Desmoplastic small round cell tumor of the kidney mimicking Wilms tumor: a case report and review of the literature.
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney.
da Silva
Rogério Cardoso
RC
Department of Urology, School of Medicine, UNESP, Botucatu, SP, Brazil.
Medeiros Filho
Plínio
P
Chioato
Lucimara
L
Silva
Tácio R B
TR
Ribeiro
Sérgio M
SM
Bacchi
Carlos E
CE
D014408:Biomarkers, Tumor / Q000096:biosynthesis* / Q000235:genetics; D018288:Carcinoma, Small Cell / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002880:Chromosomes, Human, Pair 11; D002892:Chromosomes, Human, Pair 22; D003937:Diagnosis, Differential; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000378:metabolism / Q000473:pathology; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000096:biosynthesis* / Q000235:genetics; D009396:Wilms Tumor / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology
D014408:Biomarkers, Tumor; C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion