Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state.

doi:10.1007/s00401-021-02341-z

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Article

Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state.

Acta neuropathologica - 1 Sept 2021

Kochat Veena, Raman Ayush T, Landers Sharon M, Tang Ming, Schulz Jonathan, Terranova Christopher, Landry Jace P, Bhalla Angela D, Beird Hannah C, Wu Chia-Chin, Jiang Yingda, Mao Xizeng, Lazcano Rossana, Gite Swati, Ingram Davis R, Yi Min, Zhang Jianhua, Keung Emily Z, Scally Christopher P, Roland Christina L, Hunt Kelly K, Feig Barry W, Futreal P Andrew, Hwu Patrick, Wang Wei-Lien, Lazar Alexander J, Slopis John M, Wilson-Robles Heather, Wiener Dominique J, McCutcheon Ian E, Wustefeld-Janssens Brandan, Rai Kunal, Torres Keila E

Abstract excerpt

Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that frequently harbor genetic alterations in polycomb repressor complex 2 (PRC2) components-SUZ12 and EED. Here, we show that PRC2 loss confers a dedifferentiated early neural-crest phenotype which is exclusive to PRC2-mutant MPNSTs and not a feature of neurofibromas. Neural crest phenotype in PRC2 mutant MPNSTs was validated via...

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PMID: 34283254
DOI: 10.1007/s00401-021-02341-z

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