Bronchopulmonary neuroendocrine tumors

Cancer. 2008 Jul 1;113(1):5-21. doi: 10.1002/cncr.23542.


Bronchopulmonary neuroendocrine tumors (BP-NETs) comprise approximately 20% of all lung cancers and represent a spectrum of tumors arising from neuroendocrine cells of the BP-epithelium. Although they share structural, morphological, immunohistochemical, and ultrastructural features, they are separated into 4 subgroups: typical carcinoid tumor (TC), atypical carcinoid tumor (AC), large-cell neuroendocrine carcinoma (LCNEC), and small-cell lung carcinoma (SCLC), which exhibit considerably different biological characteristics. The clinical presentation includes cough, hemoptysis, and obstructive pneumonia but varies depending on site, size, and growth pattern. Less than 5% of BP-NETs exhibit hormonally related symptoms such as carcinoid syndrome, Cushing, acromegaly, and SIADH. SCLC is the most common BP-NET, while LCNEC is rare, approximately 10% and < or =1%, respectively, of all lung cancers. Both SCLC and LCNEC progress rapidly, are aggressively metastatic, and exhibit a poor prognosis. The incidence of BP-carcinoids (TC and AC) in the US was 1.57 of 100,000 in 2003 (an unexplained and substantial increase over the last 30 years, approximately 6% per year). No curative treatment except for radical surgery (almost never feasible) exists. The slow-growing TC exhibit a fairly good prognosis ( approximately 88%, 5-year survival), whereas AC demonstrate a 5-year survival of approximately 50%, and the highly malignant LCNEC and SCLC5-year survival of 15% to 57% and <5%, respectively. This review provides a broad overview on BP-NETs and focuses on the evolution of the disease, general features, and current diagnostic and therapeutic options.

Publication types

  • Review

MeSH terms

  • Bronchial Neoplasms / diagnosis
  • Bronchial Neoplasms / genetics
  • Bronchial Neoplasms / pathology
  • Bronchial Neoplasms / therapy
  • Carcinoid Tumor / diagnosis
  • Carcinoid Tumor / genetics
  • Carcinoid Tumor / pathology
  • Carcinoid Tumor / therapy
  • Carcinoma, Large Cell / diagnosis
  • Carcinoma, Large Cell / genetics
  • Carcinoma, Large Cell / pathology
  • Carcinoma, Large Cell / therapy
  • Carcinoma, Small Cell / diagnosis
  • Carcinoma, Small Cell / genetics
  • Carcinoma, Small Cell / pathology
  • Carcinoma, Small Cell / therapy
  • Humans
  • Lung Neoplasms* / diagnosis
  • Lung Neoplasms* / genetics
  • Lung Neoplasms* / pathology
  • Lung Neoplasms* / therapy
  • Models, Biological
  • Neoplasm Metastasis
  • Neuroendocrine Tumors* / diagnosis
  • Neuroendocrine Tumors* / genetics
  • Neuroendocrine Tumors* / pathology
  • Neuroendocrine Tumors* / therapy
  • Prognosis