Sinonasal desmoplastic small round cell tumor: a case report.
A case of sinonasal desmoplastic small round cell tumor in a 21-year-old woman is presented. The tumor possessed the diagnostic histologic, immunohistochemical, and genetic characteristics of desmoplastic small round cell tumor. Histologically, the tumor was composed of nests of tumor cells surrounded by a desmoplastic stroma. Immunohistochemical staining was positive for keratin, vimentin, desmin, and, focally, neuron-specific enolase. The desmin immunopositivity was of a classic dot-like perinuclear pattern. RT-PCR analysis showed the fusion transcript resulting from the t(11;22)(p13;q12) reciprocal translocation. This case of sinonasal desmoplastic small round cell tumor, the third reported case not associated with a serosal surface, further obscures the nature and histogenesis of this entity.
Finke
Nicole M
NM
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Lae
Marick E
ME
Lloyd
Ricardo V
RV
Gehani
Suresh K
SK
Nascimento
Antonio G
AG
D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D002880:Chromosomes, Human, Pair 11; D002892:Chromosomes, Human, Pair 22; D003131:Combined Modality Therapy; D003893:Desmin / Q000378:metabolism; D005260:Female; D018221:Fibromatosis, Abdominal / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D009363:Neoplasm Proteins / Q000378:metabolism; D010255:Paranasal Sinus Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D012334:RNA, Neoplasm / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014178:Translocation, Genetic
D014408:Biomarkers, Tumor; D003893:Desmin; D009363:Neoplasm Proteins; D012334:RNA, Neoplasm