Table 1.

Molecular Subgroups of Atypical Teratoid/Rhabdoid Tumors (AT/RT) and Their Clinical Features

	AT/RT-SHH/Group 1	AT/RT-TYR/Group 2A	AT/RT-MYC/Group 2B
Location	Supratentorial > infratentorial	Infratentorial >> supratentorial	Supratentorial >> infratentorial
Mutation type	Focal SMARCB1 mutations	Broad SMARCB1 deletions (ie, 22q loss).	Focal SMARCB1 deletions
DNA methylation status	Hypermethylated	Hypermethylated	Hypomethylated
Dysregulated molecular pathways	NOTCH, SHH, EZH2, CDK6	MITF, OTX2, CCND1, VEGFA	HOX, EZH2
		BMP, PDGFRB, MYC, ERBB2
Published representative cell lines	CHLA02, CHLA04, CHLA05	BT12, BT16, SH, CHLA06, CHLA266

Abbreviations: BMP, bone morphogenic protein; CCND1, cyclin D1; MITF, micropthalmia-associated transcription factor; PDGFRB, platelet-derived growth factor receptor beta; SHH, Sonic Hedgehog; SMARCB1, SWItch/Sucrose Non-Fermentable–related, matrix-associated, actin-dependent regulator of chromatin, subfamily b, member 1; VEGFA, vascular endothelial growth factor A.