As reported in the Journal of Thoracic Oncology by Simron Singh, MD, MPH, and colleagues, the Commonwealth Neuroendocrine Tumour Research Collaboration (CommNETs) and North American Neuroendocrine Tumor Society (NANETS) have collaborated to produce a best practice guideline for the diagnosis and management of patients with lung neuroendocrine tumors. The guideline represents an endorsement and update of the 2015 European Neuroendocrine Tumor Society (ENETS) consensus guidelines.
Simron Singh, MD, MPH
The guidelines provide recommendations in the areas of pathology; tumor staging; biochemical assessment and functional syndromes; diagnosis, including radiologic imaging, functional imaging, and bronchoscopy; functional respiratory tests; surgery; locoregional therapy; adjuvant therapy; therapy for unresectable, locally advanced, or metastatic lung neuroendocrine tumors; and follow-up. Each recommendation is graded for level of evidence and strength of recommendation. Select recommendations for diagnosis and treatment are summarized/reproduced below.
Diagnosis Recommendations
Radiologic Imaging
- At initial diagnosis, imaging should include a contrast-enhanced computed tomography (CT) scan of the chest, with multiphase CT/magnetic resonance imaging (MRI) of the liver.
- Hepatobiliary phase liver MRI is more sensitive than CT or somatostatin receptor positron-emission tomography (SSTR-PET) and should be used for detection of small hepatic metastases.
Functional Imaging
- SSTR-PET can be used in patients to detect metastatic disease.
- Fluorodeoxyglucose (FDG)-PET may be useful in addition to SSTR-PET in heterogeneous disease.
- The clinical utility in small primary lung neuroendocrine tumors without evidence of metastatic disease on contrast imaging is limited.
Bronchoscopy
- Bronchoscopy is a safe and effective method for diagnosing lung neuroendocrine tumors and may be considered as the initial diagnostic modality for these tumors.
Treatment Recommendations
Surgery
- In the case of localized disease, the surgical techniques of choice are lobectomy or sleeve resection.
- Sublobar resection is a possible acceptable alternative if complete (R0) resection can be achieved in peripheral < 2 cm typical lung neuroendocrine tumors.
- Complete anatomic resection and systematic nodal dissection are recommended as the resection extent of choice for patients with peripheral tumors.
- Lung parenchymal-sparing surgery should be preferred over pneumonectomy.
- Endobronchial resection should be reserved for patients who are considered to be at unacceptably high risk for surgical resection or occasionally as a possible bridge to surgery.
- In patients with nonaggressive tumors, even with limited extrahepatic disease, palliative cytoreductive surgery of the liver should be considered.
Locoregional Therapy
- Local ablative radiation and thermal therapies can be used for local tumor control of primary lung neuroendocrine tumors and for palliation of symptoms in patients unfit for or who have declined surgery.
- Locoregional therapies including surgery should be considered for progressive or symptomatic metastases in the liver or other solid organs and the bones.
Adjuvant Therapy
- Adjuvant therapy with somatostatin analogs (SSAs), chemotherapy, or radiation is not recommended in patients with lung neuroendocrine tumors after complete resection.
Therapy for Unresectable, Locally Advanced, or Metastatic Lung Neuroendocrine Tumors
- A watch-and-see policy may be considered in a subgroup of asymptomatic patients mainly with typical carcinoid or atypical carcinoid of low proliferative index.
- SSAs are the first-line treatment of carcinoid syndrome.
- In patients with Cushing syndrome, control of cortisol secretion is needed.
- SSAs may be considered as first-line systemic antiproliferative treatment of patients with advanced unresectable lung neuroendocrine tumors of good prognosis, particularly typical carcinoid and atypical carcinoid with low proliferative index, and slowly progressive [disease] provided somatostatin receptor imaging is positive.
- Peptide receptor radionuclide therapy may be an option in patients with somatostatin receptor–positive tumors.
- External-beam radiation is an effective palliative therapy for patients with symptomatic locally advanced or metastatic disease.
- Everolimus should be considered for routine use in progressive nonfunctional lung neuroendocrine tumors and may be considered in functional lung neuroendocrine tumors.
- Use of streptozocin-based, oxaliplatin-based, etoposide-based, or temozolomide-based chemotherapy may be considered in advanced lung neuroendocrine tumors, with particular consideration in atypical carcinoid.
- There remain insufficient data to suggest the routine use of antiangiogenics in lung neuroendocrine tumors.
Dr. Singh, of the Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, is the corresponding author for the Journal of Thoracic Oncology article.
Disclosure: For full disclosures of the study authors, visit jto.org.
