Familial cancer syndromes involving the peripheral nervous system

The neurofibromatoses are members of the group of neurocutaneous disorders which also include tuberous sclerosis, neurocutaneous melanosis, hypomelanosis of Ito, incontinentia pigmenti, Sturge-Weber syndrome and von Hippel-Lindau disease. Collectively, these conditions have also been referred to as "phakomatoses," a term is derived from the Greek phakos, meaning a lentil or birthmark. All but Sturge-Weber syndrome, which is sporadic in occurrence, have an autosomal dominant pattern of inheritance. Only neurofibromatosis type I and II as well as schwannomatosis show significant involvement of the peripheral nervous system.