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"Tuberous sclerosis complex (TSC) has several renal manifestations including angiomyolipomas (AML) and renal epithelial neoplasms. A bilateral giant renal AML is extremely rare. We report a case of giant bilateral AML and discuss the diagnosis and treatment of it. The 22-year-old man was admitted due to bilateral flank pain, gross hematuria, and abdominal fullness. He had history of epilepsy, mental retardation, and delayed development during childhood. He had angiofibroma on his face since 10 years ago. Abdominal CT and MRI revealed large lobulated heterogeneous mass with fatty content. Based on those findings, we diagnosed the patient with bilateral giant renal AML. We gave conservative management for the patient and planned to total nephrectomy on the left kidney if the continued bleeding occurred. AML associated with TSC occur more frequently as multiple lesions and grows to larger size than idiopathic AML. Bilateral giant AML, which is very rare, could be treated with conservative management if no significant hemorrhage occurred.

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Kompleks tuberous sklerosis memiliki beberapa manifestasi di ginjal meliputi angiomiolipoma dan neoplasma epithelial renalis. Angiomiolipoma bilateral besar sangatlah jarang dijumpai. Kami melaporkan kasus angiomiolipoma bilateral besar dan mendiskusikan diagnosis dan manajemen kasus tersebut. Laki-laki usia 22 tahun masuk ke rumah sakit karena nyeri pinggang, hematuria, dan rasa penuh di perut. Pasien memiliki riwayat epilepsy, retardasi mental, dan gangguan perkembangan saat masa kanak-kanak. Pasien didiagnosis memiliki angiofibroma pada wajah sejak 10 tahun yang lalu. CT scan dan MRI abdomen iddapatkan massa besar berlobus-lobus yang memiliki densiteas heterogen bersesuaian dengan densitas lemak. Berdasarkan penemuan tersebut, kami mendiagnosis pasien dengan angiomiolipoma bilateral besar. Dilakukan manajemen konserfatif dan direncanakan nefrektomi total pada ginjal kira apabila perdarahan sangat masif. Angiomiolipoma yang disertai kompleks tuberous sclerosis biasanya bersifat multiple dan dapat tumbuh sangat besar. Angiomiolipoma bilateral besar, yang kasusnya sangat jarang, dapat ditatalaksana dengan terapi konserfatif apabila tidak dijumpai perdarahan yang signifikan."