作者
John R Priest, Jan Watterson, Louise Strong, Vicki Huff, William G Woods, Rebecca L Byrd, Stephen H Friend, Irene Newsham, Michael D Amylon, Alberto Pappo, Donald H Mahoney, Claire Langston, Ruth Heyn, Gloria Kohut, David R Freyer, Bruce Bostrom, Mary S Richardson, Julio Barredo, Louis P Dehner
发表日期
1996/2/1
期刊
The Journal of pediatrics
卷号
128
期号
2
页码范围
220-224
出版商
Mosby
简介
OBJECTIVE
To catalog and evaluate patterns of disease in families of children with pleuropulmonary blastoma (PPB).
METHODS
Data have been collected since 1988 on 45 children with PPB and their families. All pathologic materials were centrally reviewed. Preliminary molecular genetic analyses were performed when possible.
RESULTS
In 12 of 45 patients, an association was found between PPB and other dysplasias, neoplasias, or malignancies in the patients with or in their young relatives. The diseases found to be associated with PPB include other cases of PPB, pulmonary cysts, cystic nephromas, sarcomas, medulloblastomas, thyroid dysplasias and neoplasias, malignant germ cell tumors, Hodgkin disease, leukemia, and Langerhans cell histiocytosis. Abnormalities of the p53 tumor suppressor gene, Wilms tumor suppressor gene (WT1), and the putative second genetic locus for Wilms tumor (WT2) were …
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JR Priest, J Watterson, L Strong, V Huff, WG Woods… - The Journal of pediatrics, 1996