SDHB Positive and Ga-68 Dotatate Scan question.

Hi. I'm sdha positive with advanced metastatic paraganglioma that started in the abdominal area. I'm told the GA 68 scan is the best for finding tumors that show somatostatin uptake. Since your tumor is in the head/neck area I believe the research shows those will not likely metastasize to other areas of the body. Are there plans to remove the tumor? Are you having any of the classic symptoms of a functioning paraganglioma such as high blood pressure, headaches or sweating?

Highest blood pressure I have had to date is had is 247 over 185, you can feel your hair grow literally. Heart rate can go up to 190 and drop back in the double digits. GA68 did not find anything below the shoulders. Extremely episodic though. No pattern. I have had ultra low blood pressure since childhood, 105 over you can barely hear, two different types of migraines, developed a third one in the last 20 months.
Yes plans to remove the tumor. I do want to find out what makes that thing tick or what else besides the tumor can spin things up like that.
Dumb question on your SDHA, is yours hereditary? What kind of a mutation? When you found out, did you test for other mutations, non NEC? I have been told there are 80 some of them. SDHB mutation can trigger kidney and GIST cancers. They just removed three cysts from my kidneys past Friday. Sorry for the million questions and thanks very much in advance. V

Yes, mine was inherited. Genetic testing ordered by the endocrinologist showed a mutation in sdha 91 ct. Considering your symptoms, I would think your tumor is a functioning one, meaning it’s secreting hormones that are messing with your cardiovascular system. You should request testing be done to measure catecholomines, either plasma or 24 hour urine. Also metanephrine test can be helpful. It’s possible you will need pre-op alpha/beta blocker medication to control your blood pressure as well as heart rate.

What areas of your body did they scan? Neck to pelvis? A Ga Dotatate PET-CT is a good scan for NETs that are metabolically active. If the NET is showing in hormone markers, it will probably show on the scan.

Less accurate for NETs that are non-functional.

Or that's what I've read anyway. Still debating what type of scan I would like to request. My hormone markers are erratically elevated. My tumor markers are not: chromogranin A, IL-2 and others.

A Pet/Fdg is a reliable scan if it's not known if any possible tumors are somatostatin positive. I get Sandostatin LAR injections every 4 weeks and since that can cause interference with a GA 68 scan my oncologist orders the Pet/Fdg.

I did the catecholamines blood and 24 hours urine. I have kidney issues, test results were all over the board. Metanephrine blood was only slightly above. My heart rate jumps or ultra low drops are without pattern at all. I have been told it is highly episodic.
Apparently there is another person at MD Anderson now who is episodic functional like me. HIPAA will not allow them to share more.
Would catecholamines or metanephrine test show anything if tumor is not functioning at all time? if it only secrets whenever I wish I knew what triggers it?

Exactly. Neck to pelvis. I have had kidneys and liver issues for a while.
Out of nowhere, my new primary found that lump in my neck first week of June. Already had kidney surgery scheduled for end of June. Day before kidney surgery I learned that I have the SDHB mutation. Family medical history does not exist. They are all dead in Eastern Europe. Just about everybody I remember (been here longer then there) died from cancer of one kind or another. June has not been a very exciting month in hour family. Super glad I found this group. I will need to start a separate thread on hockey players and rare diseases here :-). Thank you again !!!

My husband is adopted. Makes medical histories interesting in our family, too. I understand.

I'm glad they did a thorough scan on you. 🙂

And best wishes for your kidney surgery. Sounds like you have a lot on your plate.

Symptoms from Pheochromocytoma/Paraganglioma can be episodic. The hormones that are secreted are in charge of the " fight or flight " syndrome so stress plays a part in it. Even feelings similar to having a panic attack can happen when those hormone levels are elevated. Having a sdhb mutation with a head/neck paraganglioma is fairly uncommon. I would stay on top of it for most of what I've read says the sdhb tumors are the more aggressive subtype and more often than not will turn out to be cancerous.

I am sending you prayers