Neuroendocrine tumors of the lung: an update

Arch Pathol Lab Med. 2010 Nov;134(11):1628-38. doi: 10.5858/2009-0583-RAR.1.


Context: The 2004 World Health Organization (WHO) classification recognizes 4 major types of lung neuroendocrine tumors: typical carcinoid, atypical carcinoid, small cell lung cancer, and large cell neuroendocrine carcinoma. Markedly different prognostic implications and treatment paradigms for these tumors underscore the importance of accurate pathologic diagnosis.

Objective: To detail the clinical and pathologic features of lung neuroendocrine tumors, with emphasis on diagnostic criteria, differential diagnoses, and application of immunohistochemistry. The emerging evidence for the utility of Ki-67 (MIB1) in the diagnosis of lung neuroendocrine tumors, particularly in small biopsy and cytology, is emphasized.

Data sources: The 2004 WHO classification, other published literature, and primary material from the author's institution.

Conclusions: The current WHO classification of neuroendocrine tumors is based on morphologic features in combination with precisely defined mitotic rate and absence or presence of necrosis. Ki-67 (MIB1) is emerging as a useful ancillary tool in the diagnosis of these tumors. Continued research efforts are needed to identify additional immunohistochemical and molecular biomarkers that can serve as ancillary diagnostic tools and as potential therapeutic targets for these diseases.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor
  • Carcinoma, Neuroendocrine / classification
  • Carcinoma, Neuroendocrine / pathology*
  • Diagnosis, Differential
  • Humans
  • Immunohistochemistry
  • Lung Neoplasms / classification
  • Lung Neoplasms / pathology*
  • Prognosis


  • Biomarkers, Tumor