Objective: A personal observation of a 20-week-old fetus with Beckwith-Wiedemann syndrome (BWS) presenting epicardial angiofibroma prompted us to evaluate cardiac neoplasms in this genetic condition. Method: We performed an autopsy and a histological evaluation of the fetus, and searched the literature for cardiac anomalies in BWS. Results: Although cardiac tumors are exceptional and although BWS is rare, we found two other cardiac neoplasms in infants with BWS, whereas no more than one was expected. Conclusion: Besides an excess of cardiac malformation, BWS seems to favor an excess of cardiac tumors, which may occur very early.
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