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Rhabdoid tumors (RTs) are a rare and aggressive pediatric cancer that commonly presents with alterations in the tumor suppressor gene  SMARCB1 . However, RT prognosis is still poor, with no standard treatment available. Moreover, no predictive biomarkers have been identified for determining its aggressiveness or chemo- and radio-sensitivities. Herein, four cases of extra-cranial RTs (ERTs) are described, two of whom are long-term survivors. These two surviving patients were positive for p16, whereas the other two were p16-negative. Our findings suggest that biologically distinct types of ERTs exist and that p16 expression may be a potential positive prognostic biomarker of ERTs. Nevertheless, further studies are required to confirm our findings.

Diverse outcomes in extra-cranial rhabdoid tumors: A single institute experience