Table 3.

Clinicopathologic characteristics of RTPS spectrum tumors

MRT (intra-/extracranial)SCCOHT (ovarian RT)
Median age of onset20 months (birth through adulthood, with most cases in infancy/early childhood)24 years (14 months–56 years)
5-year survival10%–30%∼33%
Cell typeRhabdoid cells prominent, small cell component usually present, rhabdoid cells may be difficult to identify50% small cell, 50% large cell (resembling rhabdoid cells)
Germline mutations (%)35%43%
Genes mutated/protein expression lostSMARCB1 (>98%)SMARCA4 (>98%)
SMARCA4 (<2%)SMARCB1 (<2%)