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Diverse outcomes in extra-cranial rhabdoid tumors: A single institute experience
Yoshiki Katsumi1,2, Tomoko Iehara1, Yasumichi Kuwahara3
1Department of Peditrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Abstract:
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.
Insights
Rhabdoid tumors (RTs) are aggressive pediatric cancers lacking effective treatments. p16 expression may indicate a better prognosis in extra-cranial RTs, suggesting distinct tumor subtypes.
Area of Science:
- Pediatric Oncology
- Cancer Genetics
- Tumor Biology
Background:
- Rhabdoid tumors (RTs) are rare, aggressive pediatric cancers often linked to the SMARCB1 tumor suppressor gene.
- Current treatment options for RTs are limited, and prognosis remains poor.
- Predictive biomarkers for RT aggressiveness or treatment response are currently lacking.
Observation:
- This study describes four cases of extra-cranial RTs (ERTs).
- Two patients with ERTs achieved long-term survival and were positive for p16 expression.
- The other two patients, who were p16-negative, did not survive long-term.
Findings:
- p16 expression appears to be a potential positive prognostic biomarker in extra-cranial RTs.
- The presence or absence of p16 expression suggests biologically distinct subtypes of ERTs.
- These findings indicate that p16 status could help differentiate ERT behavior.
Implications:
- p16 expression may help identify ERTs with a better prognosis.
- Further research is needed to validate p16 as a reliable prognostic marker for ERTs.
- This could lead to more personalized treatment strategies for pediatric RT patients.
