Coexistence of renal epithelioid angiomyolipoma and clear cell carcinoma in patients without tuberous sclerosis

Renal epithelioid angiomyolipoma (EAML) is a rare but distinct variant of angiomyolipoma, closely simulating renal cell carcinoma or sarcoma both clinically and histopathologically. This report presents an unusual case of unilateral simultaneous renal EAML and renal clear cell carcinoma. A 52-year-old man without any sign of tuberous sclerosis had a complaint of 6-month history of pain in left renal area and had macroscopic hematuria twice within the recent 1 month. Computed tomography showed the presence of 2 masses in the upper and lower portion of the left kidney. The patient underwent left radical nephrectomy. Histological examination revealed the upper mass was composed of medium to large epithelioid cells with clear or eosinophilic cytoplasm and numerous giant multinucleated cells. Adult-appearing adipose tissue and coagulative necrosis could also be observed focally in the mass. Immunohistochemically, the tumor cells in the upper mass showed positive reactions to actin, HMB-45, Melan-A, and CD68 but negative reactions to pan-cytokeratin (pan-CK), epithelial membrane antigen, and CD10. However, the lower mass was composed of diffusely monomorphic clear cells with strongly immunoreactive for pan-CK, vimentin, and CD10, whereas without expression for HMB-45 and actin. The patient showed no evidence of recurrence or metastasis during 1-year postoperative following-up period. To the authors' knowledge, this is the first report of coincidental renal EAML and clear cell carcinoma in the same kidney. Unlike classic triphasic angiomyolipoma, adjuvant therapy after resection should be considered for renal EAML because of its malignant potential, more aggressive behavior and poor prognosis.