The 3rd International Symposium on Pheochromocytoma (called “ISP 2011” by the organizers) is being held in a Disneyland Hotel outside Paris. I am here attending this meeting. It is a 4-day meeting but most academic activities are concentrated on Sept 15 and 16.
The specific Disneyland hotel is kind of an odd choice of venue for an American attendee like me. I would pick a downtown Paris hotel on the left bank. Apparently my European colleagues are tired of Paris and prefer a place on a theme park. The hotel is full of little cute girls and boys dressed in Disney costumes, or pestering their parents to buy them a set or 2 of. The hotel is so serious about the Disney theme that you see Monsieur Mickey everywhere, including on shower curtain. At one moment today, I felt I was at home until someone said Bonjour to me. And a bottle of coke costs 3.5 Euros.
The meeting itself is simply fantastic so far. Today’s lectures and posters are actually very educational to me even though I believe I knew a few things about pheo before the meeting (I indeed presented a poster on pheo clinical care). Really great is the discussion with colleagues. All the big shots on pheo are here, including most doctors I recommended as pheo experts in a post 2 years ago. I am pleased to say that we share similar experience and opinions on many controversial issues. Patient support groups also have a prominent presentation.
The following is an incomplete list of new things I learned today:
1. Practical half-life of plasma metanephrines is longer than most people thought. Plasma metanephrines can linger for one month after complete pheo resection.
2. Most attendees agree on the importance of preoperative preparation even when the patient does not have hypertension at the time of diagnosis.
3. First-hand accounts of the discovery processes of the novel pheo genes TMEM127, SDHAF2, and MAX, by the discoverers themselves.
4. The 3 novel genes, however fascinating for studying pheo pathogenesis, probably have limited value in clinical care for most patients.
5. Apart from family history and clinical predictors, pheo markers and pheo tissue staining can also predict specific mutations.
6. No real progress in pathological diagnosis of malignant pheo. Basically even an expert pathologist cannot accurately tell if a primary pheo is benign or malignant.
7. Surgical indications of head and neck paragangliomas.
8. Still there are no commercial labs that can do plasma methoxytyramine in the US.
9. More and more people realize that pheo causes heart damage. I have observed that for many years and it is nice to see people now agree.
10. Some new vagaries of pheo. For example, cardiomyopathy can have a waxing-and-waning course even after phenoxybenzamine.
To be continued…
Dr. Pheo
Subscribe to:
Post Comments (Atom)
Thank-you for sharing this info! You're posts always insightful and I look forward to reading them.
ReplyDeleteHi Dr Pheo, I need urgent advice if you have time. I live in the uk and since contracting a severe kidney infection (1 year ago) which gave me blood poisioning, i had several episodes of severe high blood pressure and tachycardia which put me in hospital twice (each for 7 days). The episodes got worse and worse and closer and closer together, they could last from 10, 20 mins and the longest was 16 (yes sixteen hrs) on and off. I could not sit up in bed without my pulse going to 165 and my BP going into the 190/140. After several 24hr urine and blood tests (which had to go to Germany) my noradrenaline levels were twice the upper normal limit. I have had MIBG scan which was clear, Adrenal CT scan which was clear, NO MRI as i have metal clips on my fallopian tubes. I have been diagnosed with Autonomic hypersentivity and prescribed Clondine to reduce the production of noradrenaline - I have been on this for one month now and it was greatly improved the symptoms however, the last week i have had periods of an hr or 2 with high BP again. I still have pain off and on (lower right back - kidney/adrenal area). Is it possible that I DO HAVE a very active pheo that is very small which the scans did not detect??????
ReplyDeleteThe doctors have said that I have an enlarged right kidney (not surprised as this was the one that got badly infected). They have not elaborated.
I believe that it is possible that i have one in my right kidney which started the infection in the first place and given time the clonidine will have to be increased as more and more noradrenaline is released into my system. If this is the case, and i find i am having to increase the dose every month, should i INSIST on other scans (ie F18 Dopa Pet) say in about 6 months.
I would really appreciate your thoughts on this.
I am really worried they have missed the Pheo (ps one doctor at the hospital is really convinced i have a pheo and they have just not found it).
Dear Amanda,
ReplyDeleteThe overall probability of pheo is not high. I suggest that you measure urine noradrenalin again now that you are on clonidine. The metanephrines are better if available in your area. Let's see if the levels are lower or normal now.
