I just returned from the NANETS meeting. Before the meeting, I had hoped that I would get to meet colleagues interested in pheo and see new studies on pheo at the NANETS meeting held in Santa Fe, October 29 and 30. NANETS stands for North American Neuroendocrine Tumor Society. It is a very nice society of doctors who treat neuroendocrine tumors. Pheo is a neuroendocrine tumor. Although the meeting was very informative on carcinoid and pancreatic endocrine tumors, there were literally only one study on pheo and one talk that mentioned pheo in passing.
The one study by a researcher in Dallas essentially showed that the mTOR pathway probably is not particularly important in the transformation from benign to malignant pheo and mTOR inhibitors (such as RAD001) probably won’t work for malignant pheo. The talk on modern gene testing by Dr. Marx at NIH briefly mentions the controversial issue of prenatal diagnosis of MEN2 (I had an earlier post on this topic in this blog) but did not delve further into pheo gene testing.
Dr. Pheo
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Dr. Pheo,
ReplyDeleteWhat is this report called that discusses the mTOR pathways for pheos? Who are the authors?
I'm taking a drug called sirolimus as I'm a liver transplant recipient and the thought was that this particular immunosuppressant might be inhibit the growth of new paras via the mTOR pathway.
Take care,
Dear Tisa,
ReplyDeleteI don't know if it is published or not yet. It is called "Effect of inhibition of mTOR pathway on growth of human cell line derived from pheochromocytoma tumor". Author: Hans Ghayee.
Dr. Pheo
Hi could caffine trigger an attack ????
ReplyDeletedear dr pheo,
ReplyDeleteyou mention in an much earlier post that you welcome questions which might become the topic of a future post. i have a question which may or may not be of interest.
do steroids and the withdrawal thereof cause high readings of 24hr urine catecholamine results?
6 months ago had a very elevated dopamine reading (4x normal max). that day i was very unwell: anxiety, heavy vomiting, diarrhea, headache, dizziness sweating, trembling, cold... a few days earlier i had stopped taking prednisolone tablets (40 mg for a week) and seretide 250 asthma inhaler. i had also had a steroid injection a week earlier.
i was taking all this medication for suspected asthma, replaced by suspected anaphylaxis. these diagnoses were both dropped after the consultant witnessed two attacks and started mentioning pheo. i had been taking the seretide for 2 years and every time i changed the dose, i experienced these symptoms that i would call "withdrawal symptoms". i also had these symptoms every time after taking prednisolone for 5 days.
(i have not had any other extreme catecholamine value since.)
i am sure you have lots of pheo queries already, but maybe somebody else wonders about the role of steroids.
many thanks for your fantastic blog; it is interesting and you sound so approachable:-))
indi
Dear celinabarnes2,
ReplyDeleteCaffeine stimulates release of catecholamines from pheo. The mechanism is not very clear.
Dr. Pheo
Dear indi,
ReplyDeleteThat is a great topic. I will write on it in this month's post.
Dr. Pheo
Dr. Pheo:
ReplyDeleteMy question is two fold: As you may recall, my son had Neuroblastoma and a SDHB mutation. Doing very well! What is the significance of once of these types of tumors not picking up on MIBG or having a rise in catocholomines (sp) ??
Secondly, as my son has already been dx with NB and and has a positive SDHB point mutation, could you tell me how often you would scan? Since we are not only looking for NB but for pheo, (I know you don't give advise for NB!)
My very best,
Kate
To dovetail on Kate's question on scanning. For children who do harbor a mutation that could lead to the development of pheos - at what age would scanning become appropriate? The risks associated with biochemical monitoring is a slight possibility of infection at the puncture point and a crying child. CTs and nuclear scans are not without some risk and some in the oncology field wonder if these scans (especially being done repeatly) may be nudging forward the development of cancer in patients.
ReplyDeleteDear Kate and DJPheo,
ReplyDeleteSorry for my late response.
I don't know enough to answer Kate's first question.
For Kate's second question, it is a bit complicated. For all comers with SDH mutations, the general recommendation is biochemical screening every year and imaging every 2 years. The starting age is controversial. As DJPheo observes, screening is not without risks. We need to balance the benefits and risks of screening. The traditional wisdom is to start at 10 years old. Case reports have shown early para in a 9 year old. In Kate's child's case, he already has a neuroblastoma and will need follow-up, thus he probably will start early screening anyway. It will be inappropriate for me to give specific suggestions regarding his screening regimen. I suggest that Kate talk to her child's doctor about it.
Dr. Pheo
Test comment - someone was having trouble commenting here.
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