Sertoli–Leydig cell tumors of the ovaries (SLCTs) are rare androgen-producing neoplasms. Clear cell papillary renal cell carcinoma (CCP-RCC) is another rare tumor type that occurs in the kidney. We describe a unique case of a woman who was diagnosed with both tumors simultaneously. Both tumors were successfully removed by surgery. Despite extensive genetic testing, including DICER1 mutation screening and analysis of more than 280 cancer-related genes, no mutations were detected, suggesting that these tumors may have arisen by chance or unknown mechanisms. This case underlines the importance of a multidisciplinary approach and genetic screening, even if no mutations were detected. It also raises new questions about how such rare tumors may be linked, highlighting the need for further research and long-term surveillance.

Synchronous Ovarian Sertoli–Leydig Cell and Clear Cell Papillary Renal Cell Tumors: A Rare Case Without Mutations in Cancer-Associated Genes

Paola Ungaro
;
2025

Abstract

Sertoli–Leydig cell tumors of the ovaries (SLCTs) are rare androgen-producing neoplasms. Clear cell papillary renal cell carcinoma (CCP-RCC) is another rare tumor type that occurs in the kidney. We describe a unique case of a woman who was diagnosed with both tumors simultaneously. Both tumors were successfully removed by surgery. Despite extensive genetic testing, including DICER1 mutation screening and analysis of more than 280 cancer-related genes, no mutations were detected, suggesting that these tumors may have arisen by chance or unknown mechanisms. This case underlines the importance of a multidisciplinary approach and genetic screening, even if no mutations were detected. It also raises new questions about how such rare tumors may be linked, highlighting the need for further research and long-term surveillance.
2025
Istituto degli Endotipi in Oncologia, Metabolismo e Immunologia "G. Salvatore" (IEOMI)
Sertoli-Leyding cell tumor (SLCT)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.14243/560624
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