Durable Response to Pazopanib in a Patient with Fumarate Hydratase-Mutant Tubulocystic Renal Cell Carcinoma

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...

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Bibliographic Details
Published in:Case Reports in Oncology
Main Authors: Anjum Othman, Shiyam Kumar, Asim Qureshi, Zabah M. Jawa, Ikram A. Burney
Format: Article
Language:English
Published: Karger Publishers 2023-04-01
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Online Access:https://www.karger.com/Article/FullText/528697
Description
Summary: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.
ISSN:1662-6575