title: Frequent occurrence of gastric cancer in Asian kindreds with Li-Fraumeni syndrome.
abstract: Type of cancer and age of onset in individuals with inherited aberrations in the tumour suppressor gene TP53 are variable, possibly influenced by genetic modifiers and different environmental exposure. Since 2009, the modified Chompret criteria (MCC) have been used to identify individuals for TP53 mutation screening. Using the TP53 mutation database maintained by the International Agency for Research on Cancer (IARC), we investigated if the MCC, mainly developed for a Caucasian population, was also applicable in Asia. We identified several differences in Asian families compared with similar Caucasian cohorts, suggesting that identification and management of Li-Fraumeni syndrome in Asia do not completely mirror that of North America and Western Europe. Early gastric cancer (<40 years) may be considered a new addition to the MCC especially for Asian families.
authors.affiliation: University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia.|Paediatric Haematology-Oncology Unit, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.
authors.forename: H
authors.initials: H
authors.lastname: Ariffin
authors.affiliation: Paediatric Haematology-Oncology Unit, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.
authors.forename: A S L
authors.initials: AS
authors.lastname: Chan
authors.affiliation: University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: L
authors.initials: L
authors.lastname: Oh
authors.affiliation: University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: S
authors.initials: S
authors.lastname: Abd-Ghafar
authors.affiliation: Department of Paediatrics, Sarawak General Hospital, Sarawak, Malaysia.
authors.forename: G B
authors.initials: GB
authors.lastname: Ong
authors.affiliation: Paediatrics Institute, Kuala Lumpur, Malaysia.
authors.forename: M
authors.initials: M
authors.lastname: Mohamed
authors.affiliation: Department of Paediatrics, Sultan Ismail Hospital, Johor Bahru, Malaysia.
authors.forename: H
authors.initials: H
authors.lastname: Razali
authors.affiliation: Paediatrics Institute, Kuala Lumpur, Malaysia.
authors.forename: E
authors.initials: E
authors.lastname: Juraida
authors.affiliation: Cancer Research Initiatives Foundation, Subang Jaya, Malaysia.
authors.forename: S H
authors.initials: SH
authors.lastname: Teo
authors.affiliation: University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: M
authors.initials: M
authors.lastname: Karsa
authors.affiliation: University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: J
authors.initials: J
authors.lastname: Shamsani
authors.affiliation: Institut Albert Bonniot - INSERM 823, University Grenoble-Alpes, Grenoble, France.
authors.forename: P
authors.initials: P
authors.lastname: Hainaut
mesh_terms: D044466:Asian People / Q000235:genetics; D006801:Humans; D016864:Li-Fraumeni Syndrome / Q000150:complications*; D009154:Mutation; D013274:Stomach Neoplasms / Q000453:epidemiology* / Q000235:genetics; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
chemical_list: C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53
keywords: Li-Fraumeni syndrome; TP53; cancer risk; gastric cancer