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Wunderlich's syndrome with hepatic angiomyolipoma in tuberous sclerosis
1Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata, India. riteshmongha2001@rediffmail.com
Abstract:
We present a patient of tuberous sclerosis complex with bilateral renal angiomyolipoma and hepatic angiomyolipoma with spontaneous retroperitoneal hemorrhage from right angiomyolipoma (Wunderlich's syndrome). Nephron preserving approach was tried but the right kidney could not be salvaged. Hepatic angiomyolipoma was managed conservatively. Wunderlich's syndrome has been found in around 10% of cases of renal angiomyolipomas. Hepatic Angiomyolipoma is a benign mesenchymal neoplasm of the liver which is rarely reported in literature.
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