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A common phenomenon in cancer syndromes is for an individual to have multipleprimary cancers at different sites during his/her lifetime. Patients withLi-Fraumeni syndrome (LFS), a rare pediatric cancer syndrome mainly caused bygermline TP53 mutations, are known to have a higher probability of developing asecond primary cancer than those with other cancer syndromes. In this context,it is desirable to model the development of multiple primary cancers to enablebetter clinical management of LFS. Here, we propose a Bayesian recurrent eventmodel based on a non-homogeneous Poisson process in order to obtain penetranceestimates for multiple primary cancers related to LFS. We employed afamily-wise likelihood that facilitates using genetic information inheritedthrough the family pedigree and properly adjusted for the ascertainment biasthat was inevitable in studies of rare diseases by using an inverse probabilityweighting scheme. We applied the proposed method to data on LFS, using a familycohort collected through pediatric sarcoma patients at MD Anderson CancerCenter from 1944 to 1982. Both internal and external validation studies showedthat the proposed model provides reliable penetrance estimates for multipleprimary cancers in LFS, which, to the best of our knowledge, have not beenreported in the LFS literature.

Bayesian estimation of a semiparametric recurrent event model with  applications to the penetrance estimation of multiple primary cancers in  Li-Fraumeni Syndrome