title: [Primitive renal synovial sarcoma: a cystic tumor in young patients].
abstract: BACKGROUND
  The study of the clinical, histological and immunohistochemical aspects of three kidney tumors corresponding to synovial sarcomas operated on in our center over three years.
  
  
  METHODS
  Three patients aged between 27 and 33 had an enlarged nephrectomy for kidney tumors corresponding to a histological examination of a synovial sarcoma. The tumors were symptomatic in 100% of cases with back pain and spontaneous rupture. Size varied from 5 to 13cm. The radiological aspect was a cystic tumor (BosniakIV) in two cases and in the other a spontaneous perirenal hematoma. Two were in the right kidney and one in the left kidney. The parts were analyzed after fixation. Immunohistochemical coloration and an analysis in molecular biology by RT-PCR of fusion transcripts were carried out.
  
  
  RESULTS
  One patient died because of local development and metastasis 24 months after an enlarged nephrectomy associated with radio chemotherapy (Maidprotocol). Two patients were in total remission after an average of 25 months following the same treatment. The histology found a mesenchymal fusocellular monotone proliferation corresponding to a sarcoma. In all three cases, it was a biphasic form with plaques of fusiform cells and epithelial cells. The immunohistochemical study shows a positivity of the contingent epithelial and fusiform. The muscular markers were negative. A study in molecular biology of the fusion transcript allowed for the finding of a translocation (X;18) in all three cases.
  
  
  CONCLUSIONS
  Primitive synovial sarcoma of the kidney occured in young patients. The translocation (X;18) is pathognomonic of the diagnosis. The prognosis is bleak despite complete excision, radiotherapy and chemotherapy.
authors: Service d'urologie et de la transplantation rénale, CHU de Michallon-Grenoble, 38043 Grenoble cedex 9, France. JALong@chu-grenoble.fr
authors: J-A
authors: JA
authors: Long
authors: E M
authors: EM
authors: Dinia
authors: G
authors: G
authors: Saada-Sebag
authors: J
authors: J
authors: Cyprien
authors: D
authors: D
authors: Pasquier
authors: C
authors: C
authors: Thuillier
authors: N
authors: N
authors: Terrier
authors: B
authors: B
authors: Boillot
authors: J-L
authors: JL
authors: Descotes
authors: J-J
authors: JJ
authors: Rambeaud
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001416:Back Pain / Q000209:etiology; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics / Q000276:immunology; D003560:Cysts / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000401:mortality / Q000601:surgery; D005260:Female; D006406:Hematoma / Q000209:etiology; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000401:mortality / Q000601:surgery; D008297:Male; D009392:Nephrectomy / Q000379:methods; D015514:Oncogene Proteins, Fusion / Q000032:analysis* / Q000235:genetics / Q000276:immunology; D011379:Prognosis; D012189:Retrospective Studies; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012422:Rupture, Spontaneous; D013584:Sarcoma, Synovial / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000401:mortality / Q000601:surgery; D016019:Survival Analysis; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein