Clinical analysis of 21 cases of primary synovial sarcoma of kidney
10.3760/cma.j.cn431274-20240320-00465
- VernacularTitle:21例原发性肾脏滑膜肉瘤的临床分析
- Author:
Lili JIANG
1
;
Chenxi SHI
1
;
Dongmei GU
1
Author Information
1. 苏州大学附属第一医院病理科，苏州 215000
- Publication Type:Journal Article
- Keywords:
Sarcoma, synovial;
Kidney neoplasms;
SYT- SSX
- From:
Journal of Chinese Physician
2025;27(2):265-270
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical manifestations, pathological features, treatment methods and prognosis of primary renal synovial sarcoma (PRSS) in order to improve the clinical understanding and treatment level of the disease.Methods:The clinical data, pathological features, immunohistochemical indexes, SYT gene detection, treatment and follow-up results of 21 patients with PRSS reported in the First Affiliated Hospital of Soochow University from 2018 to 2023 were retrospectively analyzed. Kaplan-Meier method was used to calculate the relationship between age and cumulative survival rate. Results:The male to female ratio of PRSS patients was 16∶5, the ratio of left and right kidneys was 7∶14, the median age was 50 years old, the mean age was 44.5 years old, the median tumor length diameter was 9.7 cm, and the mean tumor length diameter was 6.7 cm. 57%(12/21) reported low back pain and 48%(10/21) reported gross hematuria. Immunohistochemical examination showed 100% positive for Vimentin(16/16), BCL-2(18/18) and TLE-1(10/10), 69%(9/13) and 85%(6/7) positive for CD99 and CD56, respectively. Genetic testing found SYT translocation signal in 92% of cases (12/13). Next generation sequencing (NGS) detected SS18- SSX1 fusion in 2 cases and SS18- SSX2 fusion in 1 case. SS18- SSX1+ S18- SSX2 fusion was found in 1 case, and one case of SS18- NEDD4 fusion occurred. Preoperative neoadjuvant chemotherapy was performed in 2 cases, postoperative chemotherapy in 11 cases, postoperative chemoradiotherapy in 2 cases, and surgery only in 5 cases. All patients were followed up for 3 to 42 months, and 4 cases were lost to follow-up. Patients with younger onset had a longer cumulative survival time. Patients had a median survival time of 13 months and a mean survival time of 15.4 months. Conclusions:PRSS is more common in middle-aged men, the right kidney is easy to occur, the length of the mass is usually >7 cm, most cases can be initially diagnosed by pathologic morphology, immunophenotype, fluorescence in situ hybridization or NGS STY gene detection can provide accurate diagnosis. The prognosis of PRSS is very poor, and the cumulative survival rate is negatively correlated with the age of onset. Surgical resection combined with chemotherapy is the main treatment for PRSS.
