Cardiac papillary fibroelastoma: the image behind diagnosis

A 52-year-old asymptomatic man, on follow-up after a primary percutaneous coronary intervention to the left anterior descending artery, was diagnosed with a freely floating soft tissue mass measuring 23 × 25 mm with equal echogenicity with a stalk attached to the posterior mitral leaflet, on transthoracic echocardiography. Preoperative 2-dimensional (Figure 1) and 3-dimensional (Figure 2) transesophageal echocardiography showed the characteristic stippled appearance with shimmer of the peripheral edge and echolucency within the tumor, suggestive of cardiac papillary fibroelastoma. Intraoperatively, a 20 × 25 × 25-mm soft pedunculated polypoid mass was found attached to the left atrial wall 3 mm from the mitral valve P1 region. Postoperative transesophageal echocardiography following excision showed no mitral regurgitation. Histopathology showed a papillary tumor composed of filiform processes with a central acellular collagen core surrounded by myxomatous matrix. The peripheral rim and core showed coarse elastin fibrils lined by endothelial cells, confirming the diagnosis of cardiac papillary fibroelastoma (Figures 3 and 4). OPEN IN VIEWER

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Figure 2.

Three-dimensional transesophageal echocardiography showing the cardiac papillary fibroelastoma arising from the left atrium very near the P1 segment of the mitral valve.

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Figure 3.

Gross histopathological specimen of the cardiac papillary fibroelastoma.

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Figure 4.

Histology of the cardiac papillary fibroelastoma. Hematoxylin and eosin stain, original magnification ×100.