. .. Résumé,

. Patients and . .. Méthodes,

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M. Zöller, B. Rembeck, H. O. Akesson, and L. Angervall, Life expectancy, mortality and prognostic factors in neurofibromatosis type 1. A twelve-year follow-up of an epidemiological study in Göteborg, Acta Derm Venereol, vol.75, pp.136-176, 1995.

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T. Tucker, P. Wolkenstein, J. Revuz, J. Zeller, and J. M. Friedman, Association between benign and malignant peripheral nerve sheath tumors in NF1, Neurology, vol.65, pp.205-216, 2005.

E. Sbidian, S. Bastuji-garin, L. Valeyrie-allanore, S. Ferkal, and J. P. Lefaucheur,

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V. Mautner, F. A. Asuagbor, E. Dombi, C. Fünsterer, L. Kluwe et al., Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1, Neuro Oncol, vol.10, pp.593-601, 2008.

D. Evans, H. Salvador, V. Y. Chang, A. Erez, S. D. Voss et al., Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 1, Clin Cancer Res, vol.23, pp.46-53, 2017.

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, Elevated Risk for MPNST in NF1 Microdeletion Patients, Am J Hum Genet, vol.72, pp.1288-92, 2003.

V. Mautner, L. Kluwe, R. E. Friedrich, A. C. Roehl, S. Bammert et al.,

, Mb type-1 NF1 deletions, J Med Genet, vol.47, pp.623-653, 2010.

D. Evans, M. E. Baser, J. Mcgaughran, S. Sharif, E. Howard et al.,

D. H. Gutmann, D. L. Wood, and F. S. Collins, Identification of the neurofibromatosis type 1 gene product, Proc Natl Acad Sci, vol.88, issue.21, pp.9658-62, 1991.

S. A. Rasmussen and J. M. Friedman, NF1 gene and neurofibromatosis 1, Am J Epidemiol. 1 janv, vol.151, issue.1, pp.33-40, 2000.

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S. M. Huson, D. A. Compston, P. Clark, and P. S. Harper, A genetic study of von Recklinghausen neurofibromatosis in south east Wales. I. Prevalence, fitness, mutation rate, and effect of parental transmission on severity, J Med Genet, vol.26, issue.11, pp.704-715, 1989.

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T. N. Basu, D. H. Gutmann, J. A. Fletcher, T. W. Glover, F. S. Collins et al., Aberrant regulation of ras proteins in malignant tumour cells from type 1 neurofibromatosis patients, Nature. 23 avr, vol.356, issue.6371, pp.713-718, 1992.

B. Dasgupta, Y. Yi, D. Y. Chen, J. D. Weber, and D. H. Gutmann, Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors, Cancer Res. 1 avr, vol.65, issue.7, pp.2755-60, 2005.

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