Diverse outcomes in SMARCB1-deficient rhabdoid tumors: A single institute experience

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.