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Carcinoid Syndrome
Darragh Brady, MD; David Murphy, MD; Jonathan D. Dodd, MD, MSc, MRCPI, FFR(RCSI)
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Hyperserotonemia
            • Thorson-Bioerck syndrome
            • Argentaffinoma syndrome
            • Cassidy-Scholte syndrome
            • Hedinger’s syndrome (carcinoid heart disease)
          • Definitions

            • Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
              • Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
                • Most have midgut origin (ileocecal region/appendix)
                • Origin can also be from lung due to common origin of foregut and respiratory diverticulum during 4th week of fetal development; 18% of time primary origin cannot be found
              • Clinical symptoms occur when secretory products are directly released into systemic circulation or when hepatic metabolism is overwhelmed
                • Cells secrete vasoactive substances
                  • Serotonin [5-hydroxytryptamine (5-HT)] production is most prominent, especially in midgut tumors
                  • Bradykinins, tachykinins, histamine, substance P, and adrenocorticotropic hormone (ACTH) also reported
                • Tumor products activate mitogenic pathways on exposed endocardial surfaces inducing valvular fibrosis
                • Tricuspid and pulmonary valves are involved on upstream side where vasoactive substance levels are highest
                • 2nd most common cause of tricuspid stenosis (there is always concomitant regurgitation)
                • Left side can be involved in pulmonary carcinoid or patent foramen ovale but less likely due to pulmonary deactivation of vasoactive substances

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Davar J et al: Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 69(10):1288-1304, 2017
                                        2. Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
                                        3. Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
                                        4. Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
                                        5. Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
                                        6. Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
                                        7. Kesarwani M et al: First-in-human transcatheter pulmonic valve implantation through a tricuspid valve bioprosthesis to treat native pulmonary valve regurgitation caused by carcinoid syndrome. JACC Cardiovasc Interv. 8(10):e161-3, 2015
                                        8. Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
                                        9. Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
                                        10. Haugaa KH et al: Evaluation of right ventricular dysfunction by myocardial strain echocardiography in patients with intestinal carcinoid disease. J Am Soc Echocardiogr. 24(6):644-50, 2011
                                        11. Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
                                        12. Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
                                        13. Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
                                        14. Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
                                        15. Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Hyperserotonemia
                                                  • Thorson-Bioerck syndrome
                                                  • Argentaffinoma syndrome
                                                  • Cassidy-Scholte syndrome
                                                  • Hedinger’s syndrome (carcinoid heart disease)
                                                • Definitions

                                                  • Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
                                                    • Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
                                                      • Most have midgut origin (ileocecal region/appendix)
                                                      • Origin can also be from lung due to common origin of foregut and respiratory diverticulum during 4th week of fetal development; 18% of time primary origin cannot be found
                                                    • Clinical symptoms occur when secretory products are directly released into systemic circulation or when hepatic metabolism is overwhelmed
                                                      • Cells secrete vasoactive substances
                                                        • Serotonin [5-hydroxytryptamine (5-HT)] production is most prominent, especially in midgut tumors
                                                        • Bradykinins, tachykinins, histamine, substance P, and adrenocorticotropic hormone (ACTH) also reported
                                                      • Tumor products activate mitogenic pathways on exposed endocardial surfaces inducing valvular fibrosis
                                                      • Tricuspid and pulmonary valves are involved on upstream side where vasoactive substance levels are highest
                                                      • 2nd most common cause of tricuspid stenosis (there is always concomitant regurgitation)
                                                      • Left side can be involved in pulmonary carcinoid or patent foramen ovale but less likely due to pulmonary deactivation of vasoactive substances

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Echocardiographic Findings

                                                          • Angiographic Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Davar J et al: Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 69(10):1288-1304, 2017
                                                                              2. Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
                                                                              3. Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
                                                                              4. Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
                                                                              5. Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
                                                                              6. Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
                                                                              7. Kesarwani M et al: First-in-human transcatheter pulmonic valve implantation through a tricuspid valve bioprosthesis to treat native pulmonary valve regurgitation caused by carcinoid syndrome. JACC Cardiovasc Interv. 8(10):e161-3, 2015
                                                                              8. Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
                                                                              9. Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
                                                                              10. Haugaa KH et al: Evaluation of right ventricular dysfunction by myocardial strain echocardiography in patients with intestinal carcinoid disease. J Am Soc Echocardiogr. 24(6):644-50, 2011
                                                                              11. Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
                                                                              12. Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
                                                                              13. Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
                                                                              14. Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
                                                                              15. Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993