Diverse outcomes in SMARCB1-deficient rhabdoid tumors: A single
Rhabdoid tumors (RTs) are a rare and aggressive pediatric cancer that
commonly show alterations in the tumor suppressor gene SMARCB1. However,
RT prognosis is still poor, with no standard treatment, predictive
biomarkers for its aggressiveness, or chemo- and radio-sensitivity.
Herein, four cases of extra-cranial RTs are described, two of which were
in long-term survivors. These two surviving cases were positive for p16,
whereas the other two were p16-negative. These findings suggest that p16
expression may represent a potential positive prognostic biomarker in
RTs; nevertheless, further studies are required.