Understanding how plexiform neurofibromas form is an important step towards identifying new treatments for these common tumors in children and adults with neurofibromatosis type 1 (NF1). Plexiform neurofibromas are benign tumors that arise from cells surrounding the developing nerve. A recent study spearheaded by Dr. Lu Le at the University of Texas- Southwestern revealed that these tumors likely arise from a small population of immature cells that eventually give rise to Schwann cells. Loss of Nf1 gene function in these Schwann cell precursors is sufficient for a plexiform neurofibroma to develop in a mouse.
Leveraging this exciting finding, they developed methods to screen promising drugs for the treatment of plexiform neurofibromas. This report was published in the prestigious journal Cancer Cell.
Accompanying this landmark paper was an invited editorial by Dr. David Gutmann, Director of the Washington University NF Center. The importance of Dr. Le’s findings and their relevance to future treatments for NF1-associated plexiform neurofibromas was discussed.
Congratulations to Dr. Le and his colleagues.
Chen Z, Liu C, Patel AJ, Liao CP, Wang Y, Le LQ. Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma. Cancer Cell. 26(5):695-706, 2014.