| Olfactory neuroblastoma is not related to the Ewing family of tumors: absence of EWS/FLI1 gene fusion and MIC2 expression. | |
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MedLine Citation:
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PMID: 9580174 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The relationship of olfactory neuroblastoma to the Ewing sarcoma family of tumors remains controversial due to its variable histopathology and to conflicting or inconsistent cytogenetic, immunophenotypic, and molecular data. To address this issue, we performed a morphologic, immunohistochemical, and molecular study of 20 olfactory neuroblastomas. Morphologically, the tumors consisted of nests of primitive small, round, blue cells, usually set in a background of neurofibrillary stroma. Immunohistochemical stains revealed strong reactivity for neuroendocrine markers (synaptophysin, chromogranin, neuron-specific enolase) and only focal staining for cytokeratins in two cases. Immunostaining with antibody O13 to the Ewing sarcoma-associated MIC2 antigen was uniformly negative (0 of 17). Amplifiable RNA was extracted from paraffin-embedded tissue blocks of 11 cases, and no evidence of the chimeric EWS/FLI transcript, characteristic of Ewing sarcoma, was found in any case. The EWS gene was not rearranged using Southern blot analysis in one additional case in which high molecular weight DNA was available. These results disagree with the proposed classification of olfactory neuroblastoma in the Ewing sarcoma family of tumors and suggest that therapy developed for the latter tumor group may not be biologically rational for olfactory neuroblastoma. |
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Authors:
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P Argani; B Perez-Ordoñez; H Xiao; S M Caruana; A G Huvos; M Ladanyi |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The American journal of surgical pathology Volume: 22 ISSN: 0147-5185 ISO Abbreviation: Am. J. Surg. Pathol. Publication Date: 1998 Apr |
Date Detail:
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Created Date: 1998-04-23 Completed Date: 1998-04-23 Revised Date: 2010-09-01 |
Medline Journal Info:
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Nlm Unique ID: 7707904 Medline TA: Am J Surg Pathol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 391-8 Citation Subset: IM |
Affiliation:
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Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Antigens, CD / metabolism Blotting, Southern Cell Adhesion Molecules / metabolism Female Humans Immunohistochemistry Male Middle Aged Neuroblastoma / metabolism*, pathology Olfactory Nerve / metabolism*, pathology Oncogene Proteins, Fusion / metabolism* Peripheral Nervous System Neoplasms / metabolism*, pathology Polymerase Chain Reaction RNA, Neoplasm / analysis Retrospective Studies Sarcoma, Ewing's / metabolism*, pathology Transcription Factors / metabolism* Transcription, Genetic Translocation, Genetic Tumor Markers, Biological / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Antigens, CD; 0/CD99 protein, human; 0/Cell Adhesion Molecules; 0/EWS-FLI fusion protein; 0/Oncogene Proteins, Fusion; 0/RNA, Neoplasm; 0/Transcription Factors; 0/Tumor Markers, Biological |
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