Papillary fibroelastoma, unusual cause of stroke in a young man: a case report
© The Author(s). 2017
Received: 29 November 2016
Accepted: 10 May 2017
Published: 19 May 2017
Papillary fibroelastoma is the third most common primary benign tumor with an incidence of up to 0.33% in autopsy series; it accounts for approximately 75% of all cardiac valvular tumors.
We describe a rare case of a 28-Year-old man that while playing football, had a sudden onset of neurological deficit: aphasia, right hemiparesis and right facial numbness. Transthoracic echocardiography (TTE) showed a 10x10 mm mass attached to the anterior mitral valve leaflet. The patient was treated surgically for the prevention of further embolic complications. Histologic examination of the resected mass revealed a papillary fibroelastoma. It is the third most frequent primary cardiac tumor, after myxoma and fibroma, and the most common primary tumor of heart valves. Despite the benign nature of this tumor, it carries very high risk of embolic complications. The successful complete resection of the papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent.
Differential diagnosis of cardiac masses requires clinical informations, laboratory tests, blood cultures and appropriate use of imaging modalities. Papillary fibroelastoma is a potential cause of embolic stroke in the young. The prompt surgical excision of papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent.
KeywordsPapillary fibroelastoma Cardiac tumors Cardioembolic stroke Cerebrovascular disease Case report
Papillary fibroelastoma is the third most common primary cardiac benign tumor with an incidence of up to 0.33% in autopsy series; it accounts for approximately 75% of all cardiac valvular tumors and affects men and women equally with a mean age of 60 years at diagnosis. Despite the benign nature of this tumor, it carries very high risk of embolic complications. Here we described a case of stroke in a 28-year-old man due to cerebral embolization originated from a cardiac papillary fibroelastoma.
A 28-years-old man was admitted to the Emergency Department of our Hospital for the sudden onset of aphasia, right facial numbness and right hemiparesis, while playing football. He was not an athlete and never performed a medical examination for sports fitness.
Here we described a rare and very special case of stroke in a 28-year-old man due to cerebral embolization originated from a cardiac papillary fibroelastoma. Stroke in the young, with no obvious risk factors, usually requires extensive evaluation: it is important to look for secondary cause of cerebral ischemia, searching carefully especially diseases with potential cardioembolic.
Papillary fibroelastoma is the third most common primary benign tumor with an incidence of up to 0.33% in autopsy series; it accounts for approximately 75% of all cardiac valvular tumors and affects men and women equally with a mean age of 60 years at diagnosis. According to epidemiological data described cases rarely involve young patients [1, 2]. Althought papillary fibroelastomas are histologically benign neoplasms, they may result in life-threatening complications if valve obstruction or systemic embolization occurs, as described in our patient.
Most patients are asymptomatic, but some patients may experience cerebral embolic symptoms, such as stroke or transient ischemic attack or angina, acute coronary syndrome, myocardial infarction or death from coronary ostial obstruction [3–25]: transient ischemic attack/cerebrovascular accident is considered by far the most common presentation of papillary fibroelastoma . This tumor has a predilection for the left side of the heart: the aortic valve is the predominant site involved, followed by mitral leaflets. Grossly, papillary fibroelastoma resemble a “sea anemone”. This tumor usually has a gelatinous membrane on the surface and a stalk with multiple delicate papillary projections, best appreciated by immersing the specimen in water . Microscopically, it is characterized by a collection of avascular fronds of dense connettive tissue lined by endothelium and may arise from any endocardial surface. Embolic fragments may originate from the tumor itself and this occurs because of its very friable and soft texture, or from surface formation of platelet and fibrin thrombi . Most are solitary and small, some are mobile and appear more likely to give rise to embolism. Tumor mobility has been described to be an independent predictor of death or non-fatal embolization . Echocardiography is the preferred means for evaluation of papillary fibroelastomas . Due to their small size generally 0.5 to 2.0 cm in diameter and their valvular involvement, papillary fibroelastoma may be difficult to distinguish from valvular vegetation. For this reason, clinical informations, laboratory tests and blood cultures are extremely important for differential diagnosis. The differential diagnosis includes the presence of mixoma, lipoma, rhabdomyoma or amorphous tumors. Since symptomatic papillary fibroelastoma carries a definite risk of severe complications, aggressive surgical management is recommended, irrespective of the tumor’s size or the patient’s symptoms , the successful complete resection of the papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent. The patients who are not surgical candidates could be offered long-term oral anticoagulation, although non randomized controlled data are available on its efficacy.
Echocardiography transthoracic and transesophageal are essential investigations in young patients with stroke. Differential diagnosis of cardiac masses requires clinical informations, laboratory tests, blood cultures and appropriate use of imaging modalities. Papillary fibroelastoma is a potential cause of embolic stroke in the young. The prompt surgical excision of papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent.
This study received no external funding.
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All data are in the medical record of the patient.
All the authors have made substantial contributions in data interpretation, and revised the manuscript critically. All authors read and approved the final manuscript.
The authors declare that they have no competing interest.
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