Diffuse infiltrating retinoblastoma.
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The case is described of a benign medulloepithelioma in a child which originally was apparently confined to the iris.
A case of embryonal medulloepithelioma (diktyoma) presenting with perforated infected eye in a 13-year-old Black African girl is described. The tumour mass occupied most of the deformed eye, and invasion of the sclera anteriorly was seen. There was no evidence of orbital or distant tumour involvement. It is suggested that with increasing age these tumours are more likely to show frankly malignant features.
A unique teratoid medulloepithelioma showing a conspicuous vascular component with orbital involvement is described.
Sixteen cases of medulloepithelioma are described. Clinical data and follow-up were available on 15. Four patients underwent iridocyclectomy initially; all later needed enucleation and one had an orbital recurrence. The remaining 12 patients underwent primary enucleation. All 15 patients with follow-up are alive with no evidence of tumour recurrence. It is suggested that enucleation be performed for all but the most localised tumour. Rubeosis was noted in 13 of the 16 eyes, and this may assist in making the diagnosis. The World Health Organisation histological classification of medulloepithelioma was applied, but some problems were encountered, particularly where the presence of heteroplastic brain tissue was used as a criterion for teratoid tumour and where rosettes were used as a criterion for malignancy.
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BACKGROUND: Over 90% of Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) cases have the t(11;22) chromosomal rearrangement, which is also found in other small round cell tumours, including desmoplastic small round cell tumour (DSRCT) and clear cell sarcoma (CCS). Although this rearrangement can be analysed by fluorescence in situ hybridisation (FISH) using routinely formalin fixed, paraffin wax embedded (FFPE) tissues when fresh or frozen tissues are not available, a sensitive and convenient detection method is needed for routine clinical diagnosis. AIMS: To investigate the usefulness of newly developed probes for detecting EWS rearrangement resulting from chromosomal translocations using FISH and FFPE tissue in the clinical diagnosis of ES/PNET, DSRCT, and CCS. METHODS: Sixteen ES/PNETs, six DSRCTs, and six CCSs were studied. Three poorly differentiated synovial sarcomas, three alveolar rhabdomyosarcomas, and three neuroblastomas served as negative controls. Interphase FISH analysis was performed on FFPE tissue sections with a commercially available EWSR1 (22q12) dual colour, breakapart rearrangement probe. RESULTS: One fused signal and one split signal of orange and green, demonstrating rearrangement of the EWS gene, was detected in 14 of 16 ES/PNETs, all six DRSCTs, and five of six CCSs, but not in the negative controls. CONCLUSIONS: Interphase FISH using this newly developed probe is sensitive and specific for detecting the EWS gene on FFPE tissues and is of value in the routine clinical diagnosis of ES/PNET, DSRCT, and CCS.
AIMS: To determine the possible histogenesis of the intracranial variant of olfactory neuroblastoma. METHODS: Four specimens from three cases of intracranial olfactory neuroblastoma were studied by light microscopy and immuno-histochemistry, and electron microscopy in two cases. RESULTS: Light microscopical examination showed small cell tumour with additional features of epithelioid cells in one case and ganglion cells in another. Olfactory and Homer-Wright rosettes were present. All the specimens showed a uniform positive reaction to neurone specific enolase, S-100, and cytokeratin antibodies. Glial fibrillary acidic protein was absent. The salient electron microscopic features were the presence of cell junctions, cytoplasmic intermediate filaments, basal bodies and cytolasmic processes. Dense cored vesicles were absent. CONCLUSIONS: The results strongly support the view that intracranial olfactory neuroblastomas are of olfactory epithelial origin and differ from conventional neuroblastomas.
A case of olfactory neuroblastoma is reported. Light microscopic examination showed various arrangements of poorly differentiated tumour cells forming either uniform sheets or convoluted cords of multiple cell layers orientated toward a richly vascular stroma. Electron microscopic examination showed the presence of abundant cytoplasmic filaments and processes, and dense core endocrine vesicles ranging from 100-160 nm in diameter in both the perinuclear region and tumour cell processes. Immunohistochemical staining was positive in most of the tumour cells for neuron specific enolase, and in a few cells for S-100 protein, vimentin, and serotonin, but staining for desmin and keratin produced no reaction.
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A six month old boy had a mesencephalic melanotic neoplasm of the neuro-epithelial derivatives which produced mucin. Location in the mesencephalon and production of the mucin have not been described previously for this kind of intracranial tumour. The various cellular elements within the growth are in accord with diverse differentiations of the neuroepithelium of the neural tube, the neuroectodermal part of the neural crest, or both. Derivatives of the neural tube and crest can be melanotic, and some neuroepithelial derivatives (ependyma, oligodendrocyte, and choroidal epithelium) produce mucin. Hence, melanotic neuroepithelial neoplasms with mucinous product can occur. The present tumour is similar to lesions variously designated as melanotic medulloblastoma, retinal anlage tumour, and progonoma. Diverse differentiation of the neuroepithelium of the neural tube, or of the neuroectodermal part of the neural crest, or both would create these variously named tumours. The frequency of melanotic neuroepithelial neoplasms in infancy, and the presence of papillae strongly suggest congental derivation.
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