ABSTRACT
Purpose To report a rare case of renal rhabdoid synovial sarcoma and review the literature,in order to improve the realization for this disease and reduce misdiagnosis.Method The clinicopathological data of 1 case rhabdoid renal synovial sarcoma were retrospectively analyzed.The tumors were examined by immunohistochemical of EnVision two-step staining and FISH,the related literatures were reviewed.Result A 31-year-old male patient accepted the right kidney radical operation in November 2014 after imaging examination of right kidney tumor.Microscopically,the tumor cells showed short spindle cells with rich cytoplasm and eosinophilic bodies in the cytoplasm.The pathological diagnosis is the renal rhabdoid tumor for this time.The patient was found a tumor between the liver and the diaphragm by imaging examination in October 2015.The second operation was carried out successfully.Microscopically,the tumor cells were spindle with little cytoplasm and without eosinophilic bodies in the cytoplasm.It was a typical synovial sarcoma in morphology for this time.Immunohistochemical staining showed positive for vimentin,EMA,CD56,and TLE1,SS18SSX fusion gene was disclosed in the primary and recurrent tumor cells,it was therefore corrected as rhabdoid synovial sarcoma for the primary tumor.Conclusion Renal rhabdoid synovial sarcoma is rare.Renal primary rhabdoid synovial sarcoma is easily misdiagnosed as renal rhabdoid tumor.The renal rhabdoid synovial sarcoma has broadened the differential diagnosis of renal rhabdoid tumors spectrum.Even for a tumor with typical rhabdoid morphology,molecular biology method for differential diagnosis is needed.SS18-SSX fusion gene is the basis for diagnosis of synovial sarcoma.