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Metastatic carcinoid tumor to the heart: echocardiographic-pathologic study of 11 patients
Utpal H Pandya1, Patricia A Pellikka, Maurice Enriquez-Sarano
1Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Insights
Metastatic carcinoid tumor in the heart (MCH) is rare but detectable by echocardiography (echo) if tumors are at least 1.0 cm. Echocardiography is crucial for identifying MCH in carcinoid syndrome patients, even without valve issues.
Area of Science:
- Cardiology
- Oncology
- Medical Imaging
Background:
- Carcinoid heart disease typically presents as right-sided valvular dysfunction.
- Cardiac metastases from carcinoid tumors are infrequent and poorly characterized.
- Metastatic carcinoid tumor involving the heart (MCH) features require detailed investigation.
Purpose of the Study:
- To investigate the clinical and echocardiographic characteristics of metastatic carcinoid tumor in the heart (MCH).
- To describe the features of patients diagnosed with MCH.
- To assess the diagnostic utility of echocardiography in detecting MCH.
Main Methods:
- Retrospective review of echocardiograms from 11 patients with pathologically confirmed MCH (1985-1999).
- Analysis of patient demographics, primary tumor location, and metastatic tumor characteristics.
- Correlation of echocardiographic findings with pathological data and autopsy records.
Main Results:
- All MCH patients (mean age 58 years) had carcinoid syndrome, with primary tumors in the small bowel (83%) and hepatic metastases.
- Intramyocardial metastases were identified, involving ventricles and septum.
- Echocardiography detected 9 of 15 MCH tumors (mean size 2.4 cm) in 6 patients (55%); smaller tumors (<0.4 cm) were missed.
- Carcinoid valve disease was present in 8 patients; MCH was the primary indication for cardiac surgery in one patient.
Conclusions:
- Metastatic carcinoid tumor in the heart (MCH) is uncommon but identifiable by echocardiography for tumors ≥1.0 cm.
- Echocardiography should be routinely included in the cardiac evaluation of patients with carcinoid syndrome.
- MCH can be the sole cardiac manifestation of carcinoid heart disease, especially in the absence of valvular dysfunction.
Objective:
We sought to investigate the clinical and echocardiographic (echo) characteristics of metastatic carcinoid tumor in the heart.
Background:
Right-sided valvular dysfunction is the hallmark of carcinoid heart disease. Cardiac metastases are uncommon in carcinoid syndrome. Features of patients with metastatic carcinoid tumor involving the heart (MCH) have not been well described.
Methods:
From 1985 through 1999, 11 patients (8 male, 3 female), mean age +/- standard deviation, 58 +/- 6 years, were seen who had pathologically confirmed MCH. All patients had echoes, which were reviewed retrospectively.
Results:
All patients with MCH had carcinoid syndrome. The primary carcinoid tumor was in the small bowel in 83% of patients, and all patients had hepatic metastases. On pathologic review, the 11 patients had 15 MCH tumors. All metastases were intramyocardial. The MCH involved the right ventricle in 40%, left ventricle in 53%, and ventricular septum in 7%. The average size of macroscopic tumors was 1.8 +/- 1.2 cm. Nine MCH tumors were detected by echo in 6 of the 11 patients (55%). Mean echo-detected tumor size was 2.4 cm (range, 1.2 to 4). All tumors noted by echo were well circumscribed, non-infiltrating, and homogeneous. In the 5 other patients, review of autopsy records revealed 6 macroscopic tumors, mean size 0.35 cm (range, 0.2 to 0.4), none detected by echo even retrospectively. Carcinoid valve disease was present in 8 of the 11 MCH patients. The tricuspid valve was affected in all 8 patients (73%), pulmonary valve in 7 (64%), and left sided valves in 4 (36%) All patients with MCH identified by echo had cardiac surgery, 3 primarily for carcinoid valve disease and 2 for non-carcinoid cardiac disease; in 1 patient, MCH was the primary indication for cardiac surgery.
Conclusions:
MCH is uncommon but can be easily identified by echo if tumor size is >/=1.0 cm. In patients without valvular dysfunction, MCH may be the only manifestation of carcinoid heart disease. A search for MCH should be an integral part of the echo exam in patients with carcinoid syndrome.
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