Although I am not a surgeon,
I am often asked by patients about surgical approaches. One of the common
questions is how laparoscopic pheo resection can completely remove the tumor.
The ports look small; how can a tumor 5-cm large pass through the ports? Even
if the tumor can squeeze through the ports, will that cause fragmentation of the
tumor or cause tumor spillage? I usually
ask the patients to direct the questions to the surgeons but I do wonder if
tumor spillage can actually happen.
Then I saw a case report published
this year. It described a 64-year-old woman with a large (12-cm) pheo. The pheo
was resected laparoscopically. The tumor was completely removed but the tumor
capsule ruptured. Tumor histology showed relatively high cell division rate but
otherwise there was no suggestion that the tumor was malignant. Several months
later, the pheo recurred at the primary site and the abdominal wall where the
laparoscopic ports were. The tumor progressed quickly and the patient unfortunately
died of it.
The references cited in the
case report listed an older study published in 2001. Three patients with single
pheos 5.5-6.5-cm large had small multiple recurrent pheos at the same site of
the original pheos 3-4 years after laparoscopic resection. In all three cases, tumor
spillage was suspected by the surgeons. No abdominal wall seeding was found in
the 3 cases.
In retrospect, the case
reported this year probably had malignant pheo while the 3 earlier cases probably
had benign pheo. At any rate, pheo spillage indeed appears possible during
laparoscopic resection but should be very uncommon.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteThank you for this very helpful blog which has helped me understand my diagnosis.
If you are able to answer the following questions, I would really appreciate it. Thank you.
My questions relate to normotensive pheos. I have normal plasma metanephrine and 3-MT, normetanephrine is about 7.5 times the upper normal limit with an adrenal mass around 4cm.
What would make an individual normotensive, despite having high biomarkers? Is that related to the size of the adrenal tumour, its position, a lower amount of circulating catecholamines and/or metanephrines, or, is there a difference in clinical manifestations or how the body responds when only metanephrine or normetanephrine is high?
Would high metanephrine/normetanephrine in normotensive individuals’ have any ‘silent’ systemic effects on the body, despite being asymptomatic and normotensive? And can someone normotensive still have other symptoms other than hypertension and if so, are there any indicators of this?
If normotensive with bioactive markers, should alpha blockade be started as soon as pheo is diagnosed or can it wait until preop preparation will commence, just 10-14 days (or should it be longer?) before surgery, as most of the literature seems to suggest? What is the minimum/maximum length of time one should be on these medications, if surgery is the plan?
Finally, do normotensive individuals find it harder to tolerate alpha blocker medications and is there therefore, a greater risk of syncope and/or postural hypotension and how can that be managed? Which medications are preferred for managing normotensive pheos?
Thank you.
Baffled Pheo.
Dear Baffled Pheo,
DeleteAll interesting and important questions. "silent" pheo means one of two things. It either refers to a pheo with normal pheo marker levels (biochemically silent) or a pheo with high marker levels but without hypertension (clinically silent). The use of "clinically silent pheo" is probably inappropriate and I personally object the use of this term because it is misleading. The mechanisms for normal blood pressure in patients with high pheo markers are not clear. Those patients tend to be young and otherwise healthy. They have a way of keeping blood pressure normal but their heart may have subclinical cardiomyopathy and they may suddenly present with heart failure or arrhythmia. When they take alpha blockers, they usually do not have hypotension. Like all patients starting alpha blockers, the medication should start as soon as diagnosis is made but the dose should be gradually titrated up every few days. The ultimate goal of alpha blockers in pheo is not to treat hypertension but to treat or prevent cardiomyopathy. The final dose should be one that gives a patient slight orthostatic hypotension after the intravascluar volume is repleted over 2 weeks or longer.
Dr. Pheo
Dr Pheo,
ReplyDeleteThank you so much for that very helpful reply. I agree the terminology - biochemically silent and clinically silent is very confusing.
There also seems to be a real dearth in the literature regarding clinically silent pheos, unless I've missed it? There seems to be very little written about how asymptomatic pheo individuals can still have quite serious effects without knowing. I'm guessing because very few people with high markers present without symptoms?
Baffled Pheo
Dear Baffled Pheo,
DeleteThere is indeed not much research on clinically silent pheos. The number of people with clinically silent pheo may not be small. About 40% of people with pheo present with adrenal mass incidentally identified, rather than with other classical symptoms.
Dr. Pheo
Hi,
ReplyDeleteI'm not sure I have come in at the right thread, but I just wanted to check something with you.
I emailed a while ago asking if you have neg bloods/urine if you can rule out Pheo, and you said yes. I have just realized that the tests my Mum had were 5HIAA and Chromogrannin A & B. I have been reading that the tests you should have are Normetanephrine and metanephrine....I don't think my Mum had these done. So based on that and the fact that she has a benign tumor on adrenal gland, would you still rule out Pheo?
