Aace/Ace Disease State Clinical Review: Diagnosis and Management of Midgut Carcinoids |
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| Institution: | 1. From the Division of Surgical Oncology and Endocrine Surgery, Vanderbilt University, Nashville, Tennessee;2. Department of Medicine, Stanford University, Palo Alto, California;3. Department of Medicine, Eastern Virginia Medical School, Norfolk, Virginia;4. Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York;5. Department of Otolaryngology, Massachusetts General Hospital, Boston, Massachusetts.;1. From the Chair; Chairman, Grunberger Diabetes Institute; Clinical Professor, Internal Medicine and Molecular Medicine & Genetics, Wayne State University School of Medicine; Professor, Medicine, Oakland University William Beaumont School of Medicince.;2. Director, AMCR Institute; Clinical Associate Professor, USCD School of Medicine.;3. Professor of Medicine, Loyola University Medical Center; Director, Loyola University Osteoporosis and Metabolic Bone Disease Center, Maywood, IL.;4. Immediate Past President, American College of Endocrinology; Past President, American Association of Clinical Endocrinologists; Medical Director, Scripps Whittier Diabetes Institute; Clinical Professor of Medicine, University of California, San Diego; Associate Editor, Journal of Diabetes; President, Diabetes and Endocrine Associates, La Jolla, CA.;5. Professor, Departments of Medicine, Biochemistry, Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX.;6. Medical Director & Principal Investigator, Metabolic Institute of America; President Elect, American College of Endocrinology.;7. Clinical Assistant Professor, Nova Southeastern University School of Osteopathic Medicine, Davie, FL.;8. Clinical Professor of Medicine, George Washington University, Alexandria, VA.;9. Professor of Medicine, Chief, Section of Endocrinology, Univeristy of Tennessee Graduate School of Medicine of Knoxville; Past President, American Association of Clinical Endocrinologists; Past President, American College of Endocrinology.;10. Managing Partner, North Texas Endocrine Center, Dallas, TX.;11. Endocrinology Associates, Houston, TX.;1. From the Departments of Medicine-Endocrinology, Mount Sinai Hospital, New York City, New York.;2. Preventative Medicine, Mount Sinai Hospital, New York City, New York.;1. From the Division of Endocrinology and Metabolism, University of California, San Francisco, California;2. Microclinic International, San Francisco, California. |
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| Abstract: | Objective: Neuroendocrine tumors (NETs) are a collection of complex tumors that arise from the diffuse endocrine system, primarily from the digestive tract. Carcinoid tumors most commonly originate from the small intestine. These tumors are either referred to as small intestinal neuroendocrine tumors or midgut carcinoids (MGCs). The purpose of this review article is to survey the diagnostic and therapeutic pathways for patients with MGC and provide an overview of the complex multidisciplinary care involved in improving their quality of life, treatment outcomes, and survival.Methods: The current literature regarding the diagnosis and management of MGCs was reviewed.Results: Dry flushing and secretory diarrhea are the hallmarks of the clinical syndrome of MGC. Managing MGC requires attention to the overall symptom complex, including the physical effects of the tumor and biomarker levels. The somatostatin analogs (SAs) octreotide and lanreotide are highly efficacious for symptomatic improvement. MGCs require resection to encompass the primary tumor and mesenteric lymph node metastases and should include cholecystectomy if the patient is likely to receive SA therapy. Debulking of liver metastasis by resection in combination with ablative therapies and other liver-directed modalities may help palliate symptoms and hormonal overproduction in carefully selected patients. Quality of life is an important measure of patients' perception of the burden of their disease and impact of treatment modalities and may be a useful guide in deciding changes in therapy to alter apparent health status.Conclusion: MGC is a challenging malignancy that requires the input of a multidisciplinary team to develop the best treatment plan. Consultation with expert centers that specialize in NETs may also be indicated for complex cases. With expert care, patients can be cured or live with the disease and enjoy good quality of life.Abbreviations: CgA = chromogranin A CT = computed tomography 5-HIAA = 5-hydroindoleacetic acid MGC = midgut carcinoid MRI = magnetic resonance imaging mTOR = mammalian target of rapamycin NET = neuroendocrine tumor NSE = neuron-specific enolase NKA = neurokinin A PET = positron emission tomography PRRT = peptide receptor radiotherapy QOL = quality of life SA = somatostatin analogue SPECT = single-photon emission computed tomography SSTR = somatostatin receptor |
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