An unusual case of multiple papillary fibroelastoma, review of literature

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.