Desmoplastic small round cell tumor of the kidney.
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant tumor usually present with widespread abdominal serosal involvement. Isolated cases occur in limbs, head and neck, and brain. We present a case of primary DSRCT of the kidney in a 41-year-old man. The tumor showed morphologic, immunohistochemical, and molecular characteristics similar to DSRCTs arising in other sites. Epithelial and mesenchymal markers were coexpressed in the tumor cells. RT-PCR analysis showed EWS-WT1 fusion transcripts resulting from the t(11;22)(p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of small blue round cell tumors of the kidney.
Su
Min-Cheng
MC
Department of Pathology, Min-Sheng General Hospital, Taipei, Taiwan, ROC.
Jeng
Yung-Ming
YM
Chu
Yuan-Chung
YC
D000328:Adult; D001483:Base Sequence; D018288:Carcinoma, Small Cell / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008969:Molecular Sequence Data; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014057:Tomography, X-Ray Computed
C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion