Clinicopathologic analysis of 4 cases of primary renal synovial sarcoma
- VernacularTitle:肾脏原发性滑膜肉瘤4例临床病理分析
- Author:
Wang ZHI-HUI
1
;
Wang XING-CHUN
;
Xue MEI
Author Information
1. 山东临沂市肿瘤医院
- Keywords:
Kidney;
synovial sarcoma;
pathology;
prognosis
- From:Chinese Journal of Cancer
2010;29(2):228-233
- CountryChina
- Language:Chinese
-
Abstract:
Background and Objective:Primary renal synovial sarcoma is rare and might be misdiagnosed as another renal tumor.This study demonstrates the clinicopathologic and immunohistochemical features,differential diagnosis,and prognosis of such tumors.Methods:Histologic slides and clinical data were reviewed for 4 patients with primary renal synovial sarcoma and immunohistochemical staining was performed. Molecular analysis was performed on 2 cases to demonstrate the presence of the SYT-SSX gene fusion transcripts by reverse transcriptase polymerase chain reaction(RT- PCR).Results:The patients were 2 women and 2 men aged from 32 to 48 years.The tumors were 10.0-15.0 cm in diameter, grey-white and solid.and hemorrhage or necrosis was observed. Microscopically,the tumors consisted of mitotically active, monomorphic plump spindle cells with indistinct cell borders growing in short, intersecting fascicles.Hypocellular myxoid areas and a prominent hemangiopericytomatous pattern were present in all cases.The average mitotic rate was 5-8 mitoses/10 high.power fields.Hemorrhage and tumor necrosis were easily found. Scanered small cyrsts lined with flat,cuboidal,or hobnailed epithelia were found in 3 cases.Tumor CD56(3/4),and focally for EMA(3/4)and Cytokeratin(3/4).SYT.SSXl gene fusion was detected in the 2 cases in which RT-PCR analysis was performed. One patient had tumor metastasis to the lung 6 months after surgery and died 5 months later.Multiple metastasis to the liver occurred in one patient and the patient died 1 3 months after the initial surgery.The other 2 patients had tumors recur at 8 and 15 months and died at 18 and 21 months,respectively,after the initial operation.Conclusion:Primary renal synovial sarcoma is rare, with poor prognosis, characterized by SYT-SSX gene fusion,and needs to be differentiated from other renal sarcomas.
