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Ileal neuroendocrine tumors and heart: not only valvular consequences

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Abstract

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 68Ga-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.

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Acknowledgments

We thank Associate Professor Anders Höög for kindly providing Figs. 1 and 2. We also thank Professor Kenneth Caidal for valuable comments. This study was supported by the Magn. Bergvalls Foundation, the Swedish Endocrine Society, the Karolinska Institute and Stockholm County Council.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Clinical Science and Education, Södersjukhuset, Karolinska Institute, Stockholm, Sweden

    Jan Calissendorff

  2. Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm, Sweden

    Eva Maret, Anders Sundin & Henrik Falhammar

  3. Department of Clinical Physiology, Karolinska University Hospital, Stockholm, Sweden

    Eva Maret

  4. Department of Radiology, Oncology and Radiation Sciences, Uppsala University, Uppsala, Sweden

    Anders Sundin

  5. Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden

    Henrik Falhammar

  6. Division of Medicine, Department of Endocrinology, Södersjukhuset, Sjukhusbacken 10, 118 83, Stockholm, Sweden

    Jan Calissendorff

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  1. Jan Calissendorff
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Correspondence to Jan Calissendorff.

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Calissendorff, J., Maret, E., Sundin, A. et al. Ileal neuroendocrine tumors and heart: not only valvular consequences. Endocrine 48, 743–755 (2015). https://doi.org/10.1007/s12020-014-0446-0

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