Korean J Pathol.  2001 Oct;35(5):451-454.

Desmoplastic Small Round Cell Tumor of the Sigmoid Colon

• Kim KI
• Kim JY
• Kim I

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

MeSH Terms

Adult
Carcinoma, Neuroendocrine
Central Nervous System
Colon
Colon, Sigmoid*
Cytoplasm
Desmin
Desmoplastic Small Round Cell Tumor*
Eosinophils
Humans
Intermediate Filaments
Keratins
Mucin-1
Phosphopyruvate Hydratase
Pleura
Rhabdoid Tumor
Sarcoma
Strikes, Employee
Vimentin
Desmin
Keratins
Mucin-1
Phosphopyruvate Hydratase
Vimentin