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Incidental papillary fibroelastoma of the tricuspid valve
© Strecker et al.; licensee BioMed Central Ltd. 2014
Received: 4 April 2014
Accepted: 8 July 2014
Published: 10 July 2014
Primary cardiac tumors are very rare, papillary fibroelastoma (PFE) being the second most common benign tumor of the heart in previous series. However, as a consequence of increased imaging examinations, incidental PFE may represent the most common cardiac tumor. Their clinical presentation varies from incidental asymptomatic masses to severe life-threatening cardiovascular complications necessitating emergency surgery. Here we report the diagnostic evaluation and successful surgical resection of such a cardiac tumor in a 67-year-old woman. Histology confirmed diagnosis of a papillary fibroelastoma. This report demonstrates it’s necessary to include cardiac tumors in the differential diagnosis of subtle and non-specific cardiothoracic symptoms.
Primary cardiac tumors are very rare entities among heart disease. Their frequency in previous autopsy studies ranged from 0.001 to 0.03% [1, 2]. About 75% of cardiac tumors are benign, atrial myxomas being the most common type [1, 2]. Papillary fibroelastoma (PFE) is a cardiac tumor that predominantly originates from the heart valves, oftentimes from the aortic  or mitral valve . This usually small lesion shows a characteristic echocardiographic, gross, and histological appearance .
Here we present the diagnostic evaluation and successful surgical resection of such a cardiac tumor which was found on a medical check-up in a 62-year-old patient with progressive chest pain. Histology confirmed the definite diagnosis of a papillary fibroelastoma.
PFE represents the most common tumorous lesion of cardiac valves . Few cases may arise from the atrial endocardium away from the valves and the valves leaflets . Although most PFEs are less than 10 mm in diameter, rare giant examples have been reported . The clinical symptoms of these tumors are very often non-specific, and they usually present themselves insidiously that their diagnostic and surgical management is often delayed . Reported symptoms include arrhythmias, chest pain, dyspnea, syncope and pericardial effusions with tamponade as well as intracardiac blood flow obstruction, cerebrovascular and peripheral embolization or sudden death [6–8]. The observation that most PFEs arise in diseased cardiac valves or after a history of instrumentation or previous irradiation make it likely that an initial minute lesion (nidus) is necessary for further growth of the lesion . Grossly, PFE strikingly resemble the appearance of a sea anemone upon immersion in water .
The radiological evaluation of these cardiac neoplasms has been greatly facilitated by the development of noninvasive cardiac imaging . Although TTE is really useful in the initial evaluation of suspected cardiac masses, TEE is commonly required for a more comprehensive and accurate assessment .
In the majority of cases, these tumors require operative excision to prevent potentially life-threatening complications; only a few cases may be unresectable because of their large size, and only tumor debulking may be possible in such cases . Although the long-term prognosis of asymptomatic tumors is often good, fatal histories have been reported for untreated symptomatic tumors. Therefore, if a cardiac tumor is found to cause symptoms, indication for operation should be liberal .
In summary, we described an unusual case of a PFE on the tricuspid valve.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We acknowledge support by Deutsche Forschungsgemeinschaft and Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) within the funding program Open Access Publishing.
The authors have no funding, financial relationships or conflicts of interest to disclose.
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