Abstract
Tuberous sclerosis complex (TSC, tuberous sclerosis, Burneville-Pringle disease) is a multi-organ syndrome manifested by the development of benign and malignant tumours with different localizations. The main cause of death in patients with TSC is the progression of subependymal giant cell astrocytomas in the brain, angymyolipomas in the kidneys and the development of lymphangiomyomatosis. Although renal cell carcinoma is relatively rare (only 2–4% of patients with TSC), it nevertheless contributes significantly to mortality. Extrarenal manifestations of TSC in the chest, abdomen and central nervous system help in the diagnosis of TSC. The paper presents the data on the pathogenesis, clinical picture, diagnostic criteria, current approaches to therapy and results of our own observation of patient with TSC.
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