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