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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