A Rare Highly Aggressive Tumor of the Kidney: The Pure Epithelioid Angiomyolipoma

Aims: The authors presents a case of pure Renal epithelioid angiomyolipoma (EAML) and discuss the clinicopathological characteristics and the diagnosis problems of this entity.

Case Presentation: A 51 year-old man presented with a persistant pain in the left lumbar region. Imaging revealed a left kidney tumor that was hemorrhagic and necrotic on gross examination. At histology, proliferation of 99% of atypical epithelioid cells, staining for HMB-45 and Melan A was observed. Our patient had lung and right adrenal metastasis six months after surgery and succumbed one year later.

Conclusion: In summary, the pure form of EAML is extremely rare. When diagnosed, the risk of tumor progression is high, especially if other histological predictive parameters of progression are observed. In that case, active treatment should be undergone and patients should be closely monitored.