A novel case of synovial sarcoma of the kidney: impact of SS18/SSX analysis of renal hemangiopericytoma-like tumors.

We report a new case of synovial sarcoma of the kidney. The patient underwent nephrectomy because of a large tumor in the right kidney. The histologic diagnosis was hemangiopericytoma. Less than 1 year after primary surgery the patient was reoperated due to massive local recurrence. Histology now revealed a poorly differentiated tumor tissue with hemangiopericytoma-like features. Immunostainings showed immunoreactivity to cytokeratin, epithelial membrane antigen, and vimentin. The tumor was negative to CD34 and factor VIII. The tumor cell proliferation, assessed by Ki-67, was high. RT-PCR analysis and sequence analysis demonstrated the presence of SS18/SSX2 fusion gene. Review of the histologic specimens from the original tumors confirmed hemangiopericytoma-like morphology. The new diagnosis was poorly differentiated synovial sarcoma. At the time of reoperation, lung metastases were detected radiologically, reflecting a very aggressive phenotype. To our knowledge, this is the third case of poorly differentiated synovial sarcoma of the kidney. Common for all these three cases is the hemangiopericytoma-like histology and a very aggressive clinical behavior. These circumstances accentuate the impact of SS18/SSX analysis in diagnosis of renal hemangiopericytoma-like tumors.