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<p>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&#xa0;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.</p>

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A diagnostic challenge: primary renal biphasic synovial sarcoma presenting Homer Wright-like rosettes

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月10日

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  • Lizardo-Thiebaud María José,
  • Andrade-Rojas Juan José,
  • Hernandez-Frausto Arnoldo,
  • Eguía-Aguilar Pilar,
  • Corcuera Delgado Celso Tomás

摘要

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.