Dr. Pheo
I'm in the process of being tested for a pheo and find your blog very interesting. I have MS and in the past year my BP/heart rate spike very high during certain times (200/100 was the highest.). the triggers for this are well defined, stress (a doctor's office will stress me)' heat, sensory overload (as in too much noise and commotion at the same time.). these are the same stimuli that will flare my MS symptoms.
ReplyDeleteI begged my GP to figure it out, anti anxiety meds do nothing, so we tried 4 different BP pills, and now I'm on clonidine which slows down my heart rate but does nothing for the bp during stress, etc. He ran a metanephrine blood test , 24 hour urine, and chromagin A. Blood test and urine were elevated a little less than double on most values...chrom A normal. I'm now seeing a renal hypertension doc, and he's curious because my dad has NF1....I have not been diagnosed with it. 24 hour BP test shows spikes in the 200 systolic range, a few times, once in the middle of the night, which sort of blows my trigger theory. Now I'm to have an MRI of adrenals.
So finally my questions:
1. I have multiple unilateral cranial nerve problems and read that a paraganglioma sometimes causes these. they've always been blamed on my MS, yet I have no brainstem lesion to account for them. How do they look for paragangliomas in the neck and how common is it for them to cause cranial nerve problems.
2. this drastic rise in BP (I've always had some white coat hypertension) seemed to come after I had an intrathecal baclofen pump implanted. I had to have a revision surgery last July and my BP was very labile the whole time I was in the hospital. Could there possibly be any connection?
3. Is there any way a brain lesion could be causing increased production of catecholamines...I also have vagus nerve problems, but everyone seems to think that would lower my bp not raise it.
sorry for the length of this...it's just that I'm having a hard time getting my questions answered.
In
PS...a question I forgot. Couldn't catecholamines be elevated just due to the day to day stress of a chronic illness...in my case MS. I know for me the un predictability of the disease coupled with mobility problems and the myriad of symptoms is stressful. My doctors seem to dismiss that idea...but hey, walk a mile in my moccasins and then tell me it's not a stress producer, LOL. Would increased catecholamines from stress cause the same type of symptoms as a pheo? (spikes in BP, heart rate increases, flushing, etc.)
ReplyDeleteDear stillsmyelin,
ReplyDeleteWith the borderline marker levels, the probability of pheo is not very high. Sympathetic dysfunction is common in multiple sclerosis and can cause erratic blood pressure. To answer your specific questions:
1. Head and neck paragangliomas usually do not cause cranial nerve problems. A neck CT or MRI will identify those tumors.
2. It is hard to say. There are reports saying that baclofen actually helps improving hypertension.
3. There are autonomic nuclei in the brainstem. Brain lesions can cause increase of catecholamines.
4. Daily stress also increase catecholamines.
Dr. Pheo
Dr. Pheo,
ReplyDeleteMy son, 13 at the time of first surgery, had a 8 cm plus pheo removed and his left kidney and adrenal gland after 2 surgeries. He is a miracle to be alive because they thought it was a neuroblastoma when they operated the first time and took no precautions on bp issues. Jesus was with us though and it was successfully removed after the second surgery all encapsulated. We went thru extensive genetic testing after that and found that unfortunately my son and daughter are positive for sdhb. My son did great at first but is now experiencing extreme fatigue and dizziness and changes in bowel movements.No one seems to know why. Can you please help? . Thank you so much!!
Dear Sharon,
ReplyDeleteHow old is your son now?
Dr. Pheo
Hello, I just got my results back. Plasma metanephrines 95 pg/ml everything else normal including all urine tests. I am highly symptomatic, as I cannot sleep at all... for about 10 years I woke up every night at 3am then Jan 2017 I started waking up after every 3 hours of sleep. In about 5 months it got down to 2 hours then less and less until I was able to sleep about 15 minutes before I would have an adrenaline surge and wake me up from the deepest sleep. This is the point when I went to an endo and got the tests done. During the day I collected urine I was reading about pheos and gave myself a horrible panick attack which took the last 15 minutes of sleep away. Now as soon as I close my eyes I have an adrenaline surge and get completely awake. The endo put me on Keppra which is giving me about 2 hours of interrupted sleep a night. Please let me know what do you think. Thank you very much, Adina
ReplyDeleteDear Adina,
DeleteI don't know much about insomnia.
Dr. Pheo