title: Peripheral nerve sheath differentiation in malignant soft tissue tumours: an ultrastructural and immunohistochemical study.
abstract: Thirteen soft tissue sarcomas with ultrastructural evidence of nerve sheath differentiation were investigated by immunohistochemistry. Three arose in a major nerve or nerve trunk and four patients had von Recklinghausen's neurofibromatosis. Ultrastructurally, 10 cases showed variable differentiation towards Schwann cells, two resembled perineurial cells and one tumour had features suggestive of both cell types. Immunostaining for S-100 protein was positive in eight Schwann cell tumours, negative in the other two Schwann cell tumours and negative in those with perineurial-like cells. No cases demonstrated epithelial membrane antigen, so that the existence of perineurial cells in malignant nerve sheath tumours remains immunohistochemically unsubstantiated; it may be that the perineurial-like cells are merely incompletely differentiated Schwann cells, with nerve sheath differentiation manifesting a continuous spectrum. Leu 7 was detected in four Schwann cell tumours, three of which were ultrastructurally well differentiated. Cytokeratin and desmin were demonstrated only in an undifferentiated pleomorphic area of one Schwann cell tumour. Electronmicroscopy can aid diagnosis by revealing nerve sheath differentiation in malignant soft tissue tumours without demonstrable S-100 protein.
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, UK.
authors.forename: C
authors.initials: C
authors.lastname: Fisher
authors.forename: R L
authors.initials: RL
authors.lastname: Carter
authors.forename: S
authors.initials: S
authors.lastname: Ramachandra
authors.forename: D M
authors.initials: DM
authors.lastname: Thomas
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010524:Peripheral Nervous System Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure*; D009418:S100 Proteins / Q000032:analysis; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure*
chemical_list: D009418:S100 Proteins