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肾错构瘤(血管平滑肌脂肪瘤)与结节性硬化症

Renal hamartoma (angiomyolipoma) and the tuberous sclerosis complex.

作者信息

Pacis A B, Norman C H

出版信息

J Natl Med Assoc. 1979 Jan;71(1):55-7.

Abstract

Renal hamartoma is found in 40 to 80 percent of patients with tuberous sclerosis. Microscopic demonstration of fat in the tissues of the mass is felt to be the most reliable diagnostic criterion of hamartoma.Characteristically, the angiographic appearance demonstrates a large, dilated feeding vessel passing through the mass with multiple, multisacculated aneurysmal dilatations appearing like bunches of grapes. There is a delicate neovascularity without A-V shunting and an onion-peel or whorl-like appearance in the venous phase.This case is presented to point out the close association of renal hamartoma and tuberous sclerosis and the need to search for renal hamartoma when the diagnosis of tuberous sclerosis is made.

摘要

肾错构瘤见于40%至80%的结节性硬化症患者。在肿块组织中显微镜下显示脂肪被认为是错构瘤最可靠的诊断标准。其特征性表现为,血管造影显示一条粗大、扩张的供血血管穿过肿块,伴有多个多囊状动脉瘤样扩张,形似葡萄串。有纤细的新生血管,无动静脉分流,静脉期呈葱皮样或漩涡样表现。本文报告此病例是为了指出肾错构瘤与结节性硬化症的密切关联,以及在诊断结节性硬化症时需筛查肾错构瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0de3/2537241/c1dc6155d3e4/jnma00021-0058-a.jpg

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