The clinical, genetic, and immune landscape of meningioma in patients with NF2-schwannomatosis
Gregory, G. E. Islim, A. I. Hannan, C. J. Jones, A. P. Hammerbeck-Ward, C. Rutherford, S. A. Freeman, S. R. Lloyd, S. Kalamarides, M. Smith, M. J.
Gregory, G. E.
Islim, A. I.
Hannan, C. J.
Jones, A. P.
Hammerbeck-Ward, C.
Rutherford, S. A.
Freeman, S. R.
Lloyd, S.
Kalamarides, M.
Smith, M. J.
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Abstract
NF2-schwannomatosis is the most common genetic predisposition syndrome associated with meningioma. Meningioma in NF2-schwannomatosis is a major source of morbidity and mortality. This is due to accumulative tumor burden in patients with synchronous schwannomas and ependymomas, sometimes including complex collision tumors. Balancing the impact of multiple interventions against the natural history of various index tumors, and the ongoing risk of de novo tumors over an individual's lifetime makes decision-making complex. The management of any given individual meningioma is often different from a comparable sporadic tumor. There is typically a greater emphasis on conservative management and tolerating growth until a risk boundary is reached, whereby symptomatic deterioration or higher risk from anticipated future treatment is threatened. Management by high-volume multidisciplinary teams improves quality of life and life expectancy. Surgery remains the mainstay treatment for symptomatic and rapidly enlarging meningioma. Radiotherapy has an important role but carries a higher risk compared to its use in sporadic disease. Whilst bevacizumab is effective in NF2-associated schwannoma and cystic ependymoma, it has no value in the management of meningioma. In this review, we describe the natural history of the disease, underlying genetic, molecular, and immune microenvironment changes, current management paradigms, and potential therapeutic targets.
Authors
Gregory, G. E.
Islim, A. I.
Hannan, C. J.
Jones, A. P.
Hammerbeck-Ward, C.
Rutherford, S. A.
Freeman, S. R.
Lloyd, S.
Kalamarides, M.
Smith, M. J.
Couper, K.
McBain, Catherine A
Jenkinson, M. D.
Brough, D.
King, A. T.
Evans, D. G.
Pathmanaban, O. N.
Islim, A. I.
Hannan, C. J.
Jones, A. P.
Hammerbeck-Ward, C.
Rutherford, S. A.
Freeman, S. R.
Lloyd, S.
Kalamarides, M.
Smith, M. J.
Couper, K.
McBain, Catherine A
Jenkinson, M. D.
Brough, D.
King, A. T.
Evans, D. G.
Pathmanaban, O. N.
Affiliation
Geoffrey Jefferson Brain Research Centre, Manchester Academic Health Science Centre, Northern Care Alliance NHS Group, University of Manchester, Manchester, UK
Description
Date
2023
Publisher
Collections
Keywords
Type
Article
Citation
Gregory GE, Islim AI, Hannan CJ, Jones AP, Hammerbeck-Ward C, Rutherford SA, et al. The clinical, genetic, and immune landscape of meningioma in patients with NF2-schwannomatosis. Neuro-oncology advances. 2023 May;5(Suppl 1):i94-i104. PubMed PMID: 37287576. Pubmed Central PMCID: PMC10243851. Epub 2023/06/08. eng.