Primitive neuroepithelial tumours of soft tissues and of bone: further ultrastructural and immunocytochemical clarification of 'Ewing's sarcoma', including freeze-fracture analysis.

The ultrastructural appearances of 7 primitive neuroepithelial tumours (PNETs) originating in soft tissues and bone are described. Three of the tumours represented primary soft tissue lesions, while locally recurrent tumour or pulmonary metastases were studied from the 4 skeletal tumours, all of which had been diagnosed previously as Ewing's sarcomas. Rosettes were present in one of the soft tissue lesions and although not seen in the primary skeletal neoplasms, they were identified by light microscopy (LM) in 2 of 3 pulmonary metastases, one of which had the morphology of a neuroepithelioma, with innumerable Homer Wright rosettes. Conventional TEM revealed cytoplasmic processes in all cases and rosettes in varying stages of development were also evident, but the appearances did not achieve the level of cellular organization seen in neuroblastoma: microtubules were few, while dense-core granules varied in number but were generally sparse and pleomorphic, resembling lysosomes. However, typical neurosecretory granules were found in one lung metastasis; the neoplastic cells comprising the same tumour also had epithelial markers in the form of well constructed desmosomes, while freeze-fracture analysis demonstrated elaborate tight junctions. In thin sections, junctions in the other tumours appeared rudimentary, but freeze-fracture of a further case revealed small collections of membrane particles suggesting extremely poorly developed desmosomes. Immunocytochemical study of 4 tumours (2 originating in soft tissue and 2 in bone) demonstrated weak to moderate immunostaining for neurone-specific enolase and with several monoclonal antibodies reactive with neuroblastomas, but there was no evidence of immunolabelling for tyrosine hydroxylase.(ABSTRACT TRUNCATED AT 400 WORDS)