Paraganglioma / Carotid Body Tumor Question

Hello everyone. I was incorrectly dx with bladder cancer and after second opinion am one year later learning that is not what I had, but a paraganglioma. Anyone else have this?

Shari, that's so great to hear that you are doing so well!!! Did you have genetic testing done? I am honestly too scalpel shy to get a surgical opinion. Right now it seems that radiation is going to be the best option. I've been told by many doctors now that surgical intervention is not an option and could be fatal given the vascular nature of the tumor location along with the nerve involvement. I'm gearing up for evaluation at the SCCA proton beam radiation therapy center in Seattle WA for evaluation with the founder of the center. It is the closest proton beam radiation center near to MT in a 6 state radius. A 7 to 8 hour drive from where I currently reside. The literature for paraganglioma treated with octreotide shows promising tumor size reduction by 3-11%. This paired with the proton radiation is the most recent treatment option that has been recommended. I will continue to see Dr Pacak and his team at the NIH every 3 to 6 months after my treatment start. I do feel I am in great hands there. Given that I am SDHB ( B for Bad) I am at high risk for secondary malignancy and will need continued supervision for the rest of my life. This is my new "normal". I am not going to say it has been an easy adjust especially because my husband and I were trying to have another child for 7 months before I found out. We have a 4 year old daughter who is the light of our lives. Her genetic testing is pending at the NIH as we did not know prior to having her. The sooner we know the better for disease monitoring and outcomes. The mutation is heterozygous and there is a 50% chance that she has the mutation. Most of my time has been spent researching, reading and deciding what my next move is going to be. I appreciate your feedback!
Best wishes,
Ristene

I'm new to this site and just beginning testing. I've suffered for over twenty years with these symptoms. I have been to so many specialists with these symptoms and no one cared to look into this, until now. My new doctor is my hero. I feel so blessed having found him. I want to say something if anyone is just getting started like me, just getting tested for Pheochromocytoma. My lab in Southern California failed to give me instructions so my results are delayed and I have to repeat all my 24hour testing. I have taken the "proactive" patient approach the second time around and looked up all the things that would affect my results, there is a lot. It is sad that these professional individuals failed me as a patient and delayed my treatment. I'm excited when the day comes when I no longer soak out my clothes, my hair and be able to go out without feeling like I'm going to die because I'm so hot or have a heart attack.

Welcome Ristene! I am sure you are feeling very overwhelmed right now, which is understandable. These are rare tumors and it is hard to find doctors that are familiar with them. My diagnosis was a carotid body tumor (CBT) but during surgery was found to be a vagus tumor instead. To answer your questions:
1. I was diagnosed in October 2016 by my primary care doctor. She could feel the lump in my neck and immediately ordered a CT scan and ultrasound.
2. I was referred to a vascular surgeon who sent me on to Mayo. I had surgery in December 2016 with a vascular and ENT surgeon. I also had a consult with an endocrinologist.
3. Mayo is the place to go!
4. My endocrinologist was Dr. Young, vascular was Dr. Bower and ENT Dr. Kasperbauer. I cannot say enough good things about them!
5. My tumor was entangled with my vagus nerve which controls swallowing and speech. I did lose my voice for the most part as my vocal cord on that side is paralyzed. A couple of months after surgery I went back to Mayo for an injection. My voice is not quite as strong as it once was but I am the only one that notices. I also had a little trouble swallowing in the beginning but that is much better now.
6. My outcome was great! Even with the side effects, it is much better than the extreme fatigue and nausea I had prior to removal.

If you have any questions please ask! I wish you the best of luck!!
Shari

@hopeful33250 Thank you for your kind response. It does help to know that at the times you feel most alone there are other people out there struggling with similar diagnoses. I am a nurse in a busy diabetes and metabolism clinic. Late December 2018, I was on the phone with a patient and tilted my head to the right. Only seconds later my tongue rolled to the back of my mouth, I nearly lost my voice and had slurred speech. I immediately placed my patient on hold and tried to tell my co worker what had just happened. I thought I was having a stroke. I had facial numbness and tingling from the corner of my mouth on the right side to my right temple. My physician works in the same clinic so I discussed this with her. Ultimately, we decided to hold off on imaging given the end of the year and treated for Bell's Palsy with high dose antivirals and steroids. My symptoms got slightly better but not much. It was the beginning of a new year and I thought, I'll have the MRI and nearly meet the deductible. I didn't think much of it as I'm already drowning in medical bills from my suspected diagnosis of seronegative Rheumatoid Arthritis four years prior. On January 9th I had a head and neck MRI and sure enough it showed a paraganglioma. I had never heard of this nor had my physician. She called around town to the oncologists, ENT, radiologists etc and ultimately told me she didn't know what it was but would get me where I needed to be. Prior to the diagnosis I had multiple symptoms that I chalked up to other diagnoses. For example: palpitations, chest pain, shortness of breath, headaches, ringing in the ears,flushing, hot flashes, excessive sweating, intermittent diarrhea. After diagnosis, the symptoms became stronger and started occurring more frequently than they had before. Interestingly enough, my heart rate hasn't been elevated much only during times of stress (normal) and my blood pressure hasn't been high (minus during a cold, also normal).

Hello @ristene and welcome to Mayo Connect,

I am sorry to hear of this rare diagnosis. As you know, a rare disorder can be more difficult to diagnose and to treat. I hope that someone else will respond to your questions, I know how important it is to learn from the experience of others. We have many people in the NETs discussion group who have used Octreotide and some who have had PRRT treatments but not for this particular disorder.

I have also had a rare type of cancer, neuroendocrine tumors of the upper digestive tract. Right before my third surgery I found Mayo Connect and was glad to have others to share with me.

Since this is a rare disorder, would you care to share with us about your original (as well as current) symptoms and how it was diagnosed? From an article, I found on the NIH website it appears that a CT scan or MRI or the usual means of discovery. Here is a link to that article, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3328838/, however, I have not picked up on what type of symptoms are usually associated with this disorder other than excessive sweating, tachycardia and hypertension. Have you had other symptoms as well as these?

Hello, My name is Ristene and I was diagnosed with a glomus jugular paraganglioma on 1/10/19. Genetic testing showed a mutation, SDHB positive. No one where I live knows anything much about these tumors and ever since I’ve been running around in circles trying to make plans for treatment. I was evaluated by Dr Karel Pacak at the NIH who originally thought that PRRT was the best treatment option. I don’t qualify to have this done at NIH because of the lack of confirmed pathology. No one is able to biopsy my tumor given the location and surgery is not an option. Therefore, he has suggested that I start Octreotide and have proton beam radiation.
I really would like some insight from patients who have gone through similar circumstances.
1.) when were you diagnosed ?
2.) what have been your treatment(s) ?
3.) where were you evaluated ?
4.) who was your physician ?
5.) what side effects did you experience ?
6.) what was your overall outcome?

Thank you in advance,
Ristene

Hello, My name is Ristene and I was diagnosed with a glomus jugular paraganglioma on 1/10/19. Genetic testing showed a mutation, SDHB positive. No one where I live knows anything much about these tumors and ever since I’ve been running around in circles trying to make plans for treatment. I was evaluated by Dr Karel Pacak at the NIH who originally thought that PRRT was the best treatment option. I don’t qualify to have this done at NIH because of the lack of confirmed pathology. No one is able to biopsy my tumor given the location and surgery is not an option. Therefore, he has suggested that I start Octreotide and have proton beam radiation.
I really would like some insight from patients who have gone through similar circumstances.
1.) when were you diagnosed ?
2.) what have been your treatment(s) ?
3.) where were you evaluated ?
4.) who was your physician ?
5.) what side effects did you experience ?
6.) what was your overall outcome?

Thank you in advance,
Ristene