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2 cases of angiomyolipomatosis of the kidneys are taken as the basis for presenting the symptoms, diagnosis and therapy of this rare disease. In one case there was also an angiomyolipomatous change in the retroperitoneal lymph nodes. In differential diagnosis this gave rise to a suspicion of metastasis or a malignant system disease. In another case there was a coincidence with cystic degeneration of the kidneys.
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