Already on oxygen - diagnosed with DIPNECH: What happens next?

I’ve had my first significant change recently. 3 months ago it looked like I had a few new tumors in my lower lung, but they also saw 2 of my DIPNECH/NETs tumors appear to be blocking that area so it may just be trapped mucus. I just had another CT scan after 3 months instead of 6. Looks stable but not improved as hoped. They are still leaning toward trapped mucus but no way to be sure based on the CT. I’ll have the next CT scan in 3-4 months to monitor any changes. No significant change in symptoms or signs of infection. I am concerned with 50+ tumors that more areas could become blocked over time.

Hello @californiazebra

I am sorry to hear of the changes in your CT scan. I am glad to hear that there have been no significant changes in your symptoms. Please keep me posted on how you are doing. When will you have your next scan?

Hello @gprior,

I understand your interest in reading about how others have treated DIPNECH. You mentioned that In Australia there are not many medical people who have heard of this. Here is a link to the Carcinoid Cancer Foundation website with a list of NET specialists throughout the world. Here is a link to that list.
https://www.carcinoid.org/for-patients/treatment/find-a-doctor/
After listing U.S. specialists, there is an international listing of doctors. One doctor does appear under Australia,
Australian NET Doctors (Oncology, Surgery, Nuclear Medicine)
J. Harvey Turner, MD, FRACP (Interventional Radiology)

Perhaps a second opinion might help you feel more comfortable with your current treatment Have you considered a consultation to see if your current treatment might be augmented in some way?

@gprior Sorry to hear that. Ask about microwave ablation to destroy the tumor, but preserve the surrounding healthy tissue. If your tumors are slow growing like mine, not every tumor necessarily needs to be removed unless it's causing a particular issue. They have no intention of ever destroying all of mine because there are just too many. They are monitoring for any tumors that start to go rogue.

Have the lobectomies left you short of breath or what impact did that have? Painful recovery?

May I refer to you as California? Thank you for your input. My first lobectomy was done because I had numerous nodules/ tumours but one was 2cm×1.9cm and apparently needed to be removed. Recovery was about 8 weeks and afterwards i was a little more short of breath.The 2nd op last Oct was because again numerous nodules but one larger one that was close to the heart wall needed to be removed so again the whole lobe was removed, unfortunately I had complications a day after this op and was back in surgery for another op, this has left me quite short of breath even tho I am still doing the breathing exercises and yes this time it's been a painful and long recovery, I had setbacks after I came home such as a virus that caused such a lot of added coughing, today I find out what they want to do with this 3rd lobe, but I'm reluctant to have more surgery as I'm still recovering and yes veryshort of breath at times. Hope you are OK, do you have dipnech??

@gprior
Thank you for the response. You’ve been through a lot. I’m sorry to hear of complications, pain and breathing issues. Being short of breath when resting is a terrible feeling. Did the biopsy of that largest tumor indicate malignancy (NETs) or do they think they are all benign?

Yes, I have DIPNECH and lung NETs (typical carcinoids). 50+ nodules were first discovered in 2008 but my respiratory symptoms indicate I’ve had this for at least 35 years. Very slow growing. Most are a little under or over 1 cm. They don’t give me exact measurements anymore. They just watch for any going rogue. In 2021 I had the largest tumor 2.6 cm destroyed with microwave ablation. I believe if I had stayed with my breast oncologist and his local thoracic surgeon they likely would have wanted to remove that lobe. Luckily I went to a NETs multidisciplinary team who recommended ablation given every lobe is full of tumors. Can’t remove all the tumors or lobes. I don’t want to remove any lobes. I did have a partially collapsed lung after both the ablation and the initial biopsy so I can empathize with what you’ve gone through. Normally I have a CT scan every 6 months but 3 months ago they found either new tumors or debris trapped in the bottom of one lung due to a blockage caused by other tumors. Just had a scan after 3 months and no changes. Will scan again in 3-4 months to monitor the situation. My tumors are still confined to my lungs after all these years.

I’ve taken monthly octreotide injections for 4 years now. Life changing getting rid of the chronic cough for 30+ years, reduced mucus and helped shortness of breath upon exertion. It also slows tumor growth. It’s important to see specialists experienced in our specific disorders.

Good luck at your appointment. Please keep us posted. Zebra

They are going to monitor the lower lobe as before, a CT every 6 months. I received the results of my last lobectomy, this time there were 2 large tumours that were g719a EGFR / KRAS mutations . I've never heard of this before. It seems the same is in the lower lobe. I see specialist again in 6 weeks as he has put me on an inhaler to see if it improves my breathing. Will keep you posted

Hello @californiazebra

I am sorry to hear of the changes in your CT scan. I am glad to hear that there have been no significant changes in your symptoms. Please keep me posted on how you are doing. When will you have your next scan?

Hello @jan355 and welcome to Mayo Connect. I see that @californiazebra has already posted with you. I agree with her response, it is important to seek out a NET specialist and one who is familiar with treating DIPNECH.

A one-time consultation at Mayo Clinic would be a good idea now that you are diagnosed. Here is a link with information about obtaining an appointment, http://mayocl.in/1mtmR63
If you cannot be sent at a Mayo Clinic facility for any reason, here is a list of NET specialists, https://www.carcinoid.org/for-patients/treatment/find-a-doctor/.

Often, just a one-time consultation with a specialist can put you on the right road for effective treatment.

I look forward to hearing from you again. Will you keep posting and let us know how you are doing?