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Review
. 2017 Jan;6(1):45-60.
doi: 10.2217/cns-2016-0024. Epub 2016 Dec 21.

The role of the immune system in neurofibromatosis type 1-associated nervous system tumors

Souvik Karmakar  1 , Karlyne M Reilly  1
Affiliations
Review

The role of the immune system in neurofibromatosis type 1-associated nervous system tumors

Souvik Karmakar et al. CNS Oncol. 2017 Jan.

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.

Keywords: B cells; T cells; cytokines; immunotherapy; malignant peripheral nerve sheath tumor; mast cells; microglia; neurofibroma; neurofibromatosis type 1; optic pathway glioma.

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Conflict of interest statement

Financial & competing interests disclosure

The authors are supported by the Intramural Research Program of the NIH, National Cancer Institute. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Figures

<b>Figure 1.</b>
Figure 1.. Development of nervous system tumors in NF1 patients.
GBM: Glioblastoma multiforme; MPNST: Malignant peripheral nerve sheath tumors; OPG: Optic pathway gliomas; PNF: Plexiform neurofibromas.
See this image and copyright information in PMC

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