Sarcomatoid differentiation in chromophobe renal cell carcinoma (ChRCC) is a rare finding and a significant predictor of worse outcomes. When the sarcomatoid component overgrows the conventional component or is the only component on a biopsy, the differential diagnoses encompass a variety of entities. Therefore, we reviewed 22 sarcomatoid ChRCCs and characterized the immunophenotype. Given that renal carcinomas with sarcomatoid features may benefit from immune checkpoint inhibitor-based therapy we also assessed the programmed death-ligand 1 (PD-L1) (28-8) expression. DOG1, CD117, cytokeratin 7, and PAX8 were negative in 100%, 88%, 63%, and 44% of the sarcomatoid components, respectively. GATA3 was expressed in 31% of the conventional components and in 50% of the sarcomatoid components. One conventional and 3 sarcomatoid components expressed PD-L1. Sarcomatoid ChRCCs have a high propensity for metastases and cancer progression. Distant metastatic disease was seen in 73% of the cases and median survival in this cohort was <1 year. The sarcomatoid portion had increased expression of PD-L1 and frequent loss of expression of multiple immunohistochemical markers associated with ChRCC. Half of the sarcomatoid ChRCC exhibited GATA3 expression, 3 of which did not express PAX8.
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