Unclassified Renal Cell Carcinoma: Diagnostic and Molecular Updates

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Unclassified renal cell carcinoma is a diagnostic category for renal cell tumors not fitting into one of the established subtypes in our current classification system and includes a heterogeneous group of tumors with variable clinicopathologic features and biological behaviors. Unclassified renal cell carcinoma with high-grade histologic features represents a particularly challenging group of tumors for diagnosis and management, given their complex pathologic features, aggressive clinical attributes, and a lack of standard therapy. Using an example case of high-grade unclassified renal cell carcinoma, this review discusses the diagnostic approaches to these tumors in the context of the World Health Organization 2016 classification system and provides updates on the molecular characteristics of these tumors.

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