Fourth Annual Fireside Chat Ask the Doc

Dr. Karel Pacak, MD, PhD, DSc, National Institutes of Health, joins us for the fourth year in a row for an episode entirely devoted to answering questions submitted by patients. Part of the Pheo Para Alliance Education Webinar Series. Questions and timing are below: Q 1 at 07:25 “Can you give us any updates on any new research, treatments, guidelines, etc. that patients might need to know about that have come out since we spoke last year?“ Q 2 at 15:00 “Are there any novel treatments in the last couple years?“ Q 3 at 18:57 “I read a study about pharmacologic concentrations of ascorbic acid being a promising area of treatment? Are there any clinical trials underway? Is there any current data showing life expectancy for patients with metastatic PCC?“ Q 4 at 22:06 “Are there any changes to the periodic screenings for asymptomatic SDHB genetic disorder procedures?“ Q 5 at 33:48 “What do we know about life expectancy for patients with metastatic disease?“ Q 6 at 36:21 “Can you share your thoughts about Lanreotide treatment for inoperable paraganglioma?“ Q 7 at 39:45 “What are your thoughts on altitude and on whether it can be harmful for all paraganglioma patients, only those with SDH mutations, or only those with vagal paragangliomas? Q 8 at 45:10 “If I have an SDHB mutation and one or more of my children inherit the gene, how strongly would you recommend moving to a lower elevation? Will it really make a big difference?“ Q 9 at 48:41 “Can you be metastatic and on ’watch and wait’?“ Q 10 at 51: 30 “What are my metastatic pheo treatment options after PRRT?“ Q 11 at 1:00:02 “I’ve had 2 pheochromocytomas- so now have no adrenals. I was wondering why I continue to have elevated nor- metanephrine. I also have neurofibromatosis type 1 with numerous tumors, including many plexiforms in all levels of my spine. Could these numerous neurofibromas cause elevated catacholamines?“ Q 12 at 1:04:25 “Can you explain more about the use of beta blockers and ace inhibitors protocol pre surgery?“ Q 13 at 1:08:59 “What approach would you use for a paraganglioma close to aorta?“ Q 14 at 1:11:23 “What is the longest you’ve watched a patient with a vagal para that was stable and didn’t require surgical or radiation intervention?“ Q 15 at 1:18:58 “I have several surgical procedures in the future and I’m going in for a total knee replacement. I was diagnosed with two pheos one on my right adrenal and one on my left. The one on my right was removed by a right robotic adrenalectomy. The one on the left was left because they were unsure if they could do both. My question is two fold: 1) is there anything available to see if I have any more pheos - like in my bones as an example? 2) I have so many upcoming surgeries - repair a procedure that went wrong on my hand. Also they say I need neck surgery and lower back surgery with bolts and cages. I do not want - but if I had to go through that, could this left adrenal pheo be stable thru this?“ Q 16 at 1:26:40 “Could pheo/para be cyclic like Cushings can be and if so would this make a difference to testing“ Q 17 at 1:29:13 “What test to run for biochemically silent paraganglioma? My 24 hour urines are elevated about 1.5 to 2 times when having episodic episodes and just under 1 time elevated when not in an episode. So what other test would you recommend. My CT/MRI scans show liver lesions too small to characterize. I had a GA68 dotate that did not pick up any of my cysts and tumors I have on my liver and kidneys at all. The FDG/CT scan had uptake near the top part of the colon and uptake in the bone marrow and I now have 2 abnormal lymph nodes in the neck no can seem to figure me out? Q 18 at 1:36:52 “Why after almost 1 year after removal of the tumor and one adrenal gland, do I still have weakness, dizziness and heart pain? All my exams are normal.“ Q 19 at 1:40:18 “If chemo hasn’t worked on a pelvis tumor and they’ve recommended finding a clinical trial or the pill cabozantinib, do you have any recommendation? I have done chemo for 6 months and they say it isn’t working.“ Q 20 at 1:44:29 “What is the treatment for a spinal tumor?“ Q 21 at 1:48:48 “Do we have any long-term studies yet done for the long-term effect of high catecholamines?“ Q 22 at 1:51:58 “Can you explain a bit more about the correlation between tumor size and relative risk of metastatic disease?“