MULTICENTRIC AND LARGE ANGIOMYOLIPOMA OF THE KIDNEY, ADRENAL-GLAND AND PARAAORTIC LYMPH-NODES - CASE-REPORT OF A 9-YEAR-OLD BOY WITH TUBEROUS SCLEROSIS

The symptoms and therapy of a multicentric angiomyolipoma of the kidne y, adrenal gland, paraaortic lymph nodes and renal fat tissue in a 9-y ear-old boy with tuberous sclerosis are reported. Angiomyolipomas are benign mesenchymal tumours that often occur together with tuberous scl erosis. Renal cell carcinoma in an angiomyolipoma is rare. Multicentri city and lymph node involvement is not a sign of malignancy or meta st atic disease. Radiological characteristics in CT and plain X-ray may h elp in the diagnosis. Characteristically, the renal lesions are asympt omatic. Patients with incidental symptom-free angiomyolipoma should be followed. In other patients with pain in the loin, or when a solid tu mour cannot be confidently excluded, conservative surgery or nephrecto my should be performed.