Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline TP53 mutations

• Main Authors: Choong, S.S., Latiff, Z.A., Mohamed, M., Lim, L.L.W., Chen, K.S., Vengidasan, L., Razali, H., Abdul Rahman, E.J., Ariffin, H.
• Format: Article
• Language: English
• Published: Wiley 2012
• Subjects: R Medicine (General)
• Online Access: http://eprints.um.edu.my/10499/1/00000727_80283.pdf; http://eprints.um.edu.my/10499/

Li-Fraumeni syndrome (LFS) is a highly penetrant, autosomal dominant disorder where affected individuals carry a 50% risk of developing cancer before 30 years of age. It is most commonly associated with mutations in the tumour suppressor gene, TP53. Adrenocortical carcinoma (ACC) is a very rare paed...