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Presented by Fred Askin, M.D. and prepared by Carol Allan, M.D.
Case 1: Incidental subvalvular mass found in a patient undergoing aortic valve replacement.
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Question 1 of 1
1. Question
Week 81: Case 1
Incidental subvalvular mass found in a patient undergoing aortic valve replacement.images/fred1a.jpg
images/fred1b.jpg
images/fred1c.jpgCorrect
Answer: Papillary fibroelastoma (papillary elastofibroma)
Histology: See Discussion.
Discussion: Papillary elastofibroma is a benign, avascular papilloma of the endocardium. The lesion may be related to Lambl excrescences. The etiology of the lesion is unknown. It has been suggested that, like their smaller cousin the Lambl excrescence, the lesions represent organized thrombi, however the occurrence of elastofibroma in areas where there is little if any hemodynamic stress has lead to the suggestion that the lesion may be a hamartoma. It is possible that endocardial tissue may respond to sheer stresses and/or congenital factors and proliferate in an unusual way resulting in the lesion under discussion. The AFIP fascicle suggests that papillary fibroelastoma is the second most common cardiac tumor after myxoma and represents 10% of cardiac tumors. The lesions occur equally in adults of both sexes and, with the advent of cardiac surgery, are becoming common intraoperative findings. Grossly, papillary fibroelastomas are invariably located on the endocardial surface. 90% of lesions are located on valve surfaces, in contrast with the small Lambl excressions that occur at lines of closure on the valve. The lesion in the discussion occurred at the base of the calcified aortic valve. The lesion has a characteristic flower-like appearance. It may contain cystic spaces. Most tumors are 1 cm or less indiameter, but lesions as large as 5 cm have been reported. Histologically, the papillary fronds of the lesion are narrow and elongated with branching. The papillae resemble miniature chordae tendineae. The matrix of the lesion consists of mucopolysaccharides, elastic fibers and some spindle cells, which may be fibroblasts or smooth muscle in origin.
Papillary fibroelastoma differs histologically from cardiac myxoma in that the papillae lack the characteristic vessels always present in myxoma and do not have the characteristic polygonal myxoma cells. Cardiac fibroma is another fibrotic lesion of the heart but it occurs essentially within the myocardium. Cardiac hemangiomas may occur on an endocardial surface but the lesion will have the characteristic proliferation of blood vessels that marks hemangiomas in all sites. Another diagnostic problem may be the overgrowth of fibroelastoma by attached fibrin thrombi (so called marantic endocarditis). These vegetations may cause embolic symptoms and the underlying tumor (fibroelastoma) may be obscured by organizing thrombus.
Incorrect
Answer: Papillary fibroelastoma (papillary elastofibroma)
Histology: See Discussion.
Discussion: Papillary elastofibroma is a benign, avascular papilloma of the endocardium. The lesion may be related to Lambl excrescences. The etiology of the lesion is unknown. It has been suggested that, like their smaller cousin the Lambl excrescence, the lesions represent organized thrombi, however the occurrence of elastofibroma in areas where there is little if any hemodynamic stress has lead to the suggestion that the lesion may be a hamartoma. It is possible that endocardial tissue may respond to sheer stresses and/or congenital factors and proliferate in an unusual way resulting in the lesion under discussion. The AFIP fascicle suggests that papillary fibroelastoma is the second most common cardiac tumor after myxoma and represents 10% of cardiac tumors. The lesions occur equally in adults of both sexes and, with the advent of cardiac surgery, are becoming common intraoperative findings. Grossly, papillary fibroelastomas are invariably located on the endocardial surface. 90% of lesions are located on valve surfaces, in contrast with the small Lambl excressions that occur at lines of closure on the valve. The lesion in the discussion occurred at the base of the calcified aortic valve. The lesion has a characteristic flower-like appearance. It may contain cystic spaces. Most tumors are 1 cm or less indiameter, but lesions as large as 5 cm have been reported. Histologically, the papillary fronds of the lesion are narrow and elongated with branching. The papillae resemble miniature chordae tendineae. The matrix of the lesion consists of mucopolysaccharides, elastic fibers and some spindle cells, which may be fibroblasts or smooth muscle in origin.
Papillary fibroelastoma differs histologically from cardiac myxoma in that the papillae lack the characteristic vessels always present in myxoma and do not have the characteristic polygonal myxoma cells. Cardiac fibroma is another fibrotic lesion of the heart but it occurs essentially within the myocardium. Cardiac hemangiomas may occur on an endocardial surface but the lesion will have the characteristic proliferation of blood vessels that marks hemangiomas in all sites. Another diagnostic problem may be the overgrowth of fibroelastoma by attached fibrin thrombi (so called marantic endocarditis). These vegetations may cause embolic symptoms and the underlying tumor (fibroelastoma) may be obscured by organizing thrombus.
