Neurocutaneous syndromes

Abstract

The neurocutaneous syndromes are comprised of a heterogeneous group of disorders which have in common variable involvement of the neurologic, dermatologic, and ophthalmologic systems. This group of disorders was previously known as the pha-komatoses, due to their many dermatologic manifestations. Among these disorders are the neurofibromatoses (NFs), tuberous sclerosis complex (TSC), von Hippel-Lindau (VHL) disease, and Sturge-We-ber (SWS) syndrome. Less common disorders occasionally included are Osier-Weber-Rendu syndrome, Wyburn-Mason disease, nevoid basal cell carcinoma (Gorlin) syndrome, Cowden's disease, and ataxia-telangiectasia. A feature common to the majority of these syndromes is the propensity to develop tumors, both benign and malignant. These tumors, however, are not necessarily unique to those with neurocutaneous syndromes. By studying these disorders, significant insight has been gained into the etiology and behavior of various tumor types. This chapter will focus on the neurosurgical and neuro-oncological aspects of the most common neurocutaneous disorders. © Springer-Verlag Berlin Heidelberg 2006.