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Preclinical evidence for the use of sunitinib malate in the treatment of plexiform neurofibromas.

Ferguson MJ1, Rhodes SD1,2, Jiang L1,2, Li X1,2, Yuan J1,2, Yang X1,2, Zhang S3, Vakili ST3, Territo P4, Hutchins G4, Yang FC1,2,5, Ingram DA1,2, Clapp DW1,2,6, Chen S1,2.
Pediatric blood & cancer.Pediatr Blood Cancer.2015 Sep 16. doi: 10.1002/pbc.25763. [Epub ahead of print]
PURPOSE: Plexiform neurofibromas (pNF) are pathognomonic nerve and soft tissue tumors of neurofibromatosis type I (NF1), which are highly resistant to conventional chemotherapy and associated with significant morbidity/mortality. Disruption of aberrant SCF/c-Kit signaling emanating from the pNF micr
PMID 26375012

Whole Exome Sequencing Reveals the Order of Genetic Changes during Malignant Transformation and Metastasis in a Single Patient with NF1-plexiform Neurofibroma.

Hirbe AC1, Dahiya S2, Miller CA3, Li T3, Fulton RS3, Zhang X2, McDonald S2, DeSchryver K2, Duncavage EJ2, Walrath J4, Reilly KM4, Abel HJ5, Pekmezci M6, Perry A7, Ley TJ3, Gutmann DH8.
Clinical cancer research : an official journal of the American Association for Cancer Research.Clin Cancer Res.2015 Sep 15;21(18):4201-11. doi: 10.1158/1078-0432.CCR-14-3049. Epub 2015 Apr 29.
PURPOSE: Malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in individuals with neurofibromatosis type 1 (NF1), where they likely arise from benign plexiform neurofibroma precursors. While previous studies have used a variety of discovery approaches to discover genes assoc
PMID 25925892

Vessel and Mast Cell Densities in Sporadic and Syndrome-associated Peripheral Nerve Sheath Tumors.

Friedrich RE1, Naber U2, Glatzel M2, Hagel C2.
Anticancer research.Anticancer Res.2015 Sep;35(9):4713-22.
BACKGROUND/AIM: Peripheral nerve sheath (PNS) tumors constitute a heterogeneous group of solid tumors. Neurofibroma and schwannoma are the most frequently diagnosed entities. Both tumor types occur sporadically and are associated with syndromes. Current strategies to fight PNS progression by means o
PMID 26254361