Thanks, Samantha
Dear Samantha,
DeleteShe should do the metanephrines test to rule out pheo. Normal chromogranin A helps but is not definitive.
Dr. Pheo
Thank you
DeleteI am seeing a neurologist at UC Davis for hyperadrenergic dysautonomia with some significant symptoms being major fatigue, tachycardia; resting was 100-120 & at exertion upwards of 180, POTS, and what I described as "adrenaline surges" Those would entail a sudden peculiar sensation in the pit of my stomach, instant huge spike of HR, intense whole body sweating and flushing along with my skin would be very hot. Initially saw a cardiologist who confirmed inappropriate sinus tach with some PVC's during exertion. I was started on Bystolic and sent to the neuro. He ran a plasma metanephrines which they did supine and placed on ice. The normetanephrine range was 0.00-0.89. Mine came back at 3.55, metaephrine was normal. Calcitonin was normal. I have always had normal to low blood pressure. Beta blocker was switched to Clonidine with some improvement except for the "adrenaline surges" which happen upwards of 6-7 a day, half of them during the night waking me from a dead sleep. The neuro has now sent me to endocrinology and I have a CT scan this week, they are withdrawing my Clonidine to do a Suppression test, and withdrawing the H1 Histamine blockers for a 24 hour urine, urine metanephrines, cortisol and spot creatinine. I am also having a thyroid scan due to HX of Graves (2002) and hypervascular tiny nodule that was too small to biopsy. All blood thyroid values normal since 2003.
ReplyDeleteI don't present like a pheo and additionally I can specifically point to a sudden onset of these Adrenaline SX's 7 years ago during a septic Klebsiella Knee Infection followed by TKA, along with multiple surgeries (15) and infections due to eroded transvaginal mesh, reinfection of total knee, and major injuries from a car accident as well as I have Ehlers-Danlos. All the infections are presently resolved. CRP is normal as well as CBC and CMP.
Could there be another explanation for such high Normatanephrine levels? My only other meds are Lipitor and very minimal Norco.
I know this is rather complicated but this is causing significant quality of life problems for me and rather confusing.
Thank you in advance for your opinion.
The levels of normetanephrine are concerning for pheo but not very definitive. Let's see what the CT of abdomen would show.
ReplyDeleteDr. Pheo
This comment has been removed by a blog administrator.
DeleteDear Murali,
DeleteAs the iodine quickly is enriched in your thyroid, the dialysis should not matter that much.
Dr. Pheo
This comment has been removed by a blog administrator.
DeleteThis comment has been removed by a blog administrator.
DeleteDear Murali,
DeleteI note that you posted an email address. I deleted your posts for the sake of privacy. I do not communicate with readers privately. You can summarize the MIBG scan results here but make sure you do NOT put in any personal information.
Dr. Pheo
Dear Sir,
DeleteI am sorry to put my email-id, I dont know the protocol. I will ensure your advise in the future.
Coming to my MIBG Scan, Yesterday I was taken for 24hrs scan study and today also 48hrs study is done. After 48 hrs scan, The nuclear radiologist spoke to my nephrologist about a little shiny mass over the Rt adrenal area. He also advised to get my dialysis to be done today and then tomorrow will take me for 72hrs scan(final scan).
Once they issue the MIBG Report,I will share the report with you exactly with the same sentenses mentioned by the radiologist.
Today the nephrologist ordered for the metanephrines to test again. Sample sent today.
Tomorrow, they may ask to CT to get done.
As I already mentioned, I am suffering ESRD (End stage renal deseage) and so no urine out put.
I humbly request you to please guide me step by step how to go about the complete diagnosis and treatment.
Please mention if CT is better or MRI for the finding the correct location & size?
If any other things you want to bring out, please mention.
In INDIA, I dont know who is specialised in treating pheo's. If you can help me by sharing anything in this regard please help.
While going through your blog, I come across the post about high risk / low risk pheos. Now to classify my one, what information you need to know?
Hoping alot from your expertise.
Thank you.
Murali
Sir,
DeleteHere I am furnishing the SCAN Findings
- Scan reveals heterogeneous tracer concentration confirming to the shape of a kidney in the subhepatic region - non specific renal tracer retention.
- No abnormal focal concentration of tracer in the suprarenal region, abdomen or elswhere.
-Phisiological salivary glands, myocardium, liver & urinary bladder activity noted.
IMPRESSION:
- Scan is negative for any MIBG Concentrating neuroendocrine tumer.
And then the plasma metanephrine levels - 87.8pg/ml (date. 20/01/2016)
Delete96.8pg/ml (date. 02/01/2016)
Normal range is (90 pg/ml)
My symptoms in general:
Anxiety / little amount of strain to the brain increases BP. Feel like attack/ shock.
