Cardiac fibroma
General: Benign mesenchymal tumor thought to be hamartomatous. Second or third most common tumor in children, with occasional prenatal diagnosis. Range from asymptomatic and incidentally found to severe restrictive or obstructive cardiac disease requiring transplantation. However, surgical resection has a good long-term outcome even with incomplete resection, in the absence of malignant transformation.
Gross: Most common in the septum/ventricle and is usually a solitary, well-circumscribed to infiltrative, white, and rubbery to firm intramural mass.
Microscopic: Uniform spindle-shaped cells surrounded by a variably collagenous matrix; histologically similar to fibromas elsewhere.
Differential Diagnosis:
- Cardiac rhabdomyoma, rhabdomyoma
- Fibrosarcoma/malignant transformation
Stains:
- Positive: Vimentin, smooth-muscle actin
- Negative: Desmin, MyoD1, S100, HMB45
- Suggested, focused panel:
(Lefkowitch: AP Board Review)
Images:
Cases:
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