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Ileal neuroendocrine tumors and heart: not only valvular consequences.
MedLine Citation:
PMID:  25319177     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Ileal neuroendocrine tumors (NETs) often progress slowly, but because of their generally nonspecific symptoms, they have often metastasized to local lymph nodes and to the liver by the time the patient presents. Biochemically, most of these patients have increased levels of whole blood serotonin, urinary 5-hydroxyindoleacetic acid, and chromogranin A. Imaging work-up generally comprises computed tomography or magnetic resonance imaging and somatostatin receptor scintigraphy, or in recent years positron emission tomography with (68)Ga-labeled somatostatin analogs, allowing for detection of even sub-cm lesions. Carcinoid heart disease with affected leaflets, mainly to the right side of the heart, is a well-known complication and patients routinely undergo echocardiography to diagnose and monitor this. Multitasking surgery is currently recognized as first-line treatment for ileal NETs with metastases and carcinoid heart disease. Open heart surgery and valve replacement are advocated in patients with valvular disease and progressive heart failure. When valvulopathy in the tricuspid valve results in right-sided heart failure, a sequential approach, performing valve replacement first before intra-abdominal tumor-reductive procedures are conducted, reduces the risk of bleeding. Metastases to the myocardium from ileal NETs are seen in <1-4.3 % of patients, depending partly on the imaging technique used, and are generally discovered in those affected with widespread disease. Systemic treatment with somatostatin analogs, and sometimes alpha interferon, is first-line medical therapy in metastatic disease to relieve hormonal symptoms and stabilize the tumor. This treatment is also indicated when heart metastases are detected, with the addition of diuretics and fluid restriction in cases of heart failure. Myocardial metastases are rarely treated by surgical resection.
Authors:
Jan Calissendorff; Eva Maret; Anders Sundin; Henrik Falhammar
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-10-16
Journal Detail:
Title:  Endocrine     Volume:  -     ISSN:  1559-0100     ISO Abbreviation:  Endocrine     Publication Date:  2014 Oct 
Date Detail:
Created Date:  2014-10-16     Completed Date:  -     Revised Date:  2014-10-17    
Medline Journal Info:
Nlm Unique ID:  9434444     Medline TA:  Endocrine     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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