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Desmoplastic Small Round Cell Tumor of the Sigmoid Colon - A Case Report -

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±è±¤ÀÏ, Kim Jung-Yeol, ±èÀμ±,
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±è±¤ÀÏ ( Kim Kwang-Il ) 
°í·Á´ëÇб³ ÀÇ°ú´ëÇÐ º´¸®Çб³½Ç

 ( Kim Jung-Yeol ) 
°í·Á´ëÇб³ ÀÇ°ú´ëÇÐ º´¸®Çб³½Ç
±èÀμ± ( Kim In-Sun ) 
°í·Á´ëÇб³ ÀÇ°ú´ëÇÐ º´¸®Çб³½Ç

Abstract


Desmoplastic small round cell tumor (DSRCT) is a distinctive disease with a multidirectional differentiation and an aggressive clinical course. It mostly occurs in mesothelial-lined sites, and tumors originating in the paratesticular region, pleura and central nervous system are rarely reported. We report a case of DSRCT occurring in the sigmoid colon of a 39-year-old man, which was difficult to distinguish from small cell neuroendocrine carcinoma. The tumor was characterized by small round cells with irregular nests, cords, or rosette-like structures in the striking desmoplastic stroma. Some tumor cells had a rhabdoid feature with eosinophilic cytoplasmic globules. The tumor cells showed immunoreactivity for cytokeratin, epithelial membrane antigen, vimentin, desmin, neuron-specific enolase and Leu-7. Electron microscopic finding revealed perinuclear globoid whorls of intermediate filaments pushing the nucleus eccentrically.

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Sarcoma;Small cell;Colon;Rhabdoid tumor

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