Multiple angiomyolipomas mimicking metastases of concurrent clear cell renal cell carcinoma

INTRODUCTION: Concurrence of clear cell renal cell carcinoma and angiomyolipoma is quite rare. We report a case of large localized clear cell renal cell carcinoma with concurrent multiple angiomyolipomas mimicking lymph node metastases. CASE PRESENTATION: A 60‐year‐old woman presented with general malaise, weight loss, and intermittent fever. Computed tomography scan demonstrated an 8‐cm mass in the left kidney, enlarged para‐aortic lymph nodes, and small renal nodules adjacent to the main tumor. She was diagnosed preoperatively as having clear cell renal cell carcinoma (cT3a) with multiple para‐aortic lymph node metastases, and underwent laparoscopic radical nephrectomy and dissection of the para‐aortic lymph nodes. Pathologically, the main tumor was diagnosed as clear cell renal cell carcinoma. By contrast, both the para‐aortic lymph nodes and nodules were diagnosed as lipid‐poor angiomyolipomas. CONCLUSION: With the expanding first‐line use of molecular targeted therapy for metastatic renal cell carcinoma, nephrectomy may be avoided by overdiagnosis. Upfront nephrectomy can avoid overdiagnosis and undertreatment of nonmetastatic renal cell carcinoma.