Metastatic gastroenteropancreatic neuroendocrine tumors: Local options to control tumor growth and symptoms of hormone hypersecretion
- Jennifer Ang Chan, MD, MPH
Jennifer Ang Chan, MD, MPH
- Dana-Farber Cancer Institute
- Matthew Kulke, MD
Matthew Kulke, MD
- Director of Dana-Farber/Brigham and Women’s Carcinoid and Neuroendocrine Tumor Program
- Associate Professor of Medicine
- Harvard Medical School
- Thomas E Clancy, MD
Thomas E Clancy, MD
- Assistant Professor of Surgery
- Harvard Medical School
Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms that are thought to arise from neuroendocrine cells and their precursors located throughout the body. These tumors are characterized by variable but most often indolent biologic behavior. They are also classically characterized by their ability to secrete peptides resulting in distinctive hormonal syndromes.
Classification and nomenclature — NETs can arise at different sites within the body and are classified according to their histologic features. While there are differences in terminology and grading depending on site of origin, all commonly used classification systems reflect a basic separation between more indolent, well-differentiated tumors (which in the digestive system have been traditionally referred to as carcinoid and pancreatic neuroendocrine [islet cell] tumors) and far more aggressive poorly-differentiated types that behave clinically more like small cell carcinoma of the lung. (See "Metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: Presentation, prognosis, imaging, and biochemical monitoring", section on 'Classification and nomenclature'.)
This topic review will cover local treatment options for patients with well-differentiated metastatic gastroenteropancreatic NETs. Systemic treatment options to control tumor growth and/or symptoms of hormone hypersecretion from gastroenteropancreatic NETs and treatment of poorly-differentiated neuroendocrine carcinomas are discussed elsewhere, as are the clinical presentation, imaging, and biochemical monitoring for patients with advanced gastroenteropancreatic NETs; pathology and classification of gastroenteropancreatic NETs; clinical features of carcinoid tumors; diagnosis of carcinoid syndrome and tumor localization; treatment of early stage carcinoid tumors; bronchial carcinoid tumors; localization and treatment of pancreatic NETs; evaluation and management of NETs of unknown primary site; management of symptoms of the carcinoid syndrome; carcinoid heart disease; and functioning pancreatic neuroendocrine tumors.
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- Classification and nomenclature
- NATURAL HISTORY AND TREATMENT OPTIONS
- OVERVIEW OF THE APPROACH TO LOCAL TREATMENT
- HEPATIC PREDOMINANT METASTATIC DISEASE
- Surgical resection
- Nonsurgical liver-directed therapy
- - Ablation
- - Hepatic artery embolization
- Technique and outcomes
- Liver transplantation
- MANAGEMENT OF THE PRIMARY TUMOR IN PATIENTS WITH METASTATIC DISEASE
- PREVENTION AND MANAGEMENT OF CARCINOID CRISIS
- SUMMARY AND RECOMMENDATIONS