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Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma

✍ Scribed by Harding, Cary O. ;Pagon, Roberta A.

Publisher: John Wiley and Sons
Year: 1990
Tongue: English
Weight: 502 KB
Volume: 37
Category: Article
ISSN: 0148-7299
DOI: 10.1002/ajmg.1320370402

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✦ Synopsis

Cardiac rhabdomyoma, a rare benign tumor often detected in infancy, is frequently associated with tuberous sclerosis. This association is commonly stated to occur in 50% of all cases of cardiac rhabdomyoma. Recently at our institution, the prenatal detection of a cardiac rhabdomyoma in a fetus at no known risk for tuberous sclerosis emphasized the need to determine the frequency of association in order to provide accurate diagnosis and counseling in such situations. After a review of reported cases and review of patients from our institution diagnosed to have cardiac rhabdomyoma, we estimate that 5146% of cardiac rhabdomyomas are associated with tuberous sclerosis. We present the results of our literature and case review.

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