Headach, Heart palpitations, panic feel, Night sleep suddenly disturbs with heavy palpitations followed by bad dreams,
In cold atmosphre- feel like vaso constriction with high BP,
In hot atmosphere- feels heavy suffocation, sweating and pounding heart & heavy palpitations but HR <100.
When such episode starts in the brain, unable to manage the anxiety followed by Shivering at hands & feet.
Present medications-
betablocker(prolomet xl 50mg), calcium channel blocker (Cinod 20mg-BD),
Clonidine (Arkamin 0.2mcg-TID)
Prazosin (MinipressXL 5mg-BD)
BP readings: 130/80 to 180/110
So I request you to please guide me, how this could be treated.
I also wanted to send you the SCAN images. So please let me how that would be possible?
Thanks and regards,
Murali.
And then the plasma metanephrine levels - 87.8pg/ml (date. 20/01/2016)
Delete96.8pg/ml (date. 02/01/2016)
Normal range is (90 pg/ml)
My symptoms in general:
Anxiety / little amount of strain to the brain increases BP. Feel like attack/ shock.
Headach, Heart palpitations, panic feel, Night sleep suddenly disturbs with heavy palpitations followed by bad dreams,
In cold atmosphre- feel like vaso constriction with high BP,
In hot atmosphere- feels heavy suffocation, sweating and pounding heart & heavy palpitations but HR <100.
When such episode starts in the brain, unable to manage the anxiety followed by Shivering at hands & feet.
Present medications-
betablocker(prolomet xl 50mg), calcium channel blocker (Cinod 20mg-BD),
Clonidine (Arkamin 0.2mcg-TID)
Prazosin (MinipressXL 5mg-BD)
BP readings: 130/80 to 180/110
So I request you to please guide me, how this could be treated.
I also wanted to send you the SCAN images. So please let me how that would be possible?
Thanks and regards,
Murali.
Dear Murali,
DeleteThe MIBG scan results are negative. The plasma metanephrine results may be made a little lower by clonidine. Do you have the CT done yet?
Dr. Pheo
Dr Pheo,
DeletePlease suggest, CT for which region is needed to be done?
Regards,
Murali.
Dr Pheo,
DeletePlease suggest, CT for which region is needed to be done?
Regards,
Murali.
Dear Murali,
DeleteCT of abdomen.
Dr. Pheo
Is it possible my malignant pheo could be back 8 years later- even though my blood test is normal and I passed a MIBG scan?
ReplyDeleteI have all the symptoms... but we can't find it. My doctors can tell I am sick, but without finding it or any other diagnosis we are stuck.
I flunked one urine normetanephrines 5 fold that had acid in the jug. We redid it without acid and I passed 3 times.
Any thoughts?
Last time I was sick for a year before we found it on a CT scan at 2 cm. Does it take time to show up?
Thank you!
Dear Unknown,
ReplyDeleteIt the markers are normal for multiple times and your MIBG result is normal, you unlikely have pheo recurrence. Rarely but possibly, a malignant pheo may stops making the markers; if clinically indicated, FDG-PET is useful.
Dr. Pheo
Dr. Pheo,
ReplyDeleteOne abnormal 24 hour normetanephrine urine. A negative MIBG and PET scan, but my symptoms are so severe and gynecologist and endocrinologist can not figure it out. They say I look pregnant but it's not their area for the problems.
With my last malignant pheo 8 years ago, my first and most persistent sign is abdomen bloating/distention. It was there for the 18 months until my pheo was taken out and then went away overnight. This distention makes me look 8 months pregnant! The doctors are shocked by it. It came back and I am again severely distended and look 8 months pregnant AGAIN! My clothes do not even come close to fitting!! I've only had this once before and it was treated with a pheo being removed. 8 years later it's back.
I am also having scary episodes in the last 2 weeks ( 5 of them) where I feel like I am dying and am too sick to call for help. Normally I just get really sick, these episodes feel like doom has set in. They are short, but then take 20 minutes to 4 hours to recover from. It's like recovering from a seizure, it takes time.
I have orthostatic hypertension. Normally when laying down, my bloating is normal when laying down. As soon as I stand my pulse goes from 65/70 to 100-130. Once I am up for a minute it drops down a few points but never gets close to the laying down pulse again. The same with my BP- goes up and then drops a little but not too much.
The dr's start medicine but then I get too low and can not function. My high bp is 160/140. My low BP was after one of the scary attacks, by the time I got to bed and layed down my bp was 80/40, I was drenched in sweat, pale, nauseated, vomit in my throat, headache, dizzy, vision decreases, palpitations,, it's like the worst flu you could ever have only way worse!
