Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging
- Charles F Thomas, Jr, MD
Charles F Thomas, Jr, MD
- Associate Professor of Medicine
- Mayo Clinic College of Medicine
- James R Jett, MD
James R Jett, MD
- Section Editor — Lung Cancer
- Professor of Medicine Emeritus
- National Jewish Health
- Jonathan R Strosberg, MD
Jonathan R Strosberg, MD
- Associate Professor
- Department of Gastrointestinal Oncology
- H. Lee Moffitt Cancer Center
Lung neuroendocrine tumors (NETs) are an uncommon group of pulmonary neoplasms that are characterized by neuroendocrine differentiation and relatively indolent clinical behavior.
Like neuroendocrine tumors at other body sites, lung NETs are thought to derive from peptide- and amine-producing neuroendocrine cells. NETs can arise at a number of sites throughout the body, including the thymus, lung, gastrointestinal (GI) tract, and ovary. The GI tract is the most frequently involved site, while lung is the second most common. (See "Clinical characteristics of carcinoid tumors".)
The epidemiology, classification, clinical features, diagnosis, and staging of lung NETs will be reviewed here. Treatment and prognosis of lung NETs, as well as clinical characteristics of carcinoid tumors arising in other sites, are discussed elsewhere. (See "Lung neuroendocrine (carcinoid) tumors: Treatment and prognosis" and "Clinical characteristics of carcinoid tumors".)
EPIDEMIOLOGY AND RISK FACTORS
Lung NETs account for approximately 1 to 2 percent of all lung malignancies in adults and roughly 20 to 30 percent of all NETs [1-4]. Lung NETs are the most common primary lung neoplasm of children, typically presenting in late adolescence. Typical NETs, which are low-grade tumors with a low mitotic rate, are about four times more common than atypical NETs, which are intermediate-grade tumors with a higher mitotic rate and/or necrosis. (See 'Classification, histology, and histochemistry' below.)
Globally, incidence rates range from 0.2 to 2 per 100,000 population per year, and most series suggest a higher incidence in women as compared with men, and in whites as compared with blacks [1-3,5-7].
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- EPIDEMIOLOGY AND RISK FACTORS
- Risk factors
- - Inherited predisposition
- CLASSIFICATION, HISTOLOGY, AND HISTOCHEMISTRY
- WHO classification
- CLINICAL FEATURES
- Presenting signs and symptoms
- Clinical syndromes related to peptide production
- - Carcinoid syndrome and carcinoid crisis
- - Cushing's syndrome
- - Acromegaly
- DIAGNOSTIC AND STAGING WORKUP
- Staging system
- Cross sectional imaging
- Other imaging procedures
- - Somatostatin receptor-based imaging techniques
- Clinical utility
- - FDG-PET scans
- - Liver imaging
- Tumor markers
- Bronchoscopy and biopsy
- - Peripheral pulmonary nodules
- DIFFERENTIAL DIAGNOSIS
- Disorders with similar clinical manifestations
- Disorders with similar radiographic manifestations
- SUMMARY AND RECOMMENDATIONS