Bronchopulmonary neuroendocrine tumors

• Published in: Cancer Vol. 113; no. 1; pp. 5 - 21
• Main Authors: Gustafsson, Bjorn I., Kidd, Mark, Chan, Anthony, Malfertheiner, Max V., Modlin, Irvin M.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.07.2008; Wiley-Liss
• Subjects: Biological and medical sciences; Bronchial Neoplasms - diagnosis; Bronchial Neoplasms - genetics; Bronchial Neoplasms - pathology; Bronchial Neoplasms - therapy; bronchnary; carcinoid; 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; Lung Neoplasms - diagnosis; Lung Neoplasms - genetics; Lung Neoplasms - pathology; Lung Neoplasms - therapy; Medical sciences; Models, Biological; Neoplasm Metastasis; neuroendocrine; Neuroendocrine Tumors - diagnosis; Neuroendocrine Tumors - genetics; Neuroendocrine Tumors - pathology; Neuroendocrine Tumors - therapy; neuroepithelial bodies; Pneumology; Prognosis; tumor; Tumors; Tumors of the respiratory system and mediastinum; Lung disease; neuroendocrine; Respiratory disease; Lung; neuroepithelial bodies; carcinoid; Respiratory system; bronchnary; Bronchopulmonar tumor; Cancerology; tumor; Bronchus disease; Neuroendocrine tumor
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/cncr.23542

Bronchopulmonary neuroendocrine tumors (BP‐NETs) comprise ≈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, ≈10% and ≤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, ≈6% per year). No curative treatment except for radical surgery (almost never feasible) exists. The slow‐growing TC exhibit a fairly good prognosis (≈88%, 5‐year survival), whereas AC demonstrate a 5‐year survival of ≈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. Cancer 2008. © 2008 American Cancer Society. Bronchopulmonary neuroendocrine tumors (BP‐NETs) comprise ≈20% of all lung cancers. BP‐NETs include 4 histologic subgroups: typical carcinoid tumor, atypical carcinoid tumor, large‐cell neuroendocrine carcinoma, and small‐cell lung carcinoma. The review assesses their current classification and management with a focus on the features of each subgroup, emphasizing their specific diagnostic and therapeutic options.