How should adult patients with neurofibromatosis 1 be managed?

Among familial tumor syndromes, neurofibromatosis 1 (NF-1) is the most prevalent, affecting about 1 in 3000 births. The disorder leads to a microdeletion in the gene coding for neurofibromin, which is a negative regulator of the Ras oncogene signal transduction pathway. The gene is situated on 17q11.2, and mutation is transmitted as an autosomal dominant trait. However, half of affected individuals are spontaneous mutations.

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