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Article

Malignant peripheral nerve sheath tumor in children: A clinicopathologic and molecular study with parallels to the adult counterpart.

Genes, chromosomes & cancer - 1 Mar 2023

Agaram Narasimhan P, Wexler Leonard H, Chi Ping, Antonescu Cristina R

Abstract excerpt

Malignant peripheral nerve sheath tumors (MPNST) are aggressive neoplasms, arising either sporadically, in the setting of neurofibromatosis type I (NF1) or post radiation. Most MPNST occur in adults and their pathogenesis is driven by the loss of function mutations in the PRC2 complex, regardless of their clinical presentation. In contrast, pediatric MPNST are rare and their pathogenesis has not been elucidated....

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Identifiers

PMID: 36414547
DOI: 10.1002/gcc.23106
PMCID: PMC9825640

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Malignant peripheral nerve sheath tumor in children: A clinicopathologic and molecular study with parallels to the adult counterpart.PMID 36414547Read ↗

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A Sequencing Overview of Malignant Peripheral Nerve Sheath Tumors: Findings and Implications for TreatmentSemantic proximity Read ↗

Identification of Malignant Peripheral Nerve Sheath Tumor subtypes with distinct genomic identitiesSemantic proximity Read ↗

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A detailed landscape of genomic alterations in malignant peripheral nerve sheath tumor cell lines challenges the current MPNST diagnosisSemantic proximity Read ↗

Molecular targets for NF1-associated malignant peripheral nerve sheath tumorSemantic proximity Read ↗

Genomic patterns of malignant peripheral nerve sheath tumour (MPNST) evolution correlate with clinical outcome and are detectable in cell-free DNASemantic proximity Read ↗

PRC2 Restricts Malignant Peripheral Nerve Sheath Tumorigenesis in a Genetically Engineered Mouse Model of MPNSTSemantic proximity Read ↗

Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNSTSemantic proximity Read ↗

Low mutation burden and frequent loss of CDKN2A/B and SMARCA2, but not PRC2, define premalignant neurofibromatosis type 1–associated atypical neurofibromasSemantic proximity Read ↗

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PRC2 is recurrently inactivated through EED or SUZ12 loss in malignant peripheral nerve sheath tumors.Semantic proximity Read ↗

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