Affiliations 

  • 1 University of Malaya Cancer Research Institute, University of Malaya, Kuala Lumpur, Malaysia
  • 2 Paediatric Haematology-Oncology Unit, University of Malaya Medical Centre, Kuala Lumpur, Malaysia
  • 3 Department of Paediatrics, Sarawak General Hospital, Sarawak, Malaysia
  • 4 Paediatrics Institute, Kuala Lumpur, Malaysia
  • 5 Department of Paediatrics, Sultan Ismail Hospital, Johor Bahru, Malaysia
  • 6 Cancer Research Initiatives Foundation, Subang Jaya, Malaysia
  • 7 Institut Albert Bonniot - INSERM 823, University Grenoble-Alpes, Grenoble, France
Clin Genet, 2015 Nov;88(5):450-5.
PMID: 25318593 DOI: 10.1111/cge.12525

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.