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Year : 2021  |  Volume : 10  |  Issue : 2  |  Page : 112-115

Renal synovial sarcoma: A rare entity with a poor prognosis

1 Department of Urology, Sri Venkateswara Institute of medical sciences(SVIMS), Tirupati, Andhra Pradesh, India
2 Department of Urology, BYL Nair Hospital and Topiwala National Medical College, Mumbai, Maharashtra, India Where the work was carried out: Department of Urology, Sri Venkateswara Institute of medical sciences (SVIMS), Tirupati, Andhra Pradesh, India

Correspondence Address:
Dr. Suraj Pinni
Department of Urology, BYL Nair Hospital and TNMC, Mumbai - 400008, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jdrntruhs.jdrntruhs_126_21

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Renal synovial sarcoma is a rare disease with less than 200 cases reported in the literature. A 36-year-old lady presented with hematuria and underwent radical nephrectomy for a left-sided renal synovial sarcoma. She developed lung metastasis and the disease progressed further despite of chemotherapy. Renal synovial sarcoma can be diagnosed by studying morphology and performing an Immuno-histochemistry (IHC) panel (Transducin-like enhancer of split (TLE); B Cell Lymphoma (BCL)-2; CD99) on resected tumor tissue. Molecular testing for t(x; 18)(p11.2;q11.2) translocation is considered the gold standard diagnostic test. The common presenting symptoms include hematuria, pain, or a palpable lump. The imaging modalities are not diagnostic and surgical resection is the treatment of choice at present. Due to the rarity of the disease, the treatment guidelines are not well-defined. Anthracyclines alone or in combination with Ifosfamide has been reported to be used in the literature in the adjuvant as well as metastatic settings. The overall prognosis is poor with a short median survival ranging from 6 to 24 months.

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