Should I see a NET specialist?

Hi @lizzyeastwood,

I noticed in a previous post that you are being seen at Mayo in Rochester. Is that correct? If so, you are probably seeing a NET specialist. You can certainly verify that, though with your medical team.

It has probably been close to 10 years since DIPNECH was suspected in me. In the following years, I have been to Denver to Jewish Hospital for a week, they confirmed the diagnosis, and since have been followed here in Atlanta. No doc has ever mentioned anything more than a twice yearly CT scan, which has now changed to once yearly as there has been little change. Do I need to go further? The one thing that I do know is that I become very short of breath easily, am on oxygen at night only or when I fly, or if I go into the mountains. Just wondering what Mayo would advise as I want to stay on top of this tiger! Jane Weaver

@gram26

Hello Jane and welcome to the NETs support group on Mayo Connect. I so appreciated you saying, "...I want to stay on top of this tiger!" That is something all of us would like to do! You are wise to want a good follow up plan that will keep you as healthy as possible with DIPNECH.

We have several members on Connect who also have DIPNECH and I would like to invite them to post about their experiences for follow up with you. Perhaps @californiazebra will share her experiences with you. I would also encourage you to look at a discussion group that deals specifically with DIPNECH. Please read the posts, I think you will learn a lot from others, and you can begin posting your questions in that group. Here is the link.
https://connect.mayoclinic.org/discussion/new-dipnech-diagnosis-need-advice-on-doctors/
Getting another opinion, especially about the shortness of breath is probably a good idea. If you would like a consultation with Mayo Clinic here is a link that will take you to the appointment website, http://mayocl.in/1mtmR63. You can seek an appointment using this website at any of the three Mayo Clinic campuses (Minnesota, Florida or Arizona).

I look forward to hearing from you again. Has the shortness of breath become more of a problem recently, or has it remained the same since your diagnosis ten years ago?

Hi Jane @gram26

I would agree with Theresa to check out all the DIPNECH comments on the link she provided including my story. To recap here:

They first discovered over 50 tumors scattered across both of my lungs in 2008 due to a chronic cough. Couldn't biopsy with a needle at that time. Monitored with CT scans and the tumors were slowly growing, but it took 10 years for them to double in size. Diagnosed as DIPNECH and Lung NETS (typical carcinoids) in 2020 when my breast cancer came back (non-related). They could now reach the largest tumor with a needle biopsy. The largest lung tumor was now 2.6 cm so they did microwave tumor ablation at UCLA to destroy the tumor as it was at risk for metastasis. As far as we know, my lung NETS is still contained in my lungs (stage 1) after all these years. However, the DIPNECH is considered advanced. Going forward, they will ablate any tumor that hits 2 cm. Most are around 1 cm or a little more right now. They are looking for any that suspiciously take off from the pack. My interventional radiologist at UCLA just told me mine was the largest lung tumor he's ever done microwave ablation on so he's excited that it was a success.

I also started taking Octreotide injections every 4 weeks 26 months ago. That helped my symptoms tremendously. Before that, I had a chronic cough and clear mucus for 30 years! It was like having bronchitis for 30 years. I was known for it. I'm 64 now. I also had shortness of breath upon exertion (even just carry trash to the dumpster). I was told it was not the tumors that we can see causing the symptoms, but that I have hundreds or thousands of tiny tumors lining my airways that cause obstruction. I also have bronchiectasis and air trapping. They know this from pulmonary function tests and by knowing the disease. My oxygen level is about 96% when sitting in a desk chair, but 92/93% when resting in a recliner or in bed. I'm not thrilled about that. I am not on oxygen. My pulmonologist told me under 89% is the threshold of concern and for oxygen to be covered. What is your level?

Octreotide may really improve your quality of life by improving your symptoms. Ask about it. They told me I've likely had the tumors for decades. Based on what I've read: Without a biopsy, the rule of thumb is any tumor under 5 mm is considered a tumorlet and may not be cancer yet. Anything 5 mm and over is considered a carcinoid tumor or lung NETS (neuroendocrine cancer) and cancerous. Only a biopsy can confirm. I waited until they could reach the largest tumor with a needle biopsy and said no thanks when they wanted to filet my chest and lungs for a biopsy. Personal decision you'll need to make if presented with that option. I don't do well with open cutting surgeries and can't take pain meds.

I was also told that octreotide could begin to lose its effectiveness over time, but there is not enough DIPNECH historical data to say when. Sadly, the old symptoms have made an appearance the past few months. They are not nearly as bad as before the octreotide, but I have the feeling things are heading that way -- hopefully slowly. I'm not giving up on octreotide yet though. The good news, as you know, is both typical carcinoids and DIPNECH advance very slowly so it's manageable and treatable. They are not considering removing any part of my lungs due to the number of tumors.

