Bronchial neuroendocrine (carcinoid) tumors: Treatment and prognosis
- 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
Bronchial neuroendocrine (carcinoid) tumors are a rare group of pulmonary neoplasms that are often characterized by indolent clinical behavior. Like other carcinoid tumors, bronchial carcinoids are thought to derive from peptide- and amine-producing neuroendocrine cells. Bronchial neuroendocrine tumors (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 for NETs, while lung is the second most common. (See "Clinical characteristics of carcinoid tumors".)
Bronchial NETs are characterized by strikingly heterogeneous pathological features and clinical behavior. At one end of the spectrum are typical carcinoid tumors, which are well differentiated, low-grade slowly-growing neoplasms that seldom metastasize to extrathoracic structures. At the other end of the spectrum are the poorly-differentiated and high-grade neuroendocrine carcinomas, as typified by small-cell lung cancer (SCLC), which behaves aggressively, with rapid tumor growth and early distant dissemination. The biologic behavior of atypical carcinoid tumors, which are of intermediate grade and differentiation, is intermediate between typical carcinoid tumors and SCLC. The terms "typical" and "atypical" carcinoid of the lung correspond roughly to the terms "low-grade" and "intermediate-grade," which are used more commonly in extrathoracic NETs. (See "Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging", section on 'Classification, histology, and histochemistry' and "Pathobiology and staging of small cell carcinoma of the lung" and "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system", section on 'Classification and terminology'.)
Multifocal disease is not uncommon in patients with low-grade bronchial NETs. An extreme example is diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), a benign condition characterized by diffuse hyperplasia of pulmonary neuroendocrine cells and formation of multiple carcinoid tumorlets. (See "Pathology of lung malignancies", section on 'DIPNECH'.)
The treatment and prognosis of both typical and atypical bronchial neuroendocrine (carcinoid) tumors will be discussed here. The epidemiology, classification, clinical features, diagnosis, and staging of these tumors is discussed elsewhere, as is treatment of well-differentiated NETs arising in other sites and poorly-differentiated neuroendocrine cancers arising in the lung. (See "Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging" and "Staging, treatment, and posttreatment surveillance of nonmetastatic, well-differentiated gastrointestinal tract neuroendocrine (carcinoid) tumors" and "Extensive stage small cell lung cancer: Initial management" and "Role of surgery in multimodality therapy for small cell lung cancer".)
For patients with localized bronchial neuroendocrine tumors (NETs), surgical resection is the preferred treatment approach assuming adequate pulmonary reserve. For patients whose condition does not permit complete resection and for exceptional low-grade cases where the lesion is entirely endobronchial, trans-bronchoscopic resection may be an alternative. (See 'Endobronchial management' below.)
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- LOCALIZED DISEASE
- Surgical resection
- - Proximal tumors
- - Peripheral tumors
- - Role of lymph node dissection
- - Prognosis
- Typical bronchial NETs
- Atypical bronchial NETs
- - Role of adjuvant therapy
- Recommendations of expert groups
- Endobronchial management
- - Resection
- - Cryotherapy
- Inoperable and locally-advanced unresectable disease
- Posttreatment surveillance
- METASTATIC DISEASE AND MANAGEMENT OF THE CARCINOID SYNDROME
- Somatostatin analogs
- Liver-directed therapy
- Radiation therapy
- Cytotoxic chemotherapy
- Peptide receptor radioligand therapy
- SUMMARY AND RECOMMENDATIONS