ASSOCIATION OF WILMS-TUMOR WITH PRIMARY BRAIN-TUMOR IN SIBLINGS

The cases of two young male siblings independently developing unilater al Wilms' tumors and brain tumors are reported. The renal tumors were resected; the first child was treated with chemotherapy and the second child was given additional radiotherapy. Five years after treatment, both children developed a second primary neuroectodermal tumor. All fo ur tumors showed a high proliferative activity, and rapidly progressin g disease led to the death of the first child. Histopathological and m olecular studies were carried out on all four neoplasms. No functional ly relevant mutation was found in selected exons of the p53, K-ras and WT1 gene loci of tumor and germ line DNA. Since additional family mem bers had developed blain tumors and carcinomas, this peculiar associat ion of neoplasms may be due to germ line mutation of a hitherto uniden tified oncogene acting in a recessive or weakly dominant fashion.