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ESMO E-Learning: Overview on Management of Lung Neuroendocrine Tumours (Typical and Atypical Carcinoids)

E-learning video block

This content is for ESMO members only.



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Learning objectives

  1. To provide an update on epidemiology of lung neuroendocrine tumours
  2. To provide an update on diagnosis, classification, pathology and molecular biology of lung neuroendocrine tumours 
  3. To provide an overview on available treatment options for different stages of lung neuroendocrine tumours


This E-learning module provides an expert overview in the diagnosis, classification, pathology, molecular biology, staging and available treatment options for lung neuroendocrine tumours (NETs). The author underlines that patients with lung NETs should be managed within or in collaboration with a dedicated multidisciplinary team. 

As a first step in the diagnosis, it is important to separate non-small cell lung cancer (NSCLC) from lung neuroendocrine neoplasms (NENs). The author of this E-Learning Module describes typical and atypical carcinoids that belong to well differentiated lung NETs (low/intermediate grade). They make 9% among lung NENs. Furthermore, two different epidemiology perspectives of typical and atypical carcinoids are highlighted, one representing <3% of all lung cancers from the aspect of thoracic medical oncologist’s clinical practice and the other 25% of all NETs from the aspect of NEN-dedicated medical oncologist’s clinical practice. 

The author presents the WHO 2015 classification of lung NENs and different aspects of the pathological classification and furthermore, provides a comprehensive molecular biology status, as well as details about staging and characterisation. Associated clinical syndromes are also featured in description of the clinical presentation. 

The treatment part is illustrated by statements from the clinical practice guidelines and supported with findings from the clinical trials. The author also describes the settings in which the evidence from clinical trials is needed. 

The author underlines that a complete and radical anatomic resection plus N1/N2 lymphadenectomy should be performed in resectable local or locally advanced lung NETs. In terms of adjuvant treatment, a systemic therapy, mainly chemotherapy, can be discussed, on an individual patient basis, after a multidisciplinary discussion in the intermediate grade (atypical carcinoid) and intermediate stage (pN2). 

In terms of advanced, well-differentiated lung NETs, the Module presents the results with octreotide or lanreotide, everolimus, chemotherapy, peptide receptor radionuclide therapy (PRRT) and liver-directed treatments, with an emphasis on everolimus as the only approved drug for the advanced lung NETs. Octreotide or lanreotide have not been specifically approved for lung NETs but they are recommended for low grade indolent somatostatin receptor positive lung NETs.

Declaration of interest

Nicola Fazio has reported:
Personal financial interests: Novartis, Ipsen, Pfizer, Merck Serono, Advanced Accelerator Applications, MSD (Advisory board, Public speaking).
Institutional financial interests: Novartis, Ipsen, Merck Serono, MSD, Pharmacyclics, Incyte, Halozyme, Roche, Astellas, Pfizer (Clinical trials or research projects: principal investigator, steering committee member).
Non-financial interests: ESMO Coordinator of the Neuroendocrine, Endocrine neoplasms and CUP Faculty; ENETS (European Society of Neuroendocrine Tumours) advisory board speaker; AIOM coordinator for neuroendocrine neoplasms guidelines; ITANET Scientific committee member, Member of the task force for interaction with patients’ associations.

Last update: 20 Aug 2019

This E-Learning module was published in 2019. The CME test expired in 2021.

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