Why the study?
Synovial sarcoma of the kidney is extremely rare, accounting for approximately 1% of renal tumors, carries a poor prognosis, and shares features with other renal malignancies.
Primary renal synovial sarcoma is an extremely rare entity that can mimic renal cell carcinoma clinically and requires histomorphology with immunohistochemistry for accurate diagnosis and management.
Rare PRSS demands IHC vigilance in atypical renal masses; leaves open optimal therapy for this aggressive subtype.
Approximately 5%-10% of soft-tissue tumor comprised synovial sarcoma (SS) and are commonly found in close proximity of large joints affecting young adults. However, SS of the kidney is extremely unusual (1%) and has a poor prognosis. There are three subtypes of primary renal SS (PRSS). The monophasic variant is the most common and shares the same clinical, molecular, and ultrastructural features with other subtypes. Histomorphology with immunohistochemistry (IHC) differentiates it from other sarcomas of kidney and sarcomatoid renal cell carcinoma. Genetic analysis reveals specific SYT-SSX2 (X, 18) translocation. Herein, a case of PRSS (monophasic variant) is presented in a 31-year-old female with right-sided loin pain and huge mass of size (11.9 cm × 9.3 cm × 10 cm) without associated hematuria. It was clinically thought to be renal cell carcinoma. Radical nephrectomy was performed, followed by histopathology and IHC which suggested PRSS. Then, combined chemotherapy of doxorubicin with cyclophosphamide was rendered, and on follow-up, she is doing well with an uneventful course.
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Mohanty et al. (2023) studied this question.