Epigenetic Therapy in a Rare Ovarian Cancer — A Double-Edged Sword

This correspondence details the case of a 32-year-old woman with small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), a rare and aggressive malignancy marked by biallelic SMARCA4 loss. After multiple failed therapies, the patient experienced a durable near-complete response to tazemetostat, an EZH2 inhibitor. However, after four years of treatment, she developed secondary T cell acute lymphoblastic leukemia (T-ALL). The article explores the therapeutic promise and oncogenic risks of long term epigenetic modulation via EZH2 inhibition and highlights the need for further research into the safety and mechanisms of synthetic lethality approaches in cancer.

This correspondence details the case of a 32-year-old woman with small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), a rare and aggressive malignancy marked by biallelic SMARCA4 loss. After multiple failed therapies, the patient experienced a durable near-complete response to tazemetostat, an EZH2 inhibitor. However, after four years of treatment, she developed secondary T cell acute lymphoblastic leukemia (T-ALL). The article explores the therapeutic promise and oncogenic risks of long term epigenetic modulation via EZH2 inhibition and highlights the need for further research into the safety and mechanisms of synthetic lethality approaches in cancer.