Abstract
A 42-year-old female presented with right back pain. The CT scan revealed a 72-mm space-occupying lesion in the middle portion of the right kidney. No metastasis was proven. She underwent laparoscopic radical nephrectomy and lymph node disection. The histopathological examination revealed a high-grade primitive small round tumor the cells of which were strongly positive for CD99 and vimentin. Fluorescence in situ hybridization analysis using a DNA probe for the Ewing sarcoma breakpoint region 1 (EWSR 1) on chromosome 22g12 revealed a rearrangement of the EWSR 1 locus. The diagnosis was Ewing's sarcoma / primitive neuroectodermal tumor of the kidney. She underwent 13 cycles of chemotherapy, and has no evidence of recurrence 19 months after surgery.
MeSH terms
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Adult
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Biomarkers, Tumor / genetics
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Calmodulin-Binding Proteins / genetics
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Chemotherapy, Adjuvant
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Chromosomes, Human, Pair 22 / genetics
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Combined Modality Therapy
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Diagnosis, Differential
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Disease-Free Survival
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Female
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Gene Rearrangement / genetics
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Humans
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In Situ Hybridization, Fluorescence
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Kidney / pathology
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Kidney Neoplasms / diagnosis*
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Kidney Neoplasms / pathology
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Kidney Neoplasms / surgery
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Laparoscopy
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Lymph Node Excision
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Nephrectomy
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Neuroectodermal Tumors, Primitive / diagnosis*
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Neuroectodermal Tumors, Primitive / drug therapy
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Neuroectodermal Tumors, Primitive / pathology
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Neuroectodermal Tumors, Primitive / surgery
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RNA-Binding Protein EWS
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RNA-Binding Proteins / genetics
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Radionuclide Imaging
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Sarcoma, Ewing / diagnosis*
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Sarcoma, Ewing / drug therapy
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Sarcoma, Ewing / pathology
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Sarcoma, Ewing / surgery
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Tomography, X-Ray Computed
Substances
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Biomarkers, Tumor
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Calmodulin-Binding Proteins
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EWSR1 protein, human
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RNA-Binding Protein EWS
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RNA-Binding Proteins