Multilocular cystic renal cell carcinoma (MCRCC) is an uncommon low grade RCC with unique morphologic features. MCRCC also known as multilocular clear cell RCC is a rare cystic tumor of the kidney with an excellent outcome. MCRCC has been recognized as a separate subtype of RCC in the 2004 World Health Organization (WHO) classification of adult renal tumors. MCRCC is diagnosed on the basis of strict histological criteria according to 2004 WHO classification. It is usually included in the group of tumors of undetermined malignant potential with low nuclear grade. We present a case of MCRCC in a 43-year-old female patient who presented with pain in left lumbar region and hematuria. Left-sided radical nephrectomy was performed, and on histopathologic examination it was diagnosed as MCRCC with Fuhrman nuclear grade 1. Immunohistochemistry with epithelial membrane antigen and vimentin confirmed the diagnosis.
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