Indolent renal involvement with BRAF^V600E mutation: Erdheim‐Chester, a rare disease with a wide spectrum of clinical manifestations

Abstract

We present the case of a patient with three‐year indolent bilateral ureteral and perirenal masses. Clinical presentation, radiological context, and histopathological findings with detection of BRAF V600E mutation confirmed the diagnosis of Erdheim‐Chester disease (ECD). A review of current knowledge regarding diagnosis, clinical assessment, management, and treatment of ECD is also presented.