Association between hepatoblastoma and polyposis coli.
نویسندگان
چکیده
We have identified five children with hepatoblastoma who have a family history of polyposis coli affecting the mother and maternal relatives. We believe that the familial association of these two conditions is unlikely to have occurred by chance. Cytogenetic studies on three of the families have shown no evidence of a chromosomal abnormality.
منابع مشابه
Hepatoblastoma and APC gene mutation in familial adenomatous polyposis
Background-Hepatoblastoma is a rare, rapidly progressive, usually fatal childhood malignancy, which if confined to the liver can be cured by radical surgical resection. An association between hepatoblastoma and familial adenomatous polyposis (FAP), which is due to germline mutation of the APC (adenomatous polyposis coli) gene, has been confirmed, but correlation with site ofAPC mutation has not...
متن کاملHepatoblastoma and APC gene mutation in familial adenomatous polyposis.
BACKGROUND Hepatoblastoma is a rare, rapidly progressive, usually fatal childhood malignancy, which if confined to the liver can be cured by radical surgical resection. An association between hepatoblastoma and familial adenomatous polyposis (FAP), which is due to germline mutation of the APC (adenomatous polyposis coli) gene, has been confirmed, but correlation with site of APC mutation has no...
متن کاملThe case of the youngest infant with hepatoblastoma with APC gene mutation
Hepatoblastoma is a rare early malignant liver neoplasm occurring in infants and children. Some cases of hepatoblastoma are associated with genetic conditions such as trisomies of chromosomes 18, Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP). The observed increase in the risk of hepatoblastoma in APC (adenomatous polyposis coli) gene mutation carriers is low, not exceedin...
متن کاملLessons from the hepatoblastoma-familial polyposis connection.
BACKGROUND Approximately one-third of hepatoblastoma (HB) patients have associated congenital abnormalities, but familial recurrence is rare, except in association with familial adenomatous polyposis (FAP). This correlation may be missed if not actively sought, with implications for long-term outcome and management. METHODS We retrospectively investigated 3 families with an HB-familial polypo...
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Familial adenomatous polyposis is characterized by over 100 colorectal adenomas in the colorectum. The disease equally affects both sexes, with an incidence estimated at 1.14025-1.8300. The disease is premature in people with familial adenomatous polyposis. Patients suffering from familial adenomatous polyposis have a range of extra-intestinal diseases such as papillae, gastric, small intestine...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 58 12 شماره
صفحات -
تاریخ انتشار 1983