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.1Unlike the latter, which have a strong association with neurofibromatosis (NF), PS seem to affect exclusively the skin as solitary or multiple lesions.2,3Nonetheless, there seems to be a small but definite association between PS and NF,2and, 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
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Franco Rongioletti (1989) studied this question.
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