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Journal Article
Review
Epigenetic mechanisms drive the progression of neurofibromas to malignant peripheral nerve sheath tumors.
THINKING OUTSIDE THE BOX: The polycomb repressive complex 2 (PRC2) is a histone methyltransferase complex known to repress gene expression. There is a large body of experimental evidence that supports its role in promoting tumorigenicity by suppressing tumor suppressor genes. Here, we discuss the surprising findings that, in neurofibromas, it may have a completely different role as a tumor suppressor; mutations of PRC2 lead to conversion of benign neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) by de-repressing and thereby activating genes driving cell growth and development. These findings have potentially powerful clinical applications in both diagnosing and treating MPNSTs.
HYPOTHESIS: PRC2 loss drives malignant transformation of neurofibromas.
HYPOTHESIS: PRC2 loss drives malignant transformation of neurofibromas.
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