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An unusual case of multiple papillary fibroelastoma, review of literature
Fatima Eslami-Varzaneh1, Erwin A Brun, Pamela Sears-Rogan
1Department of Pathology, Washington Hospital Center, 110 Irving Street NW, Washington, DC 20010-2975, USA.
Insights
Papillary fibroelastomas are rare heart tumors, often found incidentally. This case highlights multiple tumors, a rare occurrence, emphasizing the need for prompt diagnosis and treatment to prevent serious cardiac events.
Area of Science:
- Cardiovascular Pathology
- Cardiac Oncology
- Diagnostic Imaging
Background:
- Primary cardiac tumors are rare, with papillary fibroelastoma being the third most common.
- Papillary fibroelastomas typically affect cardiac valves and are often asymptomatic incidental findings.
- Despite being asymptomatic, these tumors pose significant risks, including systemic emboli, stroke, myocardial infarction, and sudden cardiac death.
Observation:
- Multiple papillary fibroelastomas are exceptionally rare in medical literature.
- This case presents an unusual instance of multiple papillary fibroelastomas in a female patient.
- The patient presented with shortness of breath and a recent cerebrovascular accident.
Findings:
- Echocardiography is crucial for initial detection but requires histological confirmation.
- Differential diagnosis for cardiac masses includes myxoma, vegetation, thrombi, lipoma, and pseudopapillary fibroelastoma.
- The presence of multiple tumors, though rare, necessitates thorough evaluation and management.
Implications:
- Early diagnosis and surgical correction of papillary fibroelastomas are vital to prevent severe embolic events.
- This case underscores the importance of considering rare presentations of common cardiac tumors.
- Further research into the etiology and management of multiple papillary fibroelastomas may improve patient outcomes.
Abstract:
Primary neoplasms of the cardiac valves are extremely rare. However, papillary fibroelastoma is the third most common primary tumor of the heart [Ann Thorac Surg 52 (1991) 1127]. These tumors can be found anywhere in the heart, but most commonly involve the cardiac valves [Ann Thorac Surg 52 (1991) 1127; McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, vols. 1-3. Washington (DC): Armed Forces Institute of Pathology; 1978. p. 20-5]. Most papillary fibroelastomas do not cause symptoms and are usually incidental findings by routine echocardiography or at autopsy. However, early diagnosis of this condition is important, since it represents a surgically correctable cause of systemic emboli, stroke, myocardial infarction, and sudden cardiac death [Ann Thorac Surg 52 (1991) 1127; Ann Thorac Surg 68 (1999) 1881; J Am Soc Echocardiogr 9 (1996) 353; Tex Heart Inst J 22 (1995) 327; Tex Heart Inst J 26 (1999) 298]. The echocardiographic findings should be confirmed by histology, since the clinical differential diagnosis includes myxoma, vegetation, thrombi, lipoma, and pseudopapillary fibroelastoma [Tex Heart Inst J 26 (1999) 298; J Am Soc Echocardiogr 11 (1998) 92; J Natl Med Assoc 87 (1995) 68]. Review of the literature reveals that multiple papillary fibroelastomas are extremely rare [Am Heart J 125 (1993) 1443; J Am Soc Echocardiogr 7 (1994) 315; Ann Thorac Surg 48 (1989) 119]. Li Manduri et al. [J Am Soc Echocardiogr 7 (1994) 315] reported multiple masses on the tricuspid valve, the larger of which was 1 cm in diameter. De Virgilio et al. [Ann Thorac Surg 48 (1989) 119] reported a case of multiple 1-cm papillary fibroelastomas located on mitral valve, left ventricular outflow tract, and along septum. We report an unusual case of multiple papillary fibroelastomas in a woman, who initially was admitted because of a shortness of breath and recent cerebrovascular accident.
