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Hepatoblastoma in infant sisters
Author(s) -
Fraumeni Joseph F.,
Rosen Peter J.,
Hull Edgar W.,
Barth Rolf F.,
Shapiro Stephen R.,
O'Connor John F.
Publication year - 1969
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(196911)24:5<1086::aid-cncr2820240535>3.0.co;2-w
Subject(s) - hepatoblastoma , medicine , proband , wilms' tumor , hepatocellular carcinoma , beckwith–wiedemann syndrome , pathology , pediatrics , genetics , mutation , biology , gene , gene expression , dna methylation
A family in which 2 infant sisters died with hepatoblastoma, the first reported familial occurrence of this neoplasm, is presented. The children had no heritable or congenital disorders known to increase the risk of hepatoma, and laboratory studies revealed no clues to pathogenesis. The detection of serum alphafetoprotein in the proband suggests that the test may be useful in the diagnosis of hepatoblastoma as well as hepatocellular carcinoma, and in the screening of children predisposed to these neoplasms. Sibship aggregation has also been observed with adrenocortical neoplasia and Wilms' tumor, which share with hepatoblastoma an association with certain congenital malformations.