Key result
RT-PCR for SYT-SSX1 translocation successfully diagnoses primary renal synovial sarcoma in a pediatric patient.
Why the study?
Primary renal synovial sarcomas are rare mesenchymal neoplasias of uncertain differentiation that typically occur in the extremities of young patients.
This case highlights the rare presentation of Homer Wright-like rosettes in primary renal synovial sarcoma, emphasizing the importance of molecular testing (SYT-SSX1 translocation) for accurate diagnosis.
Supports molecular testing in atypical pediatric renal tumors; extends histologic spectrum of primary renal synovial sarcoma.
Synovial sarcoma is a mesenchymal neoplasia of uncertain differentiation that usually presents in the extremities of young patients. Primary renal synovial sarcomas are rare. We report the case of an 11-year-old female patient initially diagnosed with an infected renal cyst. Imaging studies showed a heterogenous lesion measuring 7.1 × 6.9 cm. The renal mass was partially resected. The specimen received was a multi-fragmented pseudocystic lesion. Microscopic examination showed a round blue cell tumour with formation of rosettes, within a myxoid background, as well as fascicles of fusiform cells with anisocytosis. The immunophenotype showed positivity to CD56, AE1/AE3, and vimentin, as well as weak positivity to TLE1, BCL2, EMA and, S100. Molecular assays were done to confirm the possibility of a synovial sarcoma. The result showed a SYT-SSX1 translocation. This case exemplifies the multifaceted histology of synovial sarcoma.
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José et al. (2026) studied Primary renal synovial sarcoma (n=1). Chemotherapy (isophosphamide and doxorubicin) was evaluated on Confirmation of diagnosis through detection of SYT-SSX1 translocation. Primary renal synovial sarcoma was successfully diagnosed through RT-PCR for SYT-SSX1 translocation in an 11-year-old girl presenting with a complex renal mass.
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