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WILMS' TUMOR, CONGENITAL HEART DEFECTS, PHEOCHROMOCYTOMAS AND THYROID CANCER

ROBERT W. MILLER, MD
Am J Dis Child. 1968;116(5):564-565. doi:10.1001/archpedi.1968.02100020568025.
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To the Editor.—The fascinating case report by Lynch and Green in the June issue of the Journal (115:723-727, 1968) stresses in the title and text the relationship between Wilms' tumor and congenital heart disease, for which there is little evidence.1 There is a way, which was not discussed, for linking each of the disorders in the child to the mother's bilateral pheochromocytomas with solid (medullary) thyroid carcinoma and amyloid stroma, a well defined familial syndrome.2-5 The possible role of multiple neurofibromatosis, which need not be clinically apparent, is of particular interest.

Neurofibromatosis, inherited as an autosomal dominant trait, carries a high risk of pheochromocytoma6 and has been reported with the neoplastic syndrome observed in the mother.3-5 Pheochromocytoma, which itself may be familial,7 apparently also occurs excessively in persons with congenital heart disease, including transposition of the great vessels,8,9 the anomaly found in the

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