Papillary fibroelastoma
| Papillary fibroelastoma | |
|---|---|
avascular branching papillae, H&E stain | |
| Specialty | Oncology, cardiology  |
A papillary fibroelastoma is a primary tumor of the heart that typically involves one of the heart valves.[1] Papillary fibroelastomas, while considered uncommon, make up about 10 percent of all primary tumors of the heart.[2] They are the third most common type of primary tumor of the heart,[3] behind cardiac myxomas and cardiac lipomas.
Signs and symptoms
A papillary fibroelastoma is generally considered pathologically benign,
Symptoms due to papillary fibroelastomas are generally due to either mechanical effects of the tumor or due to embolization of a portion of the tumor to a distal organ. In particular, chest pain or syncope may be due to transient occlusion of the left main coronary artery by the tumor,[8] while a heart attack or sudden cardiac death may be due to embolization of a portion of the tumor into a coronary artery.[9]
Diagnosis
Papillary fibroelastoma are typically found and accurately diagnosed by
]Treatment
If the tumor is found incidentally in an asymptomatic person, the treatment approach is controversial. Certainly a conservative approach is warranted in certain individuals.[10] If the tumor is large, greater than 1 cm in asymptomatic patients,[11] and pedunculated, a case may be made for surgical excision prior to symptoms developing due to the higher risk of embolism. However, this is still considered controversial.[12]
If the papillary fibroelastoma is associated with symptoms, surgical excision is generally recommended for relief of symptoms.[10] A minimally invasive approach may be possible if the tumor involves the aortic valve[13] or right atrium.[14] In the case of aortic valve involvement, excision of the tumor is often valve-sparing, meaning that replacement of the valve with a prosthetic valve is not necessary. Repair of the native valve with a pericardial patch has been described.[15]