Angiomyolipomas, renal manifestation of tuberous sclerosis complex in 17-year-old boy--a case report.

Ann Acad Med Stetin 2008 ;54(2):160-5

Klinika Pediatrii, Hematologii i Onkologii Dzieciqcej Pomorskiej Akademii Medycznej w Szczecinie ul. Unii Lubelskiej 1, 71-252 Szczecin.

Introduction: Tuberous sclerosis complex (TSC) is a genetic disorder characterised by lesions affecting brain, skin, eyes and internal organs--kidneys, heart, liver and lungs. Renal involvement in individuals with TSC is common and potentially serious, includes angiomyolipomas and cystic lesions. There may also be an increased risk of renal cell carcinoma.

Case Report: A case of 17-year-old boy with delayed diagnosis of tuberous sclerosis complex with angiomyolipomas and cysts of the kidneys has been described.

Conclusion: The authors would like to underlina a very rare incidence of tuberous sclerosis complex and associated renal angiomyolipomas in children, the possibility to maintain normal kidney function for prolonged time despite intensified renal pathology, the need for periodic abdominal imaging and significantly delayed diagnosis in the described case.

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May 2009
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