Tubulocystic carcinoma of the kidney (TC-RCC) is a rare renal tumor and has been recently included as a distinct entity in WHO classification of renal neoplasms. We report the case of a patient with metastatic tubulocystic RCC whose disease progressed through standard of care treatment for nonclear RCC. However, genetic analysis revealed a germline pathogenic variant of fumarate hydratase (FH) gene, and the patient had a sustained and durable response to pazopanib.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10105324 | PMC |
| http://dx.doi.org/10.1159/000528697 | DOI Listing |
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