Desmoplastic small round cell tumor of the kidney mimicking wilms tumor: A case report and review of the literature
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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. © 2009 by Lippincott Williams & Wilkins.
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Desmoplastic small round cell tumor, Kidney, Small round cell tumor, Wilms tumor, RNA binding protein EWS, WT1 protein, bone metastasis, cancer chemotherapy, cancer morphology, cancer radiotherapy, cancer staging, case report, child, computer assisted tomography, desmoplastic small round cell tumor, differential diagnosis, fusion gene, human, human tissue, immunohistochemistry, kidney, lung metastasis, male, nephrectomy, nephroblastoma, priority journal, radiation dose, reciprocal chromosome translocation, school child, thorax radiography, Carcinoma, Small Cell, Child, Chromosomes, Human, Pair 11, Chromosomes, Human, Pair 22, Diagnosis, Differential, Humans, Immunohistochemistry, Kidney Neoplasms, Male, Oncogene Proteins, Fusion, Tumor Markers, Biological, Wilms Tumor
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Applied Immunohistochemistry and Molecular Morphology, v. 17, n. 6, p. 557-562, 2009.