How many tumors do you have that can be seen on the CT?
Have they told you how large the tumors are?

I also did a DOTATATE Cu64 (similar to Ga68) PET scan at UCLA and found I don't have somatostatin receptors so that test didn't do any good for me. More NETS patients do have the receptors than don't. PRRT would never be effective without receptors, but thankfully octreotide still works for some reason.

There are only a couple hundred documented cases of DIPNECH (last I checked) although they believe it's underdiagnosed due to unfamiliarity. I know my tumors baffled 20 different specialists of all kinds for 12 years until the biopsy. No one even guessed DIPNECH or lung NETS in all those years. Lots of wild guesses. My interventional radiologist and thoracic oncologist at UCLA have both told me they just don't have a lot of historical data to go on for this disorder. They are happy with our successes so far.

It's good that you are staying on top of this. I hope this info was helpful. Please let me know if you have any other questions. Blessings, Zebra

Thanks so much for reaching out...you have been on the front lines! As for the questions you asked me, I do not have answers...have never been told how many tumorlets are involved, and certainly do not know the size of anything....all that is ever said is that nothing has changed very much. One of the reasons I am asking questions, as 10 years is a long time to be mostly in the dark. It would seem that I will have to go to Mayo or Vanderbilt, or maybe back out to Denver to get answers, as I cannot locate anyone in the Atlanta area who knows much about this disease. The cough is only occasional at this point, although has been worse in the past. That is something to be thankful for, at least! Will do my best to stay on top of this, and appreciate your input! Jane

I live in Louisville,KY. Just diagnosed with net on pancreas, following endoscopy with ultrasound. Pathology says “well differentiated”. Only one doctor in the area, a surgical oncologist at University of Louisville specializes in this field.

Hello @djchambers and welcome to the NETs support group on Mayo Connect. As you are probably aware, NETs are a rare form of cancer, and it is always a good plan to have a consultation with a NET specialist. NET specialists can provide you with the best guidance, especially as you begin treatment. Mayo Clinic has NET specialist at all three of their locations. If you would like to get a consultation (either in-person or virtual) here is a link to appointment information, http://mayocl.in/1mtmR63. In addition, I'm also including a list of NET specialists worldwide, https://www.carcinoid.org/for-patients/treatment/find-a-doctor/. I do see a specialist listed in Lexington, KY.

We have another discussion group that might be helpful to you,?
Should I See a NET specialist?
https://connect.mayoclinic.org/discussion/should-i-see-i-net-specialist/?commentsorder=newest#chv4-comment-stream-header. I would also like to invite @californiazebra to this discussion.

As this is your first post, could you share a bit about your diagnosis? Were you having symptoms that led to the endoscopy where the pancreatic NET was found? Are you having any blood sugar problems?

If at all possible, I would say yes. NETs are relatively rare and treatments are making great strides. I am very thankful for a specialist who is immersed in caring for NET patients. Best Wishes for you!

I’m so sorry you have to go through this. My husband was diagnosed with a PNET 15 years ago so I certainly empathize with you. I definitely think that it’s important that you see a NET specialist. This cancer doesn’t behave as your typical cancer and a general gastro oncologist may not have the knowledge that’s needed to determine the proper tests, treatments and sequencing of treatments. Even when seeing a specialist, it’s also important to educate yourself as much as possible about this disease. I’ve found it so helpful to understand this disease and the treatment options as then I feel more confident in any decisions we make. I’ve also been in situations (even with a net specialist) where what was being told to us didn’t make sense given what (little) we knew so we sought out another specialist which drastically changed my husbands prognosis for the better. A couple of good places to start to learn about things is http://www.NETRF.org. Like the other excellent site that was recommended, you can find a specialist and support groups near you as well as links to helpful information to start learning about this disease. Another great resource is http://www.netcancerawareness.org Lots of great informational videos from conferences. Lastly, take a deep breath. This will likely be a marathon and not a sprint as usually this is a slow growing disease that often can be treated as a chronic illness. My husband is proof of this. Best of luck to you!

Thank goodness the others have given you the best advice. If I had not persisted in seeing a NET specialist, my first oncologist (who only told me I had stage 4 cancer) would have begun chemo. I’m happy to say I only take lanreotide injections monthly and have minimal disruption of my normal life. My NET specialist told me I would most likely die with, but not of my NeuroEndocrine cancer! Hang in there & Merry Christmas! 🎄🎁