Cardiac tumors and associated hereditary syndromes in children

Cardiac tumors and associated hereditary syndromes in children

Cardiac Tumors and Associated Hereditary Syndromes in Children Rhabdomyomas, fibromas and myxomas account for 66 percent of all primary cardiac tumors in children. These tumors in young patients appear to be associated with familial syndromes with autosomal dominant inheritance. Vidaillet reports the characteristic physical findings that may help the clinician identify patients with these syndromes.

The most common cardiac tumor in children is rhabdomyoma. Some authorities believe these tumors are hamartomas rather than true neoplasms. Approximately 30 percent of patients with rhabdomyomas have tuberous sclerosis. Characteristically, rhabdomyomas in patients with tuberous sclerosis are located within the wall of the myocardium rather than in the cardiac cavity. Cutaneous features of tuberous sclerosis include facial angiofibromas, ungual fibromas and hypomelanotic macules.

The second most common cardiac tumor in children is fibroma. Typically, cardiac fibromas are solitary ventricular structures and occur in children under ten years of age. A small percentage of the children with these tumors have the nevoid basal cell carcinoma syndrome. Cutaneous features of this syndrome include nevoid basal cell carcinomas, milia and palmar pits.

Myxoma is the third most common cardiac tumor in children. A small subset of pediatric patients with myxomas exhibit other characteristics, including pigmented skin lesions and peripheral and endocrine neoplasms. These features in association with cardiac myxoma constitute what is known as the myxoma syndrome. A recent study reported that 23 percent of children with cardiac myxoma exhibited the myxoma syndrome. Cutaneous features include lentiginosis, myxomas, neurofibromas and blue nevi.

The common genetic mechanism that explains the association of these cardiac tumors with specific hereditary multisystem syndromes is not understood. (American Journal of Cardiology, June 1, 1988, vol. 61, p. 1355.)

COPYRIGHT 1989 American Academy of Family Physicians

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