Multiple Cutaneous Plexiform Schwannomas With Tumors of the Central Nervous System

Abstract

To the Editor.— Schwannomas (neurilemomas) are rare benign nerve tumors, usually presenting as solitary encapsulated subcutaneous nodules adherent to a peripheral nerve. It is most uncommon for schwannomas to possess multinodular intertwining characteristics (ie, a plexiform pattern). Plexiform schwannomas (PS) have often been confused with plexiform neurofibromas. 1 Unlike the latter, which have a strong association with neurofibromatosis (NF), PS seem to affect exclusively the skin as solitary or multiple lesions. 2,3 Nonetheless, there seems to be a small but definite association between PS and NF, 2 and, recently, multiple cutaneous plexiform schwannomas (MCPS) have been reported in Japan as a marker of a novel neurocutaneous syndrome, neurilemomatosis (NL). 4 We report a case of MCPS with multiple tumors of the central nervous system in the absence of any cutaneous and genetic marker for NF. Report of a Case.— An 18-year-old normally intelligent woman was born to a family without