Angiomyolipoma of the kidney - Expanding disease spectrum demonstrated by 3 cases

We report 3 recent cases of angiomyolipoma of the kidney. Although generally regarded as a benign neoplasm, angiomyolipoma rarely behaves in an aggressive manner, producing complicated clinical courses leading to metastasis and death. The presence of epithelioid elements within the tumor can result in difficulty differentiating benign from malignant angiomyolipoma and differentiating this tumor from renal adenocarcinoma. The presence of lymph node involvement can cause difficulty in differentiating multicentric disease in lymph nodes from metastasis to lymph nodes. The presence of cytologic abnormalities in the primary tumor can result in difficulty in differentiating atypia in benign angiomyolipoma from malignant sarcomatous transformation of a benign lesion. The 3 cases reported show many of these problems. Criteria for predicting malignancy in epithelioid tumors and sarcomatous transformation are not well recognized because of the rarity of this entity. The typical immunophenotype of all types of angiomyolipoma (cytokeratin-negative and melanomarkers-positive) is very useful in diagnosis but does not help in the differentiation from renal adenocarcinoma at frozen section. We report the empiric use of Ki67 and p53 in these cases as adjuncts to clinical and histologic assessment in predicting behavior. High Ki67 expression was a feature of malignant epithelioid angiomyolipoma. Low levels of p53 expression were seen in the angiomyolipoma with sarcomatous transformation. Benign angiomyolipomas were consistently negative for both Ki67 and p53.