I have daily headaches, struggle with sound and lights, chest pain on left side, chest pressure, sweats, hot/cold- changed quickly, BLOATING, After the attacks I get a metallic taste on my tongue and my nostrils heat up. I often get really hot flushed cheeks- I feel like my body is on fire.
Anne,
DeleteI am a pheo patient, too. A benign one was removed 10 years ago from my adrenal. Regarding the bloating:
Mine was recurrent or re-grown and last year had some of it resected, and it was tiny. The doctors were shocked when on the day of the surgery, right after, there was this bloating that made me go twin-pregnant in less than an hour. The pain was unbeleivable and it also felt like it was going to rip off my abdomen from the inside. I didn't let anyone touch my abdomen, even though I am used to managing pain.
This bloating took three months to go away. Who knows why, but it finally went away when I visited a GI, since all the other doctors were baffled about it. I explained to him my pheo situation along with the bloating problem and how it started. He gave me an antibiotic for the instestines citing a potential overbuild of certain natural bacteria that for some reason or another may have been caused by any one of the byproducts of having a major condition. We gave it a shot. The bloating stopped.
It could have been anything, but it almost looks as though a byproduct of the pheo condition may have caused this third-party problem, as severe, chronic constipation was one of the main symptoms associated with my first pheo diagnosis.
I know it's not much help. I really hope you find relief.
Thank you! What size is tiny?
DeleteSorry for the relative term! It was just under 2cm.
DeleteEpisodes can come on their own, or can also be caused by emotional stress (minor but still annoying), physical exertion- pushing a full grocery cart will cause an episode and half the time having a BM. The episode will start 30 min prior to having a BM and last about 1-2 hours afterwards.
ReplyDeleteI can't find or know who to go see next, Something is wrong! I am getting sicker and episodes are getting harder. My hair has just started to fall out the last 4 weeks. I was sick for Feb/March- got better and now since August I have been really sick.
I feel like I am a time bomb and one of these attacks is going to get me. After my last pheo was removed I didn't have any episodes and now this year is going down hill.
My bloating was my sign last time, and now with all these other signs.... what else is there to look at besides I have a tiny pheo we can't find yet?
Last week I woke up and had a large 3 inch red oval rash with an empty center on each side where my adrenal were/are. They were identical. They were large. no raised edges, no scales, really no itching, just huge red rashes- one over each adrenal. Started fading after 3 days and is lightly still there.
My family dr is working with me but does not know what to do. My er trip last week I had an EKG done. All blood tests and CT, MIBG, PET are normal.
Just the one 24 normetanephrine is 5 fold.
I am really sick and fighting to get better and stop these episodes. There is something about getting so sick and not being able to call for help and my BP was 80/40 by the time I was able to get help.
ReplyDeleteAny suggestions would be helpful!
My bloating starts as soon as I stand. I can feel the pressure just build. It gets the hardest right under my ribs from the bloating, which is also where the rash is. The pressure is so hard it feels like there is a baby on my cervix. It really only hurts when the skin is stretched so tight I feel like it will' rip open. It even is making my stretch marks grown and I've already had twins.
I agree I shouldn't have it if I am passing tests. But if you could see my stomach, all my symptoms, and the weird rash- something is screaming at me.
What else could it be besides my pheo is back?
I have had a thyroid work up- I passed.
I have not had my dopamine tested this time like I did 8 years ago.
Are the urine's accurate without the acid or they should be discounted? The urine I flunked was with acid, the ones I passed had no acid.
Thank you!
not sure how to change settings, unknown is the same as Anne :)
ReplyDeleteDear Anne,
DeleteI am sorry but I don't know much about abdominal bloating itself. Pheo can cause constipation but bloating is not prominent.
I recommend first establishing what is getting bigger inside the abdomen. If there is gas or fluid in the gut, then you can see a GI doctor. If there is another organ that is bigger, you can then see the corresponding doctor.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteAbout pheo spillage/seeding, I just wanted to add my personal experience with laparoscopic resection. I am a 36y/o female.
- In 2005, 4cm pheo was resected laparoscopically along with left adrenalectomy. I was advised that the tumor "broke" during resection.
- At the beginning of 2014, I was in the operating room for a laparotomy from the back, since metanephrine levels and MRI detected a 2cm pheo along with a 1cm "nodule" on the tail of the pancreas. Both were resected in that February day, but symptoms and metanephrines remained on the high side. This laparotomy was done by the same surgeon as the laparoscopy.
- By the end of the year I was under the care of an incredible doctor and pheo specialist (he's on your list). Further studies revealed pheo "pieces" seeded on L1 of the spine, the adrenal bed, and spleen. The new surgery, done in December of the same year, revealed some nodules on the splenic flexure as well and the surgeons decided not to proceed with resection. The surgeons spoke of metastasis and chemotherapy, while the pheo specialist spoke of potential seeding.
