Your browser doesn't support javascript.
loading
A Case of Familial Adenomatous Polyposis Combined with Hepatocellular Carcinoma
Article en Ko | WPRIM | ID: wpr-81452
Biblioteca responsable: WPRO
ABSTRACT
Familial adenomatous polyposis (FAP) is an autosomal dominant disease characterized by formation of multiple colorectal adenomas with nearly 100 percent potential for malignant transformation. FAP is a rare condition with an incidence of 1 in 10,000 live births. Germline mutations in the adenomatous polyposis coli gene (APC) located on chromosome 5q21 have been founded in many patients with FAP. Patients with FAP can have extracolonic manifestations of their disease. These include tumors of the upper gastrointestinal tract (hamartomatous polyps, adenomas, carcinomas), small intestine adenomas or cacinoma, bile duct adenomas, papillary thyroid carcinoma, osteomas of the mandible, skull, and long bones, a variety of soft tissue lesions, including fibromas, lipomas, and desmoid tumors, congenital hypertrophy of the retinal pigment epithelium (CHRPE) and hepatoblastoma. Hepatocellular carcinoma combined with FAP is a very rare condition. Just 8 cases of Hepatocellular carcinoma with a history of FAP have been reported in the literature. We now present a report of a case of Hepatocellular carcinoma with FAP (Gardner's syndrome) in a 19 year-old girl.
Asunto(s)
Palabras clave
Texto completo: 1 Índice: WPRIM Asunto principal: Osteoma / Pólipos / Cráneo / Neoplasias de la Tiroides / Adenoma / Incidencia / Hepatoblastoma / Mutación de Línea Germinal / Fibromatosis Agresiva / Adenoma de los Conductos Biliares Tipo de estudio: Incidence_studies / Prognostic_studies Límite: Female / Humans Idioma: Ko Revista: Journal of the Korean Society of Coloproctology Año: 2003 Tipo del documento: Article
Texto completo: 1 Índice: WPRIM Asunto principal: Osteoma / Pólipos / Cráneo / Neoplasias de la Tiroides / Adenoma / Incidencia / Hepatoblastoma / Mutación de Línea Germinal / Fibromatosis Agresiva / Adenoma de los Conductos Biliares Tipo de estudio: Incidence_studies / Prognostic_studies Límite: Female / Humans Idioma: Ko Revista: Journal of the Korean Society of Coloproctology Año: 2003 Tipo del documento: Article