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- Title
Synovial Sarcoma of the Kidney: Diagnostic Pitfalls in a Case with Myxoid Monophasic Differentiation and No Epithelial Biomarkers Expression.
- Authors
Pagliuca, Francesca; Carraturo, Emma; De Chiara, Anna; Vallese, Silvia; Giovannoni, Isabella; Alaggio, Rita; Cannella, Lucia; Tafuto, Salvatore; Franco, Renato
- Abstract
Synovial sarcomas are soft tissue tumours of uncertain origin, most commonly found in the upper or lower extremities. They are characterised by distinctive chromosomal rearrangements involving the gene SS18. Synovial sarcomas can occasionally arise also in visceral sites, but retroperitoneal SSs are very unusual. Among them, a few primary renal synovial sarcomas have been described in the scientific literature. Primary renal synovial sarcomas tend to be monophasic and often show cystic changes. Histologically, they can closely resemble other primary kidney tumours, mainly paediatric tumours such as nephroblastoma and clear cell sarcoma of the kidney. In the current work, a primary synovial sarcoma of the kidney with unusual morphological features (extensively myxoid stroma and immunohistochemical positivity for BCOR) is described. Molecular analysis, through targeted RNA sequencing, was of invaluable help in reaching the correct diagnosis. Despite locally advanced disease at presentation, the patient showed an unexpectedly brilliant response to chemotherapy.
- Subjects
SYNOVIOMA; SARCOMA; SCIENTIFIC literature; SYMPTOMS; CHROMOSOMAL rearrangement
- Publication
International Journal of Molecular Sciences, 2024, Vol 25, Issue 13, p7382
- ISSN
1661-6596
- Publication type
Article
- DOI
10.3390/ijms25137382