- Fast forward to six months after the surgery and with plenty of scans from the past year as reference, the growth on the pheo "pieces" is very negligible (les than 0.1cm).
I think we know what happened here. I don't blame anyone, since that first pheo was about to kill me and it was that resection that gave me a second chance.
Thank you for what you are doing for our community. I could write a book about my pheo story and how the lack of knowledge on the part of non-specialized health care professionals almost cost me my life several times before it was finally diagnosed in 2005 that what I had was a pheo. What you are doing needs to be spread.
Best regards,
Lucky Pheo Patient
Dear Lucky,
DeleteThank you for sharing your experience here. I guess spillage is not that rare anyway. Your experience strongly demonstrates that pheo and other rare diseases are better managed by specialists. Unfortunately the current health care system seems to consider all doctors are more or less the same.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteI am a 56 year old female.
During the seizures of my first pheo in 1989 (right adrenal, 6 cm diameter) my fingers, toes and the lateral side of my abdomen (waist) became blue (cyanosis). Together with the known symptoms like palpitation of the heart, extreme headache, sweating, bad vision, light headiness, pain in my back, vomiting, anxiety e.g. I read a lot on the internet and some articles about pheo’s. However I never read something about cyanosis as a symptom of pheo’s. Do you know if cyanosis is a known symptom of a pheo? And what caused the cyanosis in my case during the seizures of my first pheo?
My second pheo in 2014 (same location where my right adrenal has been removed, 1,5 cm diameter) emerged 25 years afterwards. My doctors told me this tumour usually recurs much sooner, if it does. What could be the reason the tumour recurred only after so many years?
And what is the reason reference values of plasma metanephrines are raised when one gets older than 55 years.
Thank you.
Dear Anonymous,
ReplyDeletePheo is known for causing constriction of blood vessels. Cyanosis of finger tips has been reported. I have not seen or read about body cyanosis but this does not mean it cannot happen.
The late recurrence probably shows the tumor is not too aggressive.
I don't known the explanation of the higher normetanephrine levels in older people.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteI am 21 and have been struggling for 5 years now. Nobody will take me or my symptoms seriously - apart from one doctor who suspected a pheo.
My symptoms:
Night sweats every night (soaks bed)
Hyperhydrosis and Hypertension
Sudden cold flashes (e.g cold extremities)
Hot flushes regularly
Adrenaline highs and lows, like rushes of energy and mood swings
Episodes of headaches that make my head feel like its buldging and hurt badly for few hours with high blood pressure (for example, 165/91 with 139 pulse ) and I have to lie down.
Feeling dizzy and losing vision going from sitting to standing (my mum is diabetic and my sister has POTs – postural orthostatic tachardia syndrome – im not sure that I have this though as my sister is very unwell with it, however I may have something autoimmune according to a few doctors)
I am always hungry
Excessively tired – nap during day if I can
Diarrhoea regularly
Shakey hands (essential resting tremor)
I am waiting to see a consultant again. I have had numerous tests suchs as x-rays, ultrasounds and 24hr catecholamine tests - my parents feel this is being done wrong as the collection bottle is see through with no acid in it – yet my sister with POTs was told by St Marys Paddington Hospital in London all urine collections should be done in a dark bottle with acid in otherwise the catecholamine’s will start to break down?
I would really just like a second opinion. All of these symptoms are making my life a misery and I feel at 21 I shouldn’t be sweating through my pyjamas and bed every night.
Thank you,
Emily, 21.
Dear Emily,
ReplyDeleteSorry for the late response. You can simply measure your plasma metanephrine levels.
Dr. Pheo
This comment has been removed by the author.
ReplyDelete
ReplyDeleteDlanor SekaoDecember 1, 2015 at 10:58 AM
Dr. Pheo.
I was diagnosed with Bilateral Adrenal Medulla Hyperplasia several years ago. My question is about Vitamin D and Pheochromocytoma.
Since Vit D3 up-regulates Tyrosine hydroxylase in the Adrenal Medulla , could correcting a very low Vit D3 level with D3 supplementation bring on symptoms of a full blown adrenal Crisis ?
My D3 level was 21 and after only a few D3 supplements my BP and Anxiety/Adrenaline along with all the other symptoms went through the roof after a few days of supplementation .
Thank You for any thoughts on this as I have not really seen it mentioned before.
Dear Dlanor,
DeletePlease see an earlier post on adrenal medulla hyperplasia. It is a controversial concept. I am not aware of vitamin D supplementation causing pheo crisis.
