Adv Anat Pathol 2021 Sep 27. Epub 2021 Sep 27.
Department of Pathology and Laboratory Medicine, University of Kansas School of Medicine, Kansas City, KS Department of Pathology, University of Utah and ARUP Laboratories, Salt Lake City, UT Departments of Pathology and Urology, Virginia Commonwealth University, School of Medicine, Richmond, VA Department of Pathology and Laboratory Medicine, University of Tennessee Health Sciences, Memphis, TN Department of Urology, USC Keck School of Medicine, Los Angeles, CA.
Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) is typically considered a high-grade, aggressive subtype of RCC that frequently arises in the setting of hereditary leiomyomatosis-renal cell carcinoma (HLRCC) syndrome. Increasing experience with HLRCC-associated RCC and FH-deficient RCC has resulted in recognition of tumors with lower grade morphologic features, overlapping with those of succinate dehydrogenase-deficient RCC and other low-grade oncocytic tumors. In this review article, we report a previously unpublished case that was recently encountered in our practice and review cases in the current literature with an aim of getting a better understanding of these oncocytic tumors and their morphologic spectrum. Read More