Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma.
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 51-86% of cardiac rhabdomyomas are associated with tuberous sclerosis. We present the results of our literature and case review.
Harding
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Department of Pediatrics, University of Washington School of Medicine, Seattle.
Pagon
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RA
D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis; D006338:Heart Neoplasms / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012207:Rhabdomyoma / Q000150:complications* / Q000453:epidemiology; D014402:Tuberous Sclerosis / Q000150:complications*