Mohanty Pranita, Singh Surabhi, Batalia Pallak
Department of Pathology, IMS and Sum Hospital, Siksha 'O' Anusandhan University, Bhubaneswar, Odisha, India.
J Microsc Ultrastruct. 2023 Mar 22;13(2):109-111. doi: 10.4103/jmau.jmau_72_22. eCollection 2025 Apr-Jun.
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.
约5%-10%的软组织肿瘤为滑膜肉瘤（SS），常见于大关节附近，好发于年轻人。然而，肾脏滑膜肉瘤极为罕见（1%），预后较差。原发性肾滑膜肉瘤（PRSS）有三种亚型。单相型是最常见的亚型，与其他亚型具有相同的临床、分子和超微结构特征。组织形态学结合免疫组化（IHC）可将其与肾脏的其他肉瘤及肉瘤样肾细胞癌区分开来。基因分析显示存在特异性的SYT-SSX2（X，18）易位。本文报告一例31岁女性PRSS（单相型）病例，患者右侧腰痛，有巨大肿块（11.9 cm×9.3 cm×10 cm），无血尿。临床诊断考虑为肾细胞癌。行根治性肾切除术，术后组织病理学及免疫组化检查提示为PRSS。随后给予阿霉素联合环磷酰胺的化疗方案，随访期间，患者病情平稳，恢复良好。
