Multilocular cystic renal cell carcinoma, a Challenging Diagnosis: Report of a case and review literature

Background: Multilocular cystic renal cell carcinoma (MCRCC) is a rare entity with grosssimilarities among various multicystic neoplasm including benign & malignant one such asbenign multilocular cysts, multilocular cystic RCCs & cystic renal cancer that can inducedifficulties in diagnosis &treatment .We report a case of MCRCC in a 43y/o female with clinical & radiological diagnosis ofbenign multilocular cystic disease of the kidney.Case presentation: A 43-Year-Old female came to Imam Khomeini hospital of Ahwaz ,deportment of urology with a mild persistent right flank pain with no relevant medicalhistory. Abdominal imaging study revealed a benign multilocular cystic disease of thekidney.A total nephrectomy has been performed and the specimen submitted to pathologylaboratory. Gross examination of the lesion revealed multilocular cystic lesion of kidneyfilled with bloody fluid. Microscopic examination revealed multiple cysts lined by flat tocuboidal epithelium & small scattered foci of nests of neoplastic cells with clear cytoplasmand rather uniform dense nuclei showing few foci of luminal growth, so overall diagnosed asMCRCC.Conclusion: MCRCC is an uncommon subtype of renal cell carcinoma & the term MCRCCshould be used exclusively to identifying cystic renal cell carcinoma with a small percentageof neoplastic clear cell within the cyst wall. Prognosis is excellent and is associated in totalcure in most cases. Local recurrence is related to inadequate local excision. A definitepreoperative diagnosis is very difficult to make in most cases & surgical exploration isconsidered to the only way to accurate diagnosis.