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KMID : 0357920010350050451
Korean Journal of Pathology
2001 Volume.35 No. 5 p.451 ~ p.454
Desmoplastic Small Round Cell Tumor of the Sigmoid Colon - A Case Report -
Kim Kwang-Il

Kim Jung-Yeol
Kim In-Sun
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.
KEYWORD
Sarcoma, Small cell, Colon, Rhabdoid tumor
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