side and back pain.
    What I have found is that as soon as I lie on my sides ( not standing, sitting or lying on my back) that my BP falls to 70/40 and my pulse lowers too. No amounts of fluids will bring my BP up, the only thing that does is if I stand. During a big attack my BP was recorded in hospital as 243/133, HR 183. Plasma Noradrenaline was recorded as 1314 pg/ml( normal 200-500pg/ml)and plasma adrenaline as 259pg/ml ( normal 20-150pg/ml).
    CT scan showed two small cystic lesions in the Liver and a hyperdense area in the dependent portion of the gallbladder only. Neck MRI showed agenesis left lobe of thyroid. all the results they say insignificant. NM MIBG(123) scan showed faint uptake in region of left adrenal and SPECT CT showed low level activity of both adrenals only.
    24 hour Urine tests were negative ( not taken during a massive attack) and all routine blood tests have been within normal ranges.
    I am at a loss as to where to go and would just like to ask your professional opinion to ask if the plasma noradrenaline, symptomology etc is ok to exclude a pheo or similar. i was under the impression that a pheo would cause constant hypertension rather than hypotension when lying on side and raised BP during an attack?
    Many Thanks

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    Replies
    1. Dear Locksmith,

      The current lab test and imaging results suggest that pheo is not very likely. If possible, please measure plasma metanephrines. On the other hand, your symptoms are consistent with pheo. Pheo can cause episodic hypertension and hypotension. These symptoms are not specific to pheo. Other diseases can cause the same symptoms.

      Dr. Pheo

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  11. Dear Dr Pheo,
    Many thanks for your quick reply and professional opinion.
    I do not think It is possible where I live to have plasma metanephrines done.The strange thing is it feels like someone turns a tap on and I have a release of adrenaline or something. I find myself looking at my hands as they don't feel right and are cold. Exercise or heat can make me worse. I have become very drug sensitive and propanalol made the palpitations/ heart thuds much worse. I don't know how I am going to be from day to day and even noises can make me feel on high alert, mainly when I am sat or lying, but then other times I can have a few hours of normality. The nausea and just feeling ill is awful.As you say other diseases can cause this also. I have had autonomic tests and they are fine which is one relief.
    The clinical psychologist and psychiatrist ( anxiety angle) have said organic but unfortunately my endocrinologist says not organic. I have now got the MUS label and so have little hope of a diagnosis.
    Thanks again for your valued opinion.

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  12. Dear Dr. Pheo,

    Great job on maintaining this blog.

    My husband (healthy,31 years old) recently underwent a surgery to remove 11 cm tumor in adrenal gland and liver.

    Background: The tumor was an incidental finding and till surgeon opened him up, he thought it was a liver tumor. When he was undergoing surgery, the surgeon notices very close proximity to right adrenal gland and that his BP was fluctuating whenever he touched the tumor. The surgery went fine. It was diagnosed as pheo in histopathology report. The tumor had invaded right liver and few blood vessels. Surprisingly, the tumor was taking supply from hepatic artery and not adrenal artery. His PET scan (done before surgery) did not show metastatic and he did not exhibit any symptoms of pheo (BP, palpitations etc). He is scheduled to undergo mIBG Scan in October.

    In light of all the above facts:

    1. Do you think its benign or malignant?
    2. In either case, what is the prognosis?
    3. Is there any way to ascertain the chances of recurrence or behavior of tumor (malignant or benign)?

    Looking forward to hearing from you.
    SM

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    1. Dear Shveta,

      The 3 questions are related. It is not necessarily malignant. The pathology report is the most important document here. It should clearly describe whether the tumor simply expands and grows against the liver (then more likely benign) or the tumor infiltrates inside the liver (then more likely malignant). The other parameters are the mitosis and Ki67 labeling index (both markers of cell proliferation). Even the pathology report can not be viewed as definitive (there are no universal criteria on pheo malignancy by pathology). The only reliable indicator of malignancy is recurrence on the same side or remote metastasis.

      Dr. Pheo

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    2. Dear Dr. Pheo,

      Thanks for the reply.

      The pathology report says : The liver shows an invasive pheochromocytoma invading into vessels and liver parenchyma. The tumor is composed of sheets and irregular invasive islands of spindle cells with moderate to severe nuclear pleomorphism and granular eosinophilic to amphophilic cytoplasm. The mitosis count is 1/10hpf. No necrosis is seen.

      Regarding the Ki-67 index, it says its less than 1%.

      Can you help me understand what it all means? Also what do you mean when you say tumor expands and grows against the liver?

      Looking forward to your response.

      Thanks.
      Shveta

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    3. Dear Shveta,

      The pathology report does suggest the tumor is invasive (grows into the liver rather than against the liver). It is important to assess whether there is residual or metastatic pheo. I recommend testing plasma metanephrines and FDG-PET in October. The MIBG scan and FDG-PET are often complementary.

      Dr. Pheo

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    4. Hi Dr. Pheo,

      Thanks for clarifying. He is done with metanephrines test (plasma metanephrine is not available in Singapore so he went for 24X7 urine test). The urine test says normal. Our doctor says this means that there is no residual tumor. Do you also think the same? Also, is plasma metanephrine is a better judge than urine test?

      He is going for mIBG in October. Since you have mentioned mIBG and FDG-PET are complementary, should he go for FDG-PET also. If yes, what is the right time for that? Please note that he did a FDG-PET before surgery and at that time metastatis was not observed. Please share your thoughts on the status.

      Delete
    5. Dear Shveta,

      The negative urine metanephrines are reassuring. The plasma and urine metanephrines are about the same. The FDG-PET may not be necessary now if it was done before surgery.

      Dr. Pheo

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    6. Thanks Dr. Pheo. I will seek your opinion again once he is done with mIBG.

      Shveta

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  13. Dear Dr Pheo,
    Just a couple of questions..(sorry)
    When the plasma catecholamines ( noradrenaline and adrenaline) were taken, would it effect the reading if they were taken on a ward and then took some time to get to the lab? Does the sample breakdown at all? I am not sure of the time span for this.
    Also if I managed to get the plasma metanephrines done privately would they need to be the 'free metanephrines' and if so does this sample need to be taken when I am symptomatic or not ( this would be hard to arrange) and also does this sample need to be frozen straight away? Sorry for the extra questions.
    Also I forgot to mention, my saliva cortisol levels throughtout the day and night are elevated apart from the noon sample and my insulin levels (saliva), fasting and non fasting are depressed despite my HbA1c and fasting plasma glucose being normal but they say this also insignificant?
    Many thanks for your expert opinion.

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    1. Dear Locksmith,

      1. The time to the lab is usually not a problem.
      2. The metanephrines are "fractionated free". The metanephrines are very stable and the blood does not need to be frozen. The blood can be drawn any time, symptomatic or not.
      3. Cortisol is the only reliable hormone that can be measured in saliva. Please talk to your endocrinologist about the cortisol.

      Dr. Pheo

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  14. Dr. Pheo,
    Hi, I am 47y.o female with recurrent metatastic pheos/ paras. My first dx was in 1974 with policythemia vera which has now been recently changed to policythemia due to not having the Jak2 gene nor axon 12 gene. 1st pheo removed from lt adrenal in 79. Hypertensive and all the other regular signs of pheos but drs said were from the poli. 2 and 3 rd operations in 2011 to remove several more pheos. I still have several in me. I test negitive for sdh b,c and d. Have not been tested for VHL as not having any head tumors. Most of my more recent ones have been along the parasympatic chain but more are coming up in rt adrenal, liver and spinal lymph nodes. Any ideas on pheos and policythemia together as a life long dx?

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    1. Dear lori,

      What a coincidence! I will put up a new post today specifically commenting on your condition. It sounds like though that your polycythemia has a clear cause, Jak2 mutation. It is hard not to think that your polycythemia and pheo are not connected, considering that both occurred at such a young age. I am not aware of any mentioning of Jak2 in pheo/para. Mutations in VHL, PHD2, and now HIF2A (to be discussed in the new post) are all possible and should be screened. I suggest that you contact the NIH researchers and they likely will include you in a study so that you can have all the mutations screened for free.

      Dr. Pheo

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  15. Dr Pheo,

    I test neg for Jak 2 and axon 12. I have been to 2 major hospitals in Ohio and NIH and still have Drs wondering what is going on. Current tumors are still causing typical problems but I have alot of over lapping symptoms since both illnesses cause similar symptoms. Current hemotologist now refuses to due plebs since they have ruled out Jak2 and seems to think that cts will go down when remaining paras are removed which is not going to be anytime soon. I have several drs that seem to think I am an accident waiting to happen yet they are all to happy to pass me off to another dr who in turn passes me off yet again. Thank you for your quick response

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    1. Dear lori,

      I misread your message on the Jak2 part. Please see my new post. I recommend that you contact the researchers who wrote the paper. Your conditions are almost identical to those of the two patients described in the paper.

