Srpski arhiv za celokupno lekarstvo 2013 Volume 141, Issue 11-12, Pages: 814-818
https://doi.org/10.2298/SARH1312814M
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Rapidly progressive course of primary renal synovial sarcoma: Case report
Marković-Lipkovski Jasmina (Medicinski fakultet, Institut za patologiju, Beograd)
Sopta Jelena (Medicinski fakultet, Institut za patologiju, Beograd)
Vještica Jelena (Medicinski fakultet, Institut za patologiju, Beograd)
Vujanić Gordan (Royal Manchester Children’s Hospital, Manchester, United Kingdom)
Tulić Cane (Medicinski fakultet, Beograd + Klinički centar Srbije, Urološka klinika, Beograd)
Introduction. Primary kidney sarcoma, especially synovial sarcoma (SS), is a
very rare neoplasm. Pre-operative 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.
Case Outline. 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 120x85 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. Conclusion.
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.
Keywords: primary renal synovial sarcoma, SYT-SSX2, RT-PCR
Projekat Ministarstva nauke Republike Srbije, br.
OI 175047