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The angiomyolipoma is a rare tumor of the kidney with benign clinical evolution which can be found in more than half of the female patients with tuberous sclerosis. An uncommon case of renal angiomyolipoma with extracapsular extension, inter-aortocaval lymph node involvement and pulmonary lymphangiomyomatosis with a clinical history of spontaneous recurrent pneumothorax but without the neurological signs of the tuberous sclerosis, is described. The natural history of this disease which has various clinical forms of presentations is still unclear. The role of the lymph-node dissection in the surgical treatment of this tumor is discussed.
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