      Dr.Pheo

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  16. Dr. Pheo,

    After a decade of heart palps I finally got a DX of a pheo in 2010 and had pheo just under 3cm removed that same year. In 2009 I had strong suspicions that I had the tumor, but my 24hr urines were 4X normal for norepinephrins and normetanephrins, but not always high for epinephrines and metanephrines, so it took a year to get my endo Dr. to do a catscan and MIBG. In any event, in my post op follow up, I continue to have low epinephrine and metaneprhines - but now much lower, below detectable levels, but normal norepinephrines and normetanephrines. I have had 4 post op 24 hr urine tests and these epi and metanephrines are always below detectable levels, and are reported out as Low. So I keep asking my endo Dr if this is common and can't get a straight answer. She said low levels of adrenaline are not clinically relevant. Post op, I immediately had dermagraphic urticaria and continue to have it, which I never had any skin issues before removal of the tumor. The endocrinologist says these are unrelated to my adrenaline levels, but she does not communicate well, so was wondering if you could shed some light.

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    1. Dear Unknown,

      Undetectable epinephrine and metanephrine are not uncommon in patients after pheo removal. Frankly I don't know if it is because patients now only have one adrenal gland or not. The clinical significance is not clear. I am not aware of any reports on skin issue after pheo removal.

      Dr. Pheo

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  17. Thanks Dr. Pheo,

    You are the first Dr. to answer that question. Thanks it is good to know this does happen in any event. My doc could not answer the question and just keeps telling me low adrenaline is not clinically relevant. But I think it is, although not seriously so as I will explain below.

    So, related to the question above about skin rashes, specifically in my case, dermographic urticaria post op. I have a medical science background and work at Stanford to help scientists translate their science to healthcare products. So I researched the noradrenaline to adrenaline conversion pathway and found that SAM-E, vit. A and C are the main cofactors to convert to noradrenalines to adrenaline. I was advised by a Baylor SAM-E researcher that Nature Made SAm-E is one of the better brands. I have gone off and on the supplements several times, and the afternoon/evening daily dermograhic urticaria rash stops appearing every time I take the supplement. When I go off the daily rashes that appear in afternoon and evening come back. I have done this 6 times now and it happens the same each time. So I think there is a relationship between low adrenaline and dermographic urticaria.

    The second thing is a possible noninvasive diagnostic for pheo. In concert with symptoms of my pheo, I started getting a deposition film on the back of my cornea. This began about 2004, 4 years after heart palps. Every year the film got larger, and then in 2 years, could be seen in the other eye. I saw several ophthalmologists, all had never seen this before, including the leading cornea doc in SF, Dr. Goodman. Every year it grew. Two months after pheo resection in 2010, I saw my optometrist, who first discovered this, and he said the deposition film was much, much smaller. Then again I had it checked by both my optometrist and ophthalmologist just recently and it was now very difficult to even see - almost gone. The film was always fairly benign as every Dr. said it did not look threatening - it was always faint, but once you looked carefully, my eye Drs. could see it and could see it spread every year. So I am wondering if this is something that could be used for early detection of pheos. The loss of the film so quickly after resection just begs the question is this related and could it be a noninvasive diagnostic for all pheos. I think it is possible that this film deposition if overlooked in most people. It is subtle but evident once you recognize it, so I am told.

    Best,

    Gregg

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    1. Dear Gregg,

      Thank you for sharing your thoughts and experience. I will begin to pay attention to urticaria and corneal deposition in my patients.

      Dr. Pheo

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  18. Hello Dr. Pheo

    I am 34yrs female and had been diagnosed with hypertension in May 2013. I had a 24 hr urine test done. Results: epinephrine is 12.87 ug/g creat; norepinephrine is 3754.64 ug/g creat and dopamine is 319.18 ug/g creat.

    Based on these results my endocrinologist recommended an MIBG scan which came up with no evidence of pheo found. Subsequently I underwent a PET/CT scan.

    i will quote the results : "No abnormal FDG uptake is seen in both adrenals. Nodule is noted on medial limb of right adrenal that measures 9X7 mm having fat density on CT - likely to represent an adenoma. Left adrenal is normal."

    Based on this my endocrinologist is still suspecting a very tiny pheo.

    i continue to monitor my BP on weekly basis. Today it was 140/90. I am taking medication for the same.

