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
MILLER RW. WILMS' TUMOR, CONGENITAL HEART DEFECTS, PHEOCHROMOCYTOMAS AND THYROID CANCER. Am J Dis Child. 1968;116(5):564-565. doi:10.1001/archpedi.1968.02100020568025