A Matter of the Heart: Myocardial Metastases in Neuroendocrine Tumors

The aim of the present study was to evaluate frequency, clinical spectrum, and treatment of myocardial metastases in patients with histologically proven neuroendocrine tumors by analysis of our database and literature review. The literature on cardiac metastases in patients with neuroendocrine tumors published from 1973 to the present was reviewed for age, sex, primary tumor localization, metastases, symptoms, complications, treatment, diagnostic methods, and histology. Patient records from our institution were analyzed retrospectively for cardiac metastases detected by any diagnostic means and detailed patient histories are given. 4 patients with myocardial metastases could be identified in our database (n = 550) while literature review identified 41 published cases. Mean age at initial diagnosis was 57.5 years (females = 13, males = 28), primary tumor localizations were foregut (n = 7), midgut (n = 28), hindgut (n = 1), or unknown (n = 3). Carcinoid syndrome was reported for 28 patients. Cardiac involvement was right-ventricular only (n = 10), left-ventricular only (n = 11), or biventricular (n = 10). Diagnosis was obtained by echocardiography (n = 21), CT/MRI (n = 12) and other methods (n = 9), or by autopsy (n = 9). We describe visualization of cardiac metastases by Ga-68-DOTATOC-PET/CT for the first time. Clinical presentation ranged from asymptomatic patients to cardiac arrest. Followup times ranged from < 1 month up to 12 years. Clinicians treating patients with neuroendocrine tumors should be aware of the heart as a possible site of metastatic disease. Echocardiography and MRI are the methods of choice for follow-up, while PET/CT might contribute to earlier and more frequent detection. Management of cardiac metastases requires close cooperation between specialists of internal medicine, nuclear medicine, and cardiac surgery.