Relative frequency and morphology of cancers in carriers of germline TP53 mutations

The spectrum and frequency of cancers associated with germline TP53 mutations are uncertain. To address this issue a cohort of individuals from 28 families with Li-Fraumeni syndrome, segregating germline TP53 mutations was established. Predicted cancers were estimated by applying age, morphology, si...

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Veröffentlicht in:	Oncogene 2001-08, Vol.20 (34), p.4621-4628
Hauptverfasser:	BIRCH, Jillian M, ALSTON, Robert D, MCNALLY, Richard J. Q, EVANS, D. Gareth R, KELSEY, Anna M, HARRIS, Martin, EDEN, Osborn B, VARLEY, Jennifer M
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Aged Biological and medical sciences Brain cancer Brain tumors Breast cancer Breast carcinoma Cancer research Cell physiology Cell transformation and carcinogenesis. Action of oncogenes and antioncogenes Cervix Child Child, Preschool Children & youth Cohort Studies Families & family life Family Health Female Fundamental and applied biological sciences. Psychology Genetic aspects Genetic Predisposition to Disease Germ-Line Mutation Heterozygote Humans Infant Leukemia Li-Fraumeni syndrome Li-Fraumeni Syndrome - epidemiology Li-Fraumeni Syndrome - genetics Li-Fraumeni Syndrome - pathology Lung carcinoma Male Medical research Medicine, Experimental Middle Aged Molecular and cellular biology Morphology Mutation Neuroblastoma Neuroendocrine tumors Osteosarcoma p53 Protein Pancreatic carcinoma Pathology Pediatrics Prostate Sarcoma Tumor proteins Tumor Suppressor Protein p53 - genetics Tumors
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Zusammenfassung:	The spectrum and frequency of cancers associated with germline TP53 mutations are uncertain. To address this issue a cohort of individuals from 28 families with Li-Fraumeni syndrome, segregating germline TP53 mutations was established. Predicted cancers were estimated by applying age, morphology, site and sex-specific UK cancer statistics to person-years at risk. Observed and predicted cancers were compared and two-sided P-values calculated. Cancer types occurring to excess and showing P-values
ISSN:	0950-9232 1476-5594
DOI:	10.1038/sj.onc.1204621