Mongha R, Bansal P, Dutta A, Das R K, Kundu A K
Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata, India.
Indian J Cancer. 2008 Apr-Jun;45(2):64-6. doi: 10.4103/0019-509x.41773.
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.
我们报告一例结节性硬化症患者，其患有双侧肾血管平滑肌脂肪瘤和肝血管平滑肌脂肪瘤，右肾血管平滑肌脂肪瘤发生自发性腹膜后出血（温德利希综合征）。尝试了保留肾单位的方法，但右肾未能保住。肝血管平滑肌脂肪瘤采取保守治疗。在约10%的肾血管平滑肌脂肪瘤病例中发现了温德利希综合征。肝血管平滑肌脂肪瘤是肝脏的一种良性间叶性肿瘤，文献中鲜有报道。
