Co-existence of neurofibromatosis type 2 and multiple sclerosis: A case report

Naghshineh, Hoda; Shahin, Daryoush; Sahraian, Mohammad Ali; Minagar, Alireza

doi:10.1016/j.msard.2013.08.008

Cited by 3 publications

(1 citation statement)

References 6 publications

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“…Bilateral acoustic schwannomas are diagnostic for neurofibromatosis type 2 (NF-2) syndrome, a disorder caused by germline or somatic mutations of the NF2/merlin gene on chromosome 22q12. 7 However, Western blot and immunohistochemical studies demonstrated that sporadic (noninherited and nonbilateral) schwannomas also exert universal loss of merlin/NF2 likely via signals targeting NF2/protein to intracytoplasmic degradation. 8 NF2/merlin is an inhibitor of NF-κB, 9 one of the major transcription factors inducing synthesis of proinflammatory cytokines and chemokines.…”

Section: General Biological and Clinical Features Of Schwannomasmentioning

confidence: 99%

Immune Pathobiology of Schwannomas: A Concise Review

Elmacı¹,

Altinöz²,

Sarı

2017

J Neurol Surg A Cent Eur Neurosurg

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Schwannomas are benign tumors treatable with neurosurgery or radiosurgery, yet a small subset may exhibit aggressive growth. Hence illuminating their immune features can help develop better treatments. A tumor-promoting inflammation exists in schwannomas. Transcription factor NF-κB triggers synthesis of inflammatory cytokines and chemokines. NF-κB is suppressed by NF2/merlin, yet it is mutated or repressed in schwannomas, and therefore MCP-1/CCL2, MIP-1α/CCL3, CXCL16, and CXCR6/Bonzo are likely expressed in these tumors. CD68+ and CD163+ macrophages may infiltrate schwannomas and promote their growth. Anti-inflammatory salicylates inhibit schwannomas in cell culture and clinically. Schwannomas that cannot be completely removed by neurosurgery or controlled by radiosurgery may be suitable targets of pharmacologic interventions focusing on immune mechanisms.

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Section: General Biological and Clinical Features Of Schwannomasmentioning

confidence: 99%