A Look at Lung NETs

Approximately 20% of all primary lung tumors are neuroendocrine (Hung 2019), including:

  • Typical carcinoid (TC)
  • Atypical carcinoid (AC)
  • Small cell carcinoma (SCC)
  • Large cell carcinoma (LCNC)

Classification of Lung NETs

“When we are classifying lung NETs we want to make one basic distinction,” said Suki Padda, MD, Stanford University, “Is this a well-differentiated lung NET, which includes low-grade typical carcinoid, or intermediate grade atypical carcinoid, or is this a poorly differentiated lung NET and that includes SCC and LCNC?” The outlook for each is different.

Classifications of lung neuroendocrine tumors 

Grade

Prevalence (% of all lung tumors)

Associated with smoking

Overall survival at 5 years

Typical  Carciniod

 

Atypical Carcinoid


Low 

Intermediate

1-2%

∼0.2%

No

No

>90%

50-80%

Small Cell Carcinoma 

 

Large Cell
Neuroendocrine
Carcinoma 

High

High

10-15%

2-3%

Yes

Yes

5%

15-40%

Source: Hung 2019

“Large cell neuroendocrine carcinoma and small cell carcinoma are not well differentiated and are very aggressive; these are the ones that we are going to give systemic therapy, such as chemotherapy,” said Nagla Karim, MD, PhD, University of Cincinnati. “The typical and atypical carcinoid are well differentiated are not very aggressive.”

Diagnosing Lung NETs

As with other NETs, the diagnosis of lung NETs can be challenging. Some patients do not show any signs of illness. Those with symptoms, such as coughing, wheezing, or tightness in the chest, may be diagnosed with more common illnesses, such as asthma. Since lung NETs develop in hormone-producing cells, a few patients experience symptoms unrelated to the lungs, such as diarrhea or flushing in the face.

Lung NET presentations from NET patient and caregiver conferences. 

Treatment for Lung NETs

A lack of consensus among leading treatment guidelines complicates the diagnosis of lung NETs. (Hendifar 2017) There are also different recommendations about the need for chemotherapy and/or radiation in early-stage patients who have undergone surgery. (Hendifar 2017, Ramirez 2017) An individual’s treatment will be based on a range of factors including disease classification, stage, lymph node involvement, and tumor biomarkers. Robert Ramirez, DO, Ochsner Medical Center, stresses the importance of a multidisciplinary treatment team for lung NETs to obtain an accurate diagnosis and avoid unnecessary procedures. NETRF has compiled a list of multidisciplinary team-based programs.

The options commonly considered by a treatment team may include:
(Ramirez 2017; NCCN Guidelines Version 3.2017)

  • Surgery
  • Somatostatin analogs
  • mTOR pathway inhibitors
  • Vascular treatments
  • Chemotherapy (may be a combination of therapies)
  • Radiotherapy, such as PRRT
  • Radiation (radiofrequency ablation, stereotactic body radiation therapy or SBRT)
  • Interventional radiology (embolization)

Clinical Trials of Lung NETs

Lung NETs were not routinely included in phase III trials for gastroenteropancreatic studies (i.e., CLARINET). The RADIANT-4 trial showed a clinical benefit of a mTOR inhibitor over placebo and led to its FDA approval for lung NETs. (Hendifar 2017)   SPINET, the first phase III study for lung NETs (NCT02683941) is underway. Participants must have a well-differentiated typical or atypical lung NET. Patients who have previously been treated with a somatostatin analog, PRRT, or more than two lines of chemotherapy/targeted therapy are excluded. Learn more about NET clinical trials in this talk by Pamela Kunz, MD, Stanford University.

Learn more about lung NETs 

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My insurance company is denying the treatment that my doctor suggests which is cryoablation for the reason stating that there is insufficient evidence that ablation therapy is successful AND that it is not medically necessary. I have one large tumor in my left lung (about 2″ in size) and then 2 smaller tumors in the right lung. Is there any documentation in your studies on ablation therapy?

We have not funded any studies on lung tumor ablation, I am sorry to say. NCCN produces clinical guidelines for care. You have to register to view/download them. https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf These do take into consideration variables like tumor grade/stage. This helpful, informative, and educational talk might also help you figure out next steps. https://www.facebook.com/norcalcarcinet/videos/1150770001739346/ Also, try looking at this article https://norcalcarcinet.org/links/81-appeal
Wishing you the best of luck,
NETRF

Kris

Is there a good resource for doctors specializing in lung NET tumor care

GB

any idea where i can get mTOR pathway inhibitors,
Vascular treatments, Radiation (radiofrequency ablation, stereotactic body radiation therapy or SBRT),
Interventional radiology (embolization) treating option in India, my Dad has pNET G1 with no SSR and no doctor is suggesting us to go for all these option they are directly suggesting Chemo. I believe we should keep Chemo as a last resort when everything stops working.

A Look at Lung NETs