Clinical and molecular predictors of mortality in neurofibromatosis 2: a UK national analysis of 1192 patients

Neurofibromatosis 2 is an inherited condition that predisposes to tumours of the nerve sheath (schwanomas) and brain lining (meningiomas). Most patients develop deafness and other handicaps that reduce life expectancy. In 1192 NF2 patients followed for an average of over 9 years, we confirmed that life expectancy was reduced when NF2 was diagnosed at a younger age, meningiomas were present, or certain particular kinds of genetic mutations caused the disease. We also showed that NF2 patients diagnosed in more recent decades had better survival than patients diagnosed earlier, reflecting improvements in diagnostic methods and treatment. (By Prof D Gareth Evans, )


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