[Multilocular, giant angiomyolipoma of the kidney, adrenal gland and para-aortic lymph nodes. Case report of a 9-year-old boy with tuberous sclerosis].

The symptoms and therapy of a multicentric angiomyolipoma of the kidney, adrenal gland, paraaortic lymph nodes and renal fat tissue in a 9-year-old boy with tuberous sclerosis are reported. Angiomyolipomas are benign mesenchymal tumours that often occur together with tuberous sclerosis. Renal cell carcinoma in an angiomyolipoma is rare. Multicentricity and lymph node involvement is not a sign of malignancy or metastatic disease. Radiological characteristics in CT and plain X-ray may help in the diagnosis. Characteristically, the renal lesions are asymptomatic. Patients with incidental symptom-free angiomyolipoma should be followed. In other patients with pain in the loin, or when a solid tumour cannot be confidently excluded, conservative surgery or nephrectomy should be performed.