Authors
Hui Min Tan, Busra Yaprak Bayrak, Katrina Collins, Ziying Ye, Rumeal D Whaley, Ming Liang Oon, Geethanjali Gude, Wai-Tung Lee, Fei Chen, Wendong Yu, Mahmut Akgul, Pedram Argani, Fang-Ming Deng, Michelle R Downes, Nancy Greenland, Sounak Gupta, Huiying He, Jiaoti Huang, Timothy D Jones, Selva Kabul, Seema Kaushal, Kemal Kosemehmetoglu, Antonio Lopez-Beltran, Fiona Maclean, Rohit Mehra, Ankur R Sangoi, Rajal B Shah, Stephanie E Siegmund, Puay Hoon Tan, Toyonori Tsuzuki, Bangchen Wang, Sean R Williamson, Chin-Lee Wu, Douglas Jian-Xian Wu, Ting Zhao, Qiu Rao, Liang Cheng
Published in
The American journal of surgical pathology. Sep 03, 2026. Epub Sep 03, 2026.
Abstract
Synovial sarcoma is a rare malignant mesenchymal neoplasm that typically occurs in soft tissue sites, and the kidney is an uncommon primary location. Previous studies of primary renal synovial sarcoma are limited by small sample sizes, and our understanding remains incomplete. Here, we present the largest multi-institutional case series to date of primary renal synovial sarcoma, with a focus on novel and molecular findings. A total of 70 cases were contributed by multiple institutions. Comprehensive clinical and histopathologic data were collected and analyzed. The mean patient age was 40 years, with a male-to-female ratio of 1.9:1. The most common presenting signs and symptoms were pain and hematuria. The mean tumor size was 11.6 cm (range: 2.3 to 26 cm), with frequent cystic change and necrosis. The mean follow-up was 29 months (range: 2 to 129 mo), and the rates of metastasis, recurrence, and death due to disease were 62.7%, 33.3%, and 50.0%, respectively. Histologically, 56.1% were monophasic synovial sarcoma, 15.1% were biphasic, and 28.8% were poorly differentiated or showed round cell features. Molecularly, SS18::SSX2 fusion was detected in the majority of cases assessed (n=19), followed by SS18::SSX1 fusion (n=6) and a rare SS18::NEDD4 fusion (n=1). Given the morphologic and immunohistochemical overlaps with many other neoplasms, accurate diagnosis with preferably molecular techniques is crucial for appropriate prognostication and treatment of this aggressive tumor.
PMID:
42687804
Bibliographic data and abstract were imported from PubMed on 03 Sep 2026.
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