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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
- Cells secrete vasoactive substances
- Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
- Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
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
- 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
- Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
- Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
- Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
- Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
- Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
- 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
- Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
- Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
- 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
- Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
- Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
- Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
- Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
- Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993
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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
- Cells secrete vasoactive substances
- Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
- Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
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
- 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
- Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
- Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
- Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
- Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
- Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
- 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
- Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
- Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
- 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
- Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
- Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
- Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
- Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
- Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993
