Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line.

摘要: Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation tumor fragments or heterogeneous primary cultures, which rarely achieved significant growth. We report practical reproducible animal model plexiform-like by an immortal human NF1 tumor-derived Schwann cell line into scid mice. The S100 p75 positive sNF94.3 was shown possess normal karyotype apparent full-length neurofibromin Western blot. These cells were constitutional microdeletion elevated Ras-GTP activity, however, suggesting loss function. Localized intraneural injection produced consistent slow growing infiltrated disrupted host nerve. resembled plexiform with low rate proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, mast infiltration. histologic features developed particularly those as well. Intraneural enables precise initiation intraneural, provides highly study tumorigenesis. This facilitates testing potential therapeutic interventions, including angiogenesis inhibitors, relevant cellular environment.