Dr. Pheo
Dear doctor,
ReplyDeleteHappy new year. fantastic blog.
i have a question: my mum (60 yo) has had hypertension and high rate crisis for two years (6 during the last month and several in may/july 2015). The duration is usually one hour (16-10 bp and 120-140 hr). Catecholamines in urine 24h in July was slighty high and normal (dopamine, adrenaline: upper normal range except noradrenaline: 87, nr: 80). The crisis come at night (when she is sleeping or as soon as she wakes up). Do you suggest further investigations about pheo or endocrine tumors?
Thank you very much
Further data: she is taking beta-blockers
DeleteDear Anonymous,
ReplyDeleteOverall risk of pheo does not appear to be high. Does she snore or have sleep apnea?
Dr. Pheo
Thank you doctor
ReplyDeleteShe snores (althought not in an exaggerated way). She is not obese and sometimews the crisis is at night before sleeping when she is calm. However she usually is anxious.
Also a strange thing happened . She has always had normal glucose levels in an analytical but after one of its hypertensive crisis , blood sugar was over 200. Doctors said it could be a lab error because the next week was normal again . I read that cos glucose spikes may be related to pheo . What would you suggest? thank you
DeleteDear Anonymous,
ReplyDeleteIs she has sleep apnea, it can cause some of her symptoms. Acute stress can raise blood sugar levels temporarily.
Dr. Pheo
This comment has been removed by the author.
ReplyDeletewanted to ask some questions regarding para/somata/polycythemia syndrome you mentioned two posts back. I don't know where to start. Sorry so long/convoluted. I'm concerned about a paraganglioma.
ReplyDeleteAfter a hospitalization in 2011 where I was in ketoacidosis with confections causing strep pneumonia, abx resistant e coli, h pylori, my discharge labs showed polycythemia. Fasting saliva insulin was low which led to glucose tolerance test that showed post prandial hypoglycemia. At that time I was concerned about an insulinoma & adrenal insufficiency. That sent my Dr down the rabbit hole of Cushing's. at that time my plasma renin was nearly 700 with aldosterone low normal so I was started on fludro to supress high renin. Meanwhile I still had high RBC & hemoglobin even though my ferritin was low. Told it was "my normal". I lost insurance. In 2014 a small pituitary tumor and beginning of empty sella noted but no biochemical proof of Cushing's. I was concerned abt a possible pheo/para instead of Cushing's but plasma & urine metanephrines and catecholamines were normal. I had been dealing with autonomic dysfunction but did not meet critera for pots. 4/2015 had a hysterectomy due to rapid worsening of endometriosis symptoms. Endo was so extensive pelvic wall, colon, both ovaries, uterous, one tube had adhesions. Woke up in surgical menopause. All masses considered benign. complications resulting in allergic sinus infection, infected tooth, a near shutdown of my GI system, high blood pressure, double vision, tachycardia, flushing, fits of anger and rage. was all chalked up to surgical menopause because surgeon had me wait 6 weeks to replace estrogen to starve of any remaining endo. Yet, after estrodiol optimal was still having bad symptoms. beta blocker for tackycardia caused increase & instability of hr and blood pressure.
I had an enlarged lymph node in left neck since 2011, chronic necrotic left inner cheek ulcer 2011, left breast mass 2012, accessory spleen 2013, & post surgery developed c5-6 radiculopathy pinching nerve in left arm, nerve pain in left leg/ft after hysterectomy.
did a 4th urinary metanephrine that showed elevated normetanephrine altho was not 4 times above normal. A nerve sleeve root cyst in sacral spine and an atypical hemangioma in c4 of neck near lymph was found. Sept after the complications had been addressed (including several cardiac tests and trips to er, one of which was metabolic acidosis with acute kidney decline) I had sinus surgery, lymphnode removal, and mouth ulcer rebiopsied, all benign. Yet, still had high RBC, hemoglobin, a rising alk phos. However I woke up from that surgery with near normal vitals!! Went from 100 mg metoperol to 25 causing bradycardia. Now I'm dxd w/ brady-tacky synd. cardiologist suspects pheo. also saw a hematologist about polycythemia. He said my EPO was inappropriately high considering the borderline high RBC and hemoglobin. I explained my 23&me results said I had a mutation in vhl linked to chuvash polycythemia. I also explained my family history of meninginoma, adrenal cortical neoplasm, metastatic stomach carcinoma, colon cancer, neurofibromatosis, breast cancer, cervical dysplasia, among others. I also forgot to mention from 2010-2015 my ca125 had been elevated and I'm hetero mthfr c677t.
I know pheo is unlikely but I really do think there are too many benign and Cancerous neuroendocrine tumors running in my family spanning last 4 generations. I'm not the only sick person in my family. My mother, as a matter of fact, has high gastrin and chromogranin a, and a very odd shaped stomach. I'm wondering if it's possible a head/neck para could present as atypical hemangioma in the neck spine or if the spinal cord nerve root cyst could be a neurofibroma or para and if it could be sporadically secreting or be an erythroprotein secreting tumor as part of that syndrome you discussed above. my endo has told me to drop the issue. What would you do in my position? Thank you kindly for your blog!
