PMID- 18473355 OWN - NLM STAT- MEDLINE DCOM- 20080721 LR - 20220408 IS - 0008-543X (Print) IS - 0008-543X (Linking) VI - 113 IP - 1 DP - 2008 Jul 1 TI - Bronchopulmonary neuroendocrine tumors. PG - 5-21 LID - 10.1002/cncr.23542 [doi] AB - 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. CI - (Copyright) 2008 American Cancer Society. FAU - Gustafsson, Bjorn I AU - Gustafsson BI AD - Department of Surgery, Yale University School of Medicine, New Haven, CT 06520-8062, USA. FAU - Kidd, Mark AU - Kidd M FAU - Chan, Anthony AU - Chan A FAU - Malfertheiner, Max V AU - Malfertheiner MV FAU - Modlin, Irvin M AU - Modlin IM LA - eng PT - Journal Article PT - Review PL - United States TA - Cancer JT - Cancer JID - 0374236 SB - IM MH - Bronchial Neoplasms/diagnosis/genetics/pathology/therapy MH - Carcinoid Tumor/diagnosis/genetics/pathology/therapy MH - Carcinoma, Large Cell/diagnosis/genetics/pathology/therapy MH - Carcinoma, Small Cell/diagnosis/genetics/pathology/therapy MH - Humans MH - *Lung Neoplasms/diagnosis/genetics/pathology/therapy MH - Models, Biological MH - Neoplasm Metastasis MH - *Neuroendocrine Tumors/diagnosis/genetics/pathology/therapy MH - Prognosis RF - 134 EDAT- 2008/05/14 09:00 MHDA- 2008/07/22 09:00 CRDT- 2008/05/14 09:00 PHST- 2008/05/14 09:00 [pubmed] PHST- 2008/07/22 09:00 [medline] PHST- 2008/05/14 09:00 [entrez] AID - 10.1002/cncr.23542 [doi] PST - ppublish SO - Cancer. 2008 Jul 1;113(1):5-21. doi: 10.1002/cncr.23542.