Malignant epitheloid angiomyolipoma of the kidney in a child treated with sunitinib, everolimus and axitinib

Abstract

The malignant variant of epithelioid angiomyolipoma (EAML) of the kidney is uncommon, extremely aggressive and behaves like a renal cell carcinoma. We present a case of a 12-year-old male with malignant EAML who was treated according to adult treatment protocols. To our knowledge, axitinib has not been used before in children. We conclude that adult protocols, in this rare case, could be safely used in rare childhood malignancies.

Fig. 1.

(A): Bilateral renal lesions evaluated as angiomyolipomas; (B): A giant mass originating from the left kidney and occupying the left abdominal cavity; (C) Residual giant mass occupying the left part of the abdomen; (D): Pleural effusion occupying the left hemithorax; (E): Regression in the size of the giant mass in the left abdominal cavity; (F): Progression in the size of the residual tumour; (G): Giant residual tumor; (H): Massive left-sided and minimal right-sided pleural effusion.

Fig .2.

(A): Epithelioid-polygonal cells, and varying degrees of nuclear atypia (arrow) (hematoxylin and eosin stain [H&E], ×100); (B): Sheets of epitheloid cells with moderate amounts of eosinophilic cytoplasm and vesicular nucleus with prominent nucleoli (arrow) (H&E, ×200); (C): The tumour cells showed positive reactivity for Melan-A (Melan A, ×100); (D): The tumour cells showed positive reactivity for HMB-45 (HMB-45, ×100).