ReplyDeleteThis comment has been removed by the author.
ReplyDeleteDear Phephe,
DeleteYour situation is too complex for me to offer suggestions. For the concern of head and neck paragangliomas: these tumors usually do not cause pheo symptoms and they are usually in the soft tissue of neck rather than in the spine.
Dr. Pheo
Thank you very much for replying. You're right. It is too complex. I have ran out of ideas on what to do at this point.
ReplyDeleteHello Dr. Pheo.
ReplyDeleteI went to the E.R. a few weeks back for high blood pressure/heart rate (160s/110s) (Heart Rate 120s).
They eventually did an abdominal CT which revealed a 2.3x2.1 adrenal adenoma on my right gland and possibly a very small one on my left gland, that one may be fatty tissue though.
I have had a 24 hour urine test and a dexamethasone supresison test so far.
Here are the results of my CT and urine blood tests:
CT: FINDINGS:
There is a 2.3 x 2.1 cm right adrenal space-occupying lesion.
It has a mean CT number of 24 units on the noncontrast study.
It has a mean CT number of 73 units on the portal venous phase.
It has a mean CT number of 25 units on the 15 minute delayed scans.
The absolute percentage washout is 98%.
An absolute percentage washout greater than 60% is consistent with an
adrenal adenoma.
There is a faint very low attenuating area involving the left adrenal
gland, having a mean CT number of -58 units on the noncontrast images.
It may represent another adenoma in in the left adrenal gland or may
represent fat adjacent to the left adrenal gland.
The liver and spleen show smooth contours.
The liver has an overall decreased attenuation.
No focal lesion or abnormal enhancement is seen in these organs.
The gallbladder, pancreas, kidneys are normal.
Both kidneys show normal nephrographic phase enhancement.
The abdominal aorta is of normal caliber.
The bowel loops are of normal caliber.
The appendix is normal.
There is no abdominal or pelvic lymphadenopathy.
24 hour urine:
Metanephrine 36 - 190 mcg/24 h mine=181
Normetanephrine 35 - 482 mcg/24 h mine=914 (marked as high)
my record states: A four fold elevation of urinary normetanephrines
is extremely likely to be due to a tumor, while a
four fold elevation of urinary metanephrines is
highly suggestive, but not diagnostic of the tumor.
Measurement of plasma Metanephrines and Chromogranin
A is recommended for confirmation.
My cortisol levels in my 24 urine results: Cortisol, Free 4.0 - 50.0 mcg/24 h mine=137.0 (marked as high)
Cortisol(Free)24hr Urine ADULTS: 3.1-42.3 mcg/g creat mine=55.7 (marked as high)
Total Creatinine 24hr Urine 0.63 - 2.50 g/24 h mmine=2.46
My A.M cortisol after dexamethasone supresison results: 4.0-22.0 MCG/DL mine=1.4 (marked as low)
I'm a 35 year old male, I'm quite overweight and have diabetes type II. I don't know of any obvious family history of adrenal issues or glandular cancers, but two of my maternal aunt had under active thyroids and goiters removed, and my sister has PCOS and diabetes as well. The health network I go to is through the University of Pittsburgh.
My first appointment with my Endo is in May. Do you have any advice on what questions I should be asking her?
I'm new to this and kinda scared and I'm just curious for your opinion on these results and such.
Thanks,
Mark
Also, in the description of the mass, it seems to have a higher uncontrasted value yet a fast washout value. Is it more likely that this is a lipid-poor functional cortex adenoma rather than a pheo? And if so, what are the odds that that I could have them on both glands (assuming it is in fact another very faint adenoma and not fat?) Are the genetic disorders (MEN, etc) only associated with pheos rather than cortex tumors? I hope that all made sense!? -Mark
ReplyDeleteDear Anonymous,
DeleteYou also need to measure renin and aldosterone. You may need a clonidine suppression test to see if the elevated normetanephrine is due to pheo.
Dr. Pheo
Thanks for your reply Dr.!
DeleteI had been doing cardio exercises (squats and step) for the first time in a very long time right before my bp spiked - I don't think my heart was ready for that type of work out (I weighed 300 pounds.) Plus I had very bad anxiety from my hospital stay and ct results. I wonder if that could have caused those higher levels in the urine test the next day.
Dear Anonymous,
DeleteThey may.
