Bilateral and Multifocal Acquired Cystic Disease–Associated Renal Cell Carcinomas in Patient With End-Stage Renal Disease Caused by Systemic Lupus Erythematosus

The disease entity of acquired cystic disease–associated renal cell carcinoma (ACD-RCC) has been recently incorporated into the international renal tumor classification. We performed a clinicopathologic study of a patient with bilateral and multifocal ACD-RCCs. The patient received long-term hemodialysis in the end-stage renal disease caused by systemic lupus erythematous. First left-sided nephrectomy and after 6 months right-sided nephrectomy was performed. None of the preoperative radiologic examinations revealed lesions suspected of malignancy. All of the 6 tumors were incidentally found on grossing radical nephrectomy specimens. Histologically, tumors consisted of a variety of growth patterns (including papillary and cribriform) of neoplastic cells with granular eosinophilic cytoplasm and intratumoral oxalate crystals. Neoplastic cells were positive for AMACR, CK AE1/AE3, and CD10; focally positive for CK7; and negative for PAX8. Seven months after the first nephrectomy, the patient still receives dialysis. There was no evidence of lymph node or distant metastases.