Intraabdominal Desmoplastic Small Round Cell Tumor With EWS/ERG Fusion Transcript
| Autor: | Javier Pardo-Mindán, Aureli Torné, Antonio Cardesa, Jaume Ordi, Begoña Mellado, Xavier Iglesias, E de Alava |
|---|---|
| Rok vydání: | 1998 |
| Předmět: |
Adult
Pathology medicine.medical_specialty Transcription Genetic Desmoplastic small-round-cell tumor CD99 Vimentin Polymerase Chain Reaction Pathology and Forensic Medicine Fusion gene Transcriptional Regulator ERG medicine Humans Oncogene Proteins biology Peripheral Primitive Neuroectodermal Tumor Flow Cytometry medicine.disease Immunohistochemistry Magnetic Resonance Imaging DNA-Binding Proteins Microscopy Electron Fusion transcript Abdominal Neoplasms Trans-Activators biology.protein Female Surgery Sarcoma Anatomy Tomography X-Ray Computed Transcription Factors |
| Zdroj: | The American Journal of Surgical Pathology. 22:1026-1032 |
| ISSN: | 0147-5185 |
| DOI: | 10.1097/00000478-199808000-00014 |
| Popis: | This report describes an intraabdominal small cell tumor in a 37-year-old woman, with clinical, topographic, and morphologic features highly suggestive of the desmoplastic small round cell tumor. Immunohistochemical analysis revealed a polyphenotypic profile consistent with this tumor--positivity for keratin, epithelial membrane antigen, neuron-specific enolase, vimentin, and desmin--but, in addition, a strong membranous immunoreactivity for CD99 (MIC2 protein). Reverse transcription polymerase chain reaction revealed a EWS/ERG fusion transcript characteristic of the Ewing's sarcoma/peripheral primitive neuroectodermal tumor group of tumors, rather than the EWS/WT1 chimeric transcript typical of the desmoplastic small round cell tumor. This is the third report of a hybrid tumor with features of the desmoplastic small round cell tumor and Ewing's sarcoma/peripheral primitive neuroectodermal tumor, and the first one with the EWS/ERG fusion gene. Our case shows the existence of some overlap between these two groups of tumors, which are considered to be histogenetically different, and the need for further studies of molecular characterization of small cell tumors, especially in those with atypical morphologic or immunohistochemical features. |
| Databáze: | OpenAIRE |
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