title: Association of Wilms' tumor with primary brain tumor in siblings.
abstract: The cases of two young male siblings independently developing unilateral 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 four tumors showed a high proliferative activity, and rapidly progressing disease led to the death of the first child. Histopathological and molecular studies were carried out on all four neoplasms. No functionally 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 members had developed brain tumors and carcinomas, this peculiar association of neoplasms may be due to germ line mutation of a hitherto unidentified oncogene acting in a recessive or weakly dominant fashion.
authors.affiliation: Neurosurgical Department, Martin-Luther-University, Halle, Germany.
authors.forename: N G
authors.initials: NG
authors.lastname: Rainov
authors.forename: J
authors.initials: J
authors.lastname: Lübbe
authors.forename: J
authors.initials: J
authors.lastname: Renshaw
authors.forename: K
authors.initials: K
authors.lastname: Pritchard-Jones
authors.forename: A R
authors.initials: AR
authors.lastname: Lüthy
authors.forename: A
authors.initials: A
authors.lastname: Aguzzi
mesh_terms: D001483:Base Sequence; D001932:Brain Neoplasms / Q000235:genetics* / Q000473:pathology; D002675:Child, Preschool; D016162:Genes, Wilms Tumor / Q000235:genetics; D016158:Genes, p53 / Q000235:genetics; D011905:Genes, ras / Q000235:genetics; D005910:Glioma / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008969:Molecular Sequence Data; D016609:Neoplasms, Second Primary / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D016133:Polymerase Chain Reaction; D009396:Wilms Tumor / Q000235:genetics* / Q000473:pathology