TABLE 1.

List of Syndromes, Mutations, Pathways, and Sporadic Relationships

Syndrome	Mutated gene	Involved pathway	Relationship to sporadic tumors
NF2	NF2 tumor suppressor	EGFR, YAP/TAZ, MAPK, cytoskeletal architecture	Mutated in 40%-60%
Werner	WRN	DNA processing, maintenance and repair	Methylated with decrease in expression
BAP1-TPDS	BAP1	Chromatin Remodeling, DNA repair	Found in high-grade rhabdoid meningiomas
SMARCE1	SMARCE1	Nucleosome remodeling, apoptosis	Found in Clear cell meningiomas
SMARCB1	SMARCB1	Nucleosome remodeling, apoptosis	Mutated in 3%
Gorlin	PTCH1	SHH – PTCH1/SMO/SUFU/GLI-1,2,3	SMO mutated in 5%
Cowden	PTEN	PI3K – RTK/AKT/PI3K/mTOR/PTEN	AKT mutated in 14%; PIK3CA mutated in 7%