Our seminal studies detailing EZH2 dysregulation in RB, orbital and ocular adnexal lymphoma, and cutaneous basal cell carcinoma have uncovered “the tip of the iceberg” of the potential of using epigenetics to better understand how ocular, ocular adnexal, and orbital tumors form, and to develop novel therapeutics.6, –8 Taken together, this study indicates EZH2 is a biomarker for IM, and highlights the possibility that EZH2 could be exploited as a therapeutic target for this pediatric tumor, a cancer that lacks biologically targeted treatments. Here, EZH2 is linked to ocular adnexal lymphoma.