Wilms' Tumor Associated with Congenital Aniridia

The third case of the aniridia-Wilms' tumor complex in Japan is reporetd herein. The patient received a radical nephrectomy and survived the period of risk. A radiotherapy and six series of chemotherapy treatments have been used for adjuvant therapy. It was learned from this case that even 6-month intervals are not frequent enough for Wilms' tumor examinations in children with nonfamiliar aniridia. Aniridia is a very rare anomaly, with an estimated incidence of 1 in 50,000_15). Similarly, Wilms' tumor, although one of the most commonly encountered malignant tumors in infants, is also rare. An intimate relationship between these uncommon conditions has been recently discovered. According to Miller and his associates_13), who first reported the association between the two conditions in 1964, in a group of 440 patients with Wilms' tumor six had aniridia, a prevalence of 1:73. Up to 1974, 24 cases of aniridia-Wilms' tumor syndrome have been reported in English language medical literature, but there have been only two cases reported in Japan.