Abstract: In order to study nerve sheath myxoma,arecently described rare soft tisue tumor,the lesions of eighteen cases of this disease have beenexamined by ligh tmicroscopy and immunohis-tochemical methods.The lesion ocured usually inteenagers.It tended to ocur in the upper limbs and faces.Light microscopicaly,the lobulated tumor was composed of spindle and stelate tumor cells in the abundant myxoid matrix,which looked like Pacinian corpuscles.S-100 protein immunohis-tochemistry was done in ten cases and demonstrated strongly positive in nine patients,further supporting this disease is originated from nerve sheath.A few nomenclatures have benn used for the identical lesions.The name dermal nerve sheath myxoma and neurothekeom a are recommended.

Key words: Dermal nerve sheath myxoma