PRACTICAL IMPLICATIONS
Cardiac papillary fibroelastoma is an uncommon cause of cardioembolic stroke with the potential to achieve a good outcome after acute reperfusion therapies (both IV fibrinolysis and endovascular thrombectomy) and surgical removal of the tumor as soon as possible after stroke.
We present 3 cases of cardiac papillary fibroelastoma (PFE) revealed by acute ischemic stroke (table).
Table.
Principal features of cases 1, 2, and 3
Case 1 is a 38-year-old man who presented with sudden expressive language disorder and right hemiparesis. CT angiography showed a left middle cerebral artery (MCA) occlusion. Acute reperfusion therapy with endovascular thrombectomy (EVT) was achieved successfully. He was monitored in a stroke unit. The aspirated tissue was macroscopically congruent with a fibrin clot but the material was insufficient for pathologic analysis. A brain MRI performed a few days after admission showed a subacute ischemic stroke (figure, A). Blood tests, including hypercoagulability and autoimmunity assays, were normal or negative. Serial EKG and EKG Holter showed sinus rhythm. Transthoracic echocardiography (TTE) revealed an intracardiac mobile mass, with no history of fever or general malaise and negative blood cultures, suggestive of a primary cardiac tumor. Transesophageal echocardiography (TEE) (figure, B) confirmed the presence of an 11-mm pediculate mass bulging from the auricular surface of the mitral valve, with no valve or cardiac dysfunction. The suspicion of a primary cardiac tumor was supported.
Figure. MRI of the brain, transesophageal echocardiography, and pathologic examination of cases 1, 2, and 3.
(A) Fluid-attenuated inversion recovery MRI sequence of the brain shows a subacute ischemic stroke in all cases, involving the left deep structures (caudate, putamen nucleus, and adjacent white matter) in case 1 (left), the right precentral region in case 2 (middle), and the left temporoparietal lobe in case 3 (right). (B) Transesophageal echocardiography reveals an 11-mm pediculate mobile mass (arrows) bulging from the auricular surface of the mitral valve in case 1 (left), 14 mm bulging from the auricular surface of the mitral valve in case 2 (middle), and 9 mm bulging from left atrial appendage in case 3 (right). Frayed edges of the mass can be seen in the image on the right. (C) Pathologic examination demonstrates avascular papillomas with a single layer of endocardial cells covering the papillary surface of a matrix of connective tissue (left, middle, and right).
Case 2 is a 64-year-old man who presented with sudden left hemiparesis and dysarthria. CT angiography showed a right MCA occlusion. Endovascular acute reperfusion was attempted, but when the arteriography was performed, there had been a distal migration of the thrombus. He was monitored in a stroke unit. A brain MRI showed an established stroke (figure, A). Blood tests and EKG Holter were normal. TTE showed a pediculate mobile mass on the mitral valve. TEE (figure, B) confirmed its presence, 14 mm, in absence of valve dysfunction. There was no history of fever, raising the suspicion of a primary cardiac tumor.
Case 3 is a 73-year-old man who presented with sudden expressive language disorder and right hemiparesis. There was no established stroke or arterial occlusion on CT scan/CT angiography. IV alteplase (recombinant tissue plasminogen activator [rtPA]) was administered in a stroke unit, with a good response. The brain MRI demonstrated a left temporoparietal stroke (figure, A). Blood tests were normal. Serial EKG and EKG Holter showed sinus rhythm. TTE revealed an auricular mass. TEE (figure, B) defined a 9-mm pediculate mass with frayed edges bulging from the left atrial appendage, suggestive of a primary cardiac tumor. There was no cardiac dysfunction.
In all 3 cases, a multidisciplinary team decided on surgical treatment of the intracardiac masses by scheduled surgical excision 2 weeks after the ischemic stroke. Simple excision of the tumor was performed in cases 1 and 3. Excision with biologic valve replacement was needed in case 2. No major surgical complications were reported. Pathologic examination of the surgically removed pieces confirmed the diagnosis of PFE (figure, C).
Discussion
The frequency of primary cardiac tumors is very low (0.02%)1,2; hence, a primary cardiac tumor embolization is a very rare entity. Cardiac PFE are the second most common primary cardiac tumors (10%)1,3 after myxomas.1,2,4 Most PFE are found incidentally, but when they are symptomatic, stroke or TIA is the most common clinical presentation.2,5 Echocardiogram usually demonstrates a small (≤1 cm) mobile mass arising from the mitral valve (less frequently from the aortic or tricuspid valve or auricular or ventricular surface), with a frayed pattern near the edges, correlated with the papillary projections of the tumor.1,2 Pathologic examination of the surgical piece confirms the diagnosis,2 as it did in our cases. The definitive and curative treatment for symptomatic PFE is surgical excision of the tumor, performed as soon as possible to diminish the risk of early recurrences.1–6 Prognosis after surgical removal is excellent.2 However, patients with known fibroelastoma may still have a stroke while waiting for cardiac surgery. Furthermore, some patients could reject surgery or not be surgical candidates. There are limited data about acute stroke reperfusion therapy outcomes due to PFE embolization.6 The underlying pathologic process is difficult to achieve, since emboli could arise either from the papillary fronds of the PFE or from the thrombi formed on the surface of the tumor.2,3,5 In our cases, the good clinical outcomes (NIH Stroke Scale score 0 in all cases at discharge), related to the use of IV rtPA in case 3, to EVT in case 1, and the probable spontaneous recanalization of the thrombus in case 2, suggest that the underlying pathologic process was a thrombus formed on the surface of the PFE. In this setting, acute reperfusion therapies could improve outcomes in patients with acute stroke with known and unknown PFE. No complications derived from IV rtPA or EVT were reported. This case series suggests that IV rtPA or EVT have the potential to be beneficial in this population.
Acknowledgment
The authors thank Dr. Saiz Camin and Dr. García de Casasola (Pathology Department, Cruces University Hospital, Barakaldo, Spain), who confirmed the pathologic diagnosis of papillary fibroelastomas, and Dr. Rodríguez Delgadillo, Dr. Cortés Correa, Dr. Crespo de Hubsch, Dr. Rivas Figueredo, Dr. Cubero Idoiaga, and Dr. Voces Sánchez (Cardiovascular Surgery Department, Cruces University Hospital, Barakaldo, Spain), who performed the surgical procedures.
Author contributions
A. Moreno-Estébanez: drafting/revising the manuscript, contribution of vital reagents/tools/patients, acquisition of data. A. Luna Rodriguez: drafting/revising the manuscript, acquisition of data, study supervision. I. Ugarriza Serrano: drafting/revising the manuscript, contribution of vital reagents/tools/patients, E. Blanco Martín: drafting/revising the manuscript, acquisition of data, study supervision. T. Gonzalez-Pinto Gonzalez: drafting/revising the manuscript, study supervision. J. Larrazabal López: drafting/revising the manuscript, contribution of vital reagents/tools/patients. T. Perez Concha: drafting/revising the manuscript, study concept or design, analysis or interpretation of data, acquisition of data, study supervision.
Study funding
No targeted funding reported.
Disclosure
The authors report no disclosures. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.
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