{"id":"703393ac-0a1b-47eb-8bee-ddf026b275b1","slug":"epigenetic-mechanisms-drive-the-progression-of-neurofibromas-to-malignant-peripheral-nerve-sheath-tumors","title":"Epigenetic mechanisms drive the progression of neurofibromas to malignant peripheral nerve sheath tumors","authors":["Krish Suresh","Tamara Kliot","Andrea Piunti","Michel Kliot"],"abstract":"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.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/703393ac-0a1b-47eb-8bee-ddf026b275b1/featured/hero-1782420874099.png","publishDate":"2016-11-14T00:00:00.000Z","doi":"10.4103/2152-7806.194058","categories":["General Neurosurgery","Review Article"],"fullTextUrl":"http://surgicalneurologyint.com/wp-content/uploads/2016/11/8133/SNI-7-797.pdf"}