Abstract
Renal Synovial Sarcoma (SS) are rare type of tumors arising from the mesenchymal tissue of the kidney. Its presenting features overlap with other renal tumors there by creating a diagnostic dilemma. Very few cases of primary renal SS are described so far in the literature with no defined treatment protocol described. We hereby describe a case report of this rare tumor in a 12 year old girl who presented to us with hematuria and flank pain. Histopathological analysis and the Immunohistochemistry (IHC) confirmed the diagnosis of primary monophasic synovial sarcoma. Follow-up post nephrectomy at six months showed no evidence of metastasis or recurrence.
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