OBJECTIVE: To present two patients with the tuberous sclerosis complex in whom giant angiomyolipomas (AMLs) were managed conservatively for >20 years, as large (>4 cm) symptomatic AMLs are generally treated with embolization or nephrectomy because of the risks of haemorrhage. PATIENTS AND METHODS: The first patient initially presented with bilateral renal AMLs replacing >70% of his parenchymal volume, and a large left renal cyst. Since presentation he was hospitalized three times, but has had no transfusions or procedures to treat his AMLs. His renal function has remained stable during this 21-year interval. The second patient initially presented with bilateral renal AMLs that were not amenable to nephron-sparing surgery. After removing her nonfunctioning left kidney, the 24-cm AML in her right kidney was managed conservatively for >20 years, during which she had 44 transfusions and 11 hospitalizations before uncomplicated right nephrectomy and subsequent need for haemodialysis. CONCLUSION: Conservative management of AMLs can preserve renal function for >20 years in patients with tuberous sclerosis at high risk of end-stage renal disease.
OBJECTIVE: To present two patients with the tuberous sclerosis complex in whom giant angiomyolipomas (AMLs) were managed conservatively for >20 years, as large (>4 cm) symptomatic AMLs are generally treated with embolization or nephrectomy because of the risks of haemorrhage. PATIENTS AND METHODS: The first patient initially presented with bilateral renal AMLs replacing >70% of his parenchymal volume, and a large left renal cyst. Since presentation he was hospitalized three times, but has had no transfusions or procedures to treat his AMLs. His renal function has remained stable during this 21-year interval. The second patient initially presented with bilateral renal AMLs that were not amenable to nephron-sparing surgery. After removing her nonfunctioning left kidney, the 24-cm AML in her right kidney was managed conservatively for >20 years, during which she had 44 transfusions and 11 hospitalizations before uncomplicated right nephrectomy and subsequent need for haemodialysis. CONCLUSION: Conservative management of AMLs can preserve renal function for >20 years in patients with tuberous sclerosis at high risk of end-stage renal disease.
Authors: Sara Catarino Santos; Liliana Duarte; Fernando Valério; Júlio Constantino; Jorge Pereira; Carlos Casimiro Journal: Am J Case Rep Date: 2017-12-08