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sinonasal desmoplastic small round cell tumor

Friday 6 November 2009

Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young male patients and is characterized by a reciprocal translocation t(11;22)(p13;q12) associated with the EWS-WT1 gene fusion transcript.

Synopsis

- nests of tumor cells surrounded by a desmoplastic stroma

Immunohistochemistry

- Positivity: keratin, vimentin, desmin, and, focally, neuron-specific enolase.
- desmin immunopositivity : classic dot-like perinuclear pattern.

RT-PCR analysis

- fusion transcript EWS-WT1 resulting from the t(11;22)(p13;q12) reciprocal translocation.

See also

- desmoplastic small round cell tumor

References

- Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG. Am J Surg Pathol. 2002 Jul;26(7):823-35. PMID: #12131150#

- Sinonasal desmoplastic small round cell tumor: a case report. Finke NM, Lae ME, Lloyd RV, Gehani SK, Nascimento AG. Am J Surg Pathol. 2002 Jun;26(6):799-803. PMID: #12023587#