Table 4.

Summary of the main alterations in mesothelioma and relative targeted agents trial.

Gene	Type of Molecular Alteration	Alteration Prevalence (%)	Drug Tested, Primary Endpoint, Phase Trial
CDKN2A	Deletions, allelic losses, somatic inactivations	Pleural: 45–49% [13,14,15,16,17,18,20,21] Peritoneal: <10–26% [21,23,24]	–Abemaciclib, DCR, 12-week DCR (54%), positive trial [30]
BAP1	Somatic-inactivating mutations	Pleural: 23–57% [13,14,15,16,17,18,20,21] Peritoneal: 48–60% [21,23,24]	PARP –Olaparib, phase II ORR (4%), negative trial [31] –Rucaparib, phase II, DCR (54%), positive trial [32] –Tazemetostat, phase II, DCR (54%), positive trial [33]
NF2	Somatic-inactivating mutations, deletions, allelic losses	Pleural: 23–30% [13,14,15,16,17,18,20,21] Peritoneal: 27% [21,23,24]	PI3K/AKT/mTOR –Everolimus, phase II, 6 months PFS (29%), negative trial [34] –Samotolisib, Phase I/II, ORR (2%), negative trial [35] –FAK –GSK2256098, Phase I, negative [36] –GSK2256098 plus trametinib, Phase I, negative [37] –defactinib, phase II, no PFS improvement over placebo, negative [38]