PubMed · 14428181
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G J OBERT, K D DEVINE, J R MCDONALD. Olfactory neuroblastomas.. https://doi.org/10.1002/1097-0142(196001%2F02)13%3A1%3C205%3A%3Aaid-cncr2820130132%3E3.0.co%3B2-g
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We describe an extremely rare case of a primary intrasellar esthesioneuroblastoma.
INTRODUCTION: Esthesioneuroblastoma is an uncommon malignancy of the olfactory neuroepithelium. The best treatment has yet to be defined. The purpose of this study is to analyze the tumors's behaviour to choose the ideal treatment, the therapeutic strategy and the patterns of failure. MATERIALS AND METHODS: We carry out a revision of the series published between 1994 to 2004. In these series, we found 39 papers with 713 patients. DISCUSSION CONCLUSIONS: In this review the 5-years survival rate is 51.2%. Through the analysis of 583 partients found in 34 publications, surgery (alone or combined) is the treatment most used (78%). The commonly management is surgical in combination with radiotherapy (47%).
A rare case of olfactory neuroepithelioma with rhabdomyoblasts in a 61-year-old man was investigated using electron microscopic and immunohistochemical methods. A large tumor enhanced by gadolinium-diethylenetriamine pentaacetic acid (DTPA) was demonstrated on magnetic resonance imaging (MRI), located within the anterior cranial fossa without bone destruction. The tumor mostly consisted of small cells with scant cytoplasm. Tubular rosettes were often found. Immunoreactivity for cytokeratin and epithelial membrane antigen (EMA) was strongly positive. Most of the tumor cells were shown to be positive for neuron-specific enolase (NSE) and vimentin and weakly positive for synaptophysin and S-100. Rhabdomyoblasts, which showed oval cells with abundant eosinophilic cytoplasm and a nucleus sometimes displaced toward the periphery of the cell body, were frequently intermingled with the tumor cells. The immunoreactivity for myoglobin was frequently positive in these oval cells. The MIB-1 index showed high values, of 20%-40%. About 10% of the tumor cells revealed positivity for p53 protein and vascular endothelial growth factor (VEGF). Ultra-structurally, numerous junctional complexes were observed between cell bodies and processes. The cell processes frequently contained numerous microtubules. There were sometimes numerous filaments with small aggregates of Z-band material and thick filament-ribosomal complexes in the oval cells. They were concluded to be consistent with rhabdomyoblasts on light microscopic and immunohistochemical findings.