uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
Ileal neuroendocrine tumors and heart: not only valvular consequences
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Radiology, Oncology and Radiation Science.
2015 (English)In: Endocrine (Basingstoke), ISSN 1355-008X, Vol. 48, no 3, 743-755 p.Article, review/survey (Refereed) Published
Abstract [en]

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 Ga-68-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.

Place, publisher, year, edition, pages
2015. Vol. 48, no 3, 743-755 p.
Keyword [en]
Carcinoid heart disease, Cardiac imaging, Heart metastases, Neuroendocrine tumors
National Category
Endocrinology and Diabetes
URN: urn:nbn:se:uu:diva-252455DOI: 10.1007/s12020-014-0446-0ISI: 000351661100004PubMedID: 25319177OAI: oai:DiVA.org:uu-252455DiVA: diva2:810829
Available from: 2015-05-08 Created: 2015-05-07 Last updated: 2015-05-08Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Sundin, Anders
By organisation
Department of Radiology, Oncology and Radiation Science
In the same journal
Endocrine (Basingstoke)
Endocrinology and Diabetes

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 168 hits
ReferencesLink to record
Permanent link

Direct link