title: The role of the immune system in neurofibromatosis type 1-associated nervous system tumors.
abstract: With the recent development of new anticancer therapies targeting the immune system, it is important to understand which immune cell types and cytokines play critical roles in suppressing or promoting tumorigenesis. The role of mast cells in promoting neurofibroma growth in neurofibromatosis type 1 (NF1) patients was hypothesized decades ago. More recent experiments in mouse models have demonstrated the causal role of mast cells in neurofibroma development and of microglia in optic pathway glioma development. We review here what is known about the role of NF1 mutation in immune cell function and the role of immune cells in promoting tumorigenesis in NF1. We also review the therapies targeting immune cell pathways and their promise in NF1 tumors.
authors.affiliation: Rare Tumors Initiative, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 37 Convent Dr, Bethesda, MD 20814, USA.
authors.forename: Souvik
authors.initials: S
authors.lastname: Karmakar
authors.affiliation: Rare Tumors Initiative, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 37 Convent Dr, Bethesda, MD 20814, USA.
authors.forename: Karlyne M
authors.initials: KM
authors.lastname: Reilly
mesh_terms: D000818:Animals; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007107:Immune System / Q000473:pathology / Q000502:physiology*; D009154:Mutation / Q000235:genetics*; D009423:Nervous System Neoplasms / Q000235:genetics / Q000276:immunology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000276:immunology*; D025542:Neurofibromin 1 / Q000235:genetics*; D015398:Signal Transduction / Q000235:genetics
chemical_list: D025542:Neurofibromin 1
keywords: B cells; T cells; cytokines; immunotherapy; malignant peripheral nerve sheath tumor; mast cells; microglia; neurofibroma; neurofibromatosis type 1; optic pathway glioma