Thyroid-like follicular carcinoma of the kidney (TLFCK) is an extremely rare primary renal malignancy that typically has an indolent course and good prognosis. Histologically, this tumor mimics follicular carcinoma of the thyroid; however, typical thyroid markers are negative. There are fewer than 40 cases reported in the literature, and thus, the prognosis and course of disease is not well understood. Sarcomatoid differentiation has never been reported in a case of TLFCK. We present a case of a 48-year-old woman with an aggressive TLFCK with extensive sarcomatoid differentiation and metastatic disease at presentation. We performed targeted next-generation sequencing of both the thyroid-like component and the poorly differentiated sarcomatoid component using our solid tumor panel to evaluate for any disease-associated mutations and to better understand the molecular profile of these tumors.

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