    So what is your opinion on this- is this more likely to be a pheo? Could I have anything else? What should I do next?

    Thank you. Look forward to your response

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  19. Dear Baljeet,

    You unlikely have pheo. Other causes of hypertension should be tested.

    Dr. Pheo

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  20. Dr - thanks in advance for your response. I have metastases in the Liver (multiple lesions in the right lobe) and the Celiac Lymph node. No symptopms. Have done multiple scans, endoscopies - CT, DOTONAC, PET MRI and unable to find primary. Have done multiple blood markers - serotonin, 5HIAA, PP, CgA, Gastrin. CgA and PP both come out considerably elevated. Biopsy of liver suggest slow growing G1. Our Drs have concluded NET and like pancreatic. We are now moving to treatement and debating surgery of secondaries vs. chemo. Appreciate your perspective on the diagnosis and treatment options

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    1. Dear Anonymous,

      You have metastatic NET of unknown origin. Most likely primary sites are small intestine and pancreas. You will be better served at a center with NET experience. The best treatment options are based on the specifics of your tumor. Chemotherapy unlikely will help. NETs of G1 grade do not respond well to regular chemotherapy.

      Dr. Pheo

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  22. This entry was of particular interest to me. I'm 56 and have just been diagnosed with a 1 cm paraganglioma in my bladder. It was removed via resection (no adverse BP problems, but had twilight and spinal anesthesia)and sent to pathology. Was initially diagnosed with high-grade, invasive, urothelial cancer with micropapillary features. Went for a second opinion at UCSF and was diagnosed correctly with a paraganglioma.

    For years prior, I had "attacks" of sweating, flushing, tremors, nausea, weakness, dizziness and sometimes headaches usually brought on by exercise or getting overheated or sometimes by BM/micturition. But no high BP. Actually, I have low BP most of the time. Even perhaps POTS.

    In any case after removable by TURBT, on June 28, 2016, I have still had a few attacks although less frequent and less severe. Plasma metanephrines after tumor removal (two months after) were normal.

    Going in for another surgery to scoop out bladder area were tumor was. Endo wants me on phenoxybenzomine.

    I guess my question is, can a 1 cm para cause my symptoms, even though no high BP? And can one still have some symptoms after tumor is out for a while while body is "adjusting"?

    P.S. Getting a DOTATOC next week. PET scan was clear.

    Thank you for your time and valuable feedback. Much appreciate for such a busy doctor.

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    1. Dear Lori,

      I doubt that the small bladder para caused your symptoms. Patients usually don't feel much difference after resection of small pheo/para.

      Dr. Pheo

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    2. Lori, hi, I am just wondering, did you have your surgery at UCSF?

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    3. Dear Dr. Pheo. I'm actually now quite positive my symptoms were from my paraganglioma. My symptoms, though continued for a short time after the tumor was removed, were less frequent and about two and a half months out ceased altogether. My DOTATOC was clear. Now, almost a year later, I have no symptoms and have never felt better.

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    4. Hi Jan. Sorry for the late reply, I never got notified of this question. I had my initial surgery to remove tumor at UC Davis and then a follow-up surgery to make sure margins were clear at UCSF. My Urology surgeon suggested I get a partial cystectomy, but I opted for a removal of surrounding tissue which was done through the urethra.

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    5. Dear Lori,

      Thanks for sharing.

      Dr. Pheo

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  23. Dear Dr Pheo,
    I have a small left adrenal pheo that has very violent tendencies. My issue is that I can not find anyone to remove it and my borderline Mets does not help mattwrs. I'm positive my Mets get higher but we never test at a proper time. I've been on the east and west coast looking for a surgeon or visiting Doctors who claim they can help. Only to be sent home with the same tests I've taken 50 times. They hope they can catch my higher levels bit they only happen in short acute moments of stress or sickness. Can you please direct me to someone who knows about these and can handle a small one. My daughter is not well either and has borderline Mets too. I need to speak more about her too. We have got to find someone who knows these tumor . I can't sleep or function most days. I experience extreme bp fluctuationsas well as intense and unpredictable heart rates. I'm a 39 year old female and I live in South Carolina.I've knownot my tumor location since February 2016 from mibg, spect and CT.and I've been very useful well for over 13 years. Please help me

    Sincerely,
    Jan

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  24. Dear Jan,

    Based on your description, you unlikely have a pheo or a small pheo. A small pheo usually does not cause symptoms unless it is agitated.

    Dr. Pheo

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