Dr. Pheo
Just thought I'd update to say that I had my plasma metanephrines tested and they were in normal range. My endo is confident that it's not a pheo based on the imaging characteristics and my plasma results. It seems it was just a coincidence that I had slightly higher normatanephrines (likely due to my stress at the time) as well as an adenoma. I guess I would have to have those other tests done to be 100% sure, but I trust my endo's opinion.
DeleteAgain, thanks for your input Dr. Pheo.
-Mark
Dear dr. Pheo,
ReplyDeleteThank you so much for replying :)
I'm writing to you on behalf of my mom. She's almost 50 and has always been in good health.
Her symptoms are hypertension, which sometimes spikes, and general feeling of being unwell during the night and other times, but this is not constant.
She had som tests come back with 'slightly elevated' adrenaline and dopamine. I haven't got the actual test results with me, unfortunately, but to my understanding, a pheo is suggested when the results are more than just 'slightly' elevated? Apparently other factors are more likely to cause slightly elevated numbers.
CgA and 5-hiaa have been normal (except the 1st 5-hiaa, but for this test she hadnt been instructed about food/medicine she couldn't take - since then, two tests have had much lower results)
She has had MRI, CT and Ga-68 PET scan (sent her off to Switzerland for that last one). They haven't shown indication for pheo. Only abnormality was a benign cyst on one of the kidneys.
What do you think? Is pheo likely? What else could we be searching for?
Thank you se much in advance. /Louise
Also, she had endoscopy after the scans to look at panceas and the kidney I mentioned. Nothing noticed then either
ReplyDeleteDear Louise,
ReplyDeleteThe slightly abnormal test results and negative imaging studies make pheo quite unlikely.
Dr. Pheo
Thank you so much for you answer! You help so many people!
ReplyDeleteShe saw an endo this friday, who seemed to agree with you. They did an ultrasound of her thyroid which showed no nodules or ugly lymp nodes, so that's wonderful. It did reveal some inflammation, and her thyroid-antibodies were high, so now they're looking doing some tests for the thyroid function - so maybe that is causing her some of the symptoms (being either hyper- og hypothyroid). She will also be seeing an allergist, as she has a million sensitivities/intolerances, which may be causing her body a lot of stress.
Hello Dr. Pheo,
ReplyDeleteIt's been quite a long time since I've written.
If someone is suspected of having a pheo, would not putting the blood on ice, or not lying down after the plasma free test corrupt the results?
Thank you for all your kind help.
Best,
Frances
Dear Louise,
ReplyDeleteNice to hear from you. Those should not matter much.
Dr. Pheo
Thanks Dr. Pheo,
ReplyDeleteOne more, please. Do you have to be having an "episode" while the blood is being drawn for the plasma free or even the urine test? I always thought that a pheo/para would show up regardless of having an episode.
Thanks again for all your kind help with this cunning disease.
Best regards,
Frances
Dear Frances,
ReplyDeleteThe marker levels are higher during an episode. The marker levels are still abnormal when not having an episode. So we do not require the blood or urine collected during an episode.
Dr. Pheo
Thank you so much.
ReplyDeleteHello Dr Pheo.
ReplyDeleteMy questions are if a person has a neck lymphnode removed can that indicate if there is a head and neck paraganglioma through flow cytometry if there is 100% positive marker for cd57? If this same lymphnode was near the carotid bifurcation, butting up against a complex thick walled cyst in thyroid cartilage, as well as in a straight line across to a vascular tumor in the cervical vertebrae, is it possible for an unidentified para to be lurking even with all but one normal normal normetanephrines? I am guessing you will say very unlikely. Does it make it more likely if the mutation in SDHD gene related to familial paragangliomas and pheos is present in patient? Thank you
Dear Anonymous,
ReplyDeleteI am not aware whether lymphoma is associated with SDHD mutation.
Dr. Pheo
Thank you for answering Dr Pheo. No the lymphnodes were said to be negative for non Hodgkin's lymphoma. The flow cytometry however was listed as positive for cd57 in 100% of cells. I've found some references to cd57 as being a neuroendocrine marker used to check for metastatic spread to lymph nodes. Some of those references are about paragangliomas. I suppose my bigger concern is whether the vascular tumor in the cervical spine is possibly a paraganglioma. The terms "likely atypical hemangioma" on the mri it was seen on is vague and indicates it does not have the characteristic appearance of a hemangioma. With the SDHD mutation I thought that was a mutation that can cause head and neck paras as well as other neuroendocrine tumors. Is it possible for paragangliomas to be in spine or spread to spine and lymphnodes? Could the MRI interpretation be wrong?
ReplyDeleteDear Anonymous,
DeleteOn tissue slides, para and lymphoma look quite different. There are clear markers to tell each apart. Aslo lymphoma is usually further classified into specific categories. It is unlikely CD57 alone is used as a marker. You may want to ask your oncologist about the lymphoma diagnosis.
Dr. Pheo