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Association of Wilms' tumor with primary brain tumor in siblings.


Rainov, N G; Lübbe, J; Renshaw, J; Pritchard-Jones, K; Lüthy, A R; Aguzzi, A (1995). Association of Wilms' tumor with primary brain tumor in siblings. Journal of Neuropathology and Experimental Neurology, 54(2):214-223.

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.

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.

Citations

9 citations in Web of Science®
8 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:1 March 1995
Deposited On:11 Feb 2008 12:26
Last Modified:05 Apr 2016 12:20
Publisher:Lippincott Wiliams & Wilkins
ISSN:0022-3069
Publisher DOI:10.1097/00005072-199503000-00008
PubMed ID:7876889

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