title: Rapidly progressive course of primary renal synovial sarcoma--case report.
abstract: BACKGROUND
  Primary kidney sarcoma, especially synovial sarcoma (SS), is a very rare neoplasm. Preoperative signs and symptoms are very similar to renal cell carcinoma, therefore, the proper diagnosis is very difficult and usually made after nephrectomy.This is a case report of primary renal SS.
  
  
  METHODS
  A 38-year-old man presented with a history of fever and hematuria, and right flank pain 3 weeks ago. Abdominal computerized tomography revealed a heterogeneous well-marginated soft tissue mass arising in the lower part of the right kidney. Right nephrectomy was performed. A cystic tumor of 120 x 85 mm in size with soft solid growth, and with the extensive areas of hemorrhage and necrosis was seen on gross examination. Histopathology revealed a neoplasm composed of solid monomorphic sheets of spindle cells. Immunohistochemistry showed tumor cells strongly positive for BCL2, CD99, CD56 and vimentin, and focally positive for epithelial membrane antigen (EMA). The histological diagnosis of primary renal SS was based on morphology and immunohistochemistry. FISH analysis and RT-PCR was carried out on formalin-fixed paraffin-embedded tissue sections. The molecular analysis demonstrated translocation of SYT gene on chromosome 18 and SSX2 gene on chromosome X. The findings were consistent with diagnosis of SS.
  
  
  CONCLUSIONS
  Our case shows that histopathological diagnosis of primary kidney SS, although difficult, is possible to be made on the basis of morphological and immunohistochemical analysis. However, this diagnosis should be corroborated by molecular techniques confirming SYT-SSX translocation on chromosome 18 and chromosome X. Here we present visceral monophasic SS with aggressive clinical course and poor outcome.
authors: Institute of Pathology, School of Medicine, University of Belgrade, Belgrade, Serbia. acal@matf.bg.ac.rs
authors: Jasmina
authors: J
authors: Marković-Lipkovski
authors: Institute of Pathology, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors: Jelena
authors: J
authors: Sopta
authors: Institute of Pathology, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors: Jelena
authors: J
authors: Vjestica
authors: Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors: Gordan
authors: G
authors: Vujanić
authors: School of Medicine, University of Belgrade, Belgrade, Serbia.
authors: Cane
authors: C
authors: Tulić
mesh_terms: D000328:Adult; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D013584:Sarcoma, Synovial / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
chemical_list: D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein