No abstract

No abstract

The prognosis for patients with INI1-deficient pediatric cancers is poor and therapeutic options, particularly in the relapsed or refractory setting, are limited. We sought to characterize the association between SMARCB1 genetic variants identified by next-generation sequencing and INI1 protein expression and to evaluate PD-L1 and CD8 expression in INI1-deficient tumors. Available evidence suggests a low rate of PD-L1 expression and response to immune checkpoint blockade in pediatric cancers. INI1-negative pediatric cancers may represent an important exception to this despite remarkably low tumor mutational burden. Our findings provide evidence supporting a novel strategy for treating INI1-deficient cancers. This study contributes to emerging data that immune checkpoint blockade either as monotherapy or in combination with other agents may be an effective treatment approach for these aggressive cancers. Research.

No abstract