Bronchopulmonary neuroendocrine tumors.
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.
Gustafsson
Bjorn I
BI
Department of Surgery, Yale University School of Medicine, New Haven, CT 06520-8062, USA.
Kidd
Mark
M
Chan
Anthony
A
Malfertheiner
Max V
MV
Modlin
Irvin M
IM
D001984:Bronchial Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D018287:Carcinoma, Large Cell / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D018288:Carcinoma, Small Cell / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D008954:Models, Biological; D009362:Neoplasm Metastasis; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D011379:Prognosis