Abstract

TEJERINA GONZALEZ, E. et al. Small round cell desmoplastic intraabdominal tumor: Literature review on the subject of a case with cytological, histopathological, immunohistochemical and molecular. Oncología (Barc.) [online]. 2007, vol.30, n.1, pp.35-41. ISSN 0378-4835.

PURPOSE: To review the cyto-histopathological, immunohistochemical and molecular characteristics of small round cell desmoplastic tumor (DSRCT). MATERIAL AND METHODS: A 18 year old male with an intraabdominal epigastric mass. RESULTS: The tumor consisted of nests and masses of undifferentiated small round cells embedded in a desmoplastic stroma, with areas of rosette formation immuno-reactive to cytokeratins, vimentin, desmin, enolase, and WT1 fraction of the EWS/WT1 fusion protein transcript. The molecular study demonstrated the existence of the translocation t(11;22)(p13;q12). CONCLUSIONS: The solid areas and rosette formation have been described in other cases of DSRCT. The chimeric transcription product can be detected by RT-PCR, Southern-blot, Western-blot, and immunohistochemistry. Cytology is especially useful in recurrences. The differential diagnosis should be made with the small round undifferented cell tumors group.

Keywords : Intraabdominal small round cell desmoplastic tumor; Immunohiostochemistry; EWT/ WT1 fusion transcript.

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