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 [
26]. 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 [
27]. 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 [
28]. 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 [
29]. Echocardiography is the preferred means for evaluation of papillary fibroelastomas [
30]. 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 [
26], 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.