Targeting EZH2 in SMARCB1-deficient sarcomas: Advances and opportunities to potentiate the efficacy of EZH2 inhibitors

Lanzi, Cinzia; Arrighetti, Noemi; Pasquali, Sandro; Cassinelli, Giuliana

doi:10.1016/j.bcp.2023.115727

Cited by 5 publications

(2 citation statements)

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“…SMARCB1/A4‐loss leads to dependency on PRC2 and its enzymatic subunit EZH2, 69 so efforts have been made to inhibit EZH2 in SMARC‐deficient tumors. Twenty patients with tumors with loss of SMARCB1/A4 or mutations in EZH2 were treated with the EZH2‐inhibitor tazemetostat.…”

Section: Targeted Therapiesmentioning

confidence: 99%

“…[66][67][68] In a phase II trial, eight of 30 patients with recurrent ATRT achieved stable disease for greater than 6 months, though alisertib was not found to have a statistically significant impact in EFS or OS. 68 SMARCB1/A4-loss leads to dependency on PRC2 and its enzymatic subunit EZH2, 69 so efforts have been made to inhibit EZH2 in SMARC-deficient tumors. Twenty patients with tumors with loss of SMARCB1/A4 or mutations in EZH2 were treated with the EZH2inhibitor tazemetostat.…”

Section: Targeted Therapiesmentioning

confidence: 99%

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Rhabdoid tumor predisposition syndrome: A historical review of treatments and outcomes for associated pediatric malignancies

Andres,

Huang,

Shatara

et al. 2024

Pediatric Blood & Cancer

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Rhabdoid tumor predisposition syndrome (RTPS) is a rare disorder associated with malignant rhabdoid tumor of the kidney (RTK), atypical teratoid rhabdoid tumor (ATRT), and/or other extracranial, extrarenal rhabdoid tumors (EERT), and these pediatric malignancies are difficult to treat. Presently, most of the information regarding clinical manifestations, treatment, and outcomes of rhabdoid tumors comes from large data registries and case series. Our current understanding of treatments for patients with rhabdoid tumors may inform how we approach patients with RTPS. In this manuscript, we review the genetic and clinical features of RTPS and, using known registry data and clinical reports, review associated tumor types ATRT, RTK, and EERT, closing with potential new approaches to treatment. We propose collaborative international efforts to study the use of SMARC (SWI/SNF‐related, matrix‐associated, actin‐dependent regulator of chromatin)‐targeting agents, high‐dose consolidative therapy, and age‐based irradiation of disease sites in RTPS.

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Section: Targeted Therapiesmentioning

confidence: 99%