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结节性硬化症患者肾血管平滑肌脂肪瘤的演变

The evolution of renal angiomyolipomas in patients with tuberous sclerosis.

作者信息

van Baal J G, Smits N J, Keeman J N, Lindhout D, Verhoef S

机构信息

Department of Surgery, Sint Lucas Hospital, Amsterdam, The Netherlands.

出版信息

J Urol. 1994 Jul;152(1):35-8. doi: 10.1016/s0022-5347(17)32809-4.

DOI:10.1016/s0022-5347(17)32809-4
PMID:8201681
Abstract

In 1986, 23 patients with renal angiomyolipomas as part of tuberous sclerosis were assessed by ultrasonography. In 1991, 20 patients in this group were reexamined with special attention paid to the renal pathological condition. Ultrasonography was performed by the same radiologist who performed the examination in 1986. Of 20 patients 7 had severe hemorrhage necessitating hospital admission (5 had a renal lesion larger than 3.5 cm. in diameter). In 2 patients the exact diameter of the renal angiomyolipomas could not be determined and they underwent nephrectomy. Three patients underwent successful selective embolization of the bleeding angiomyolipoma. One patient died. The hemorrhage resolved spontaneously in 1 patient and treatment was not feasible. In 4 patients the lesions increased in size between 1986 and 1991. Based on these results there is a relationship between the size of the angiomyolipomas and the risk of bleeding. Renal angiomyolipomas larger than 3.5 cm. in diameter have a substantial risk for severe hemorrhage. Some angiomyolipomas show progression. Periodic followup is mandatory every 6 months. For angiomyolipomas larger than 3.5 cm. in diameter an aggressive approach is advised. Selective embolization is the initial method of choice.

摘要

1986年,对23例患有作为结节性硬化症一部分的肾血管平滑肌脂肪瘤的患者进行了超声检查。1991年,对该组中的20例患者进行了复查,特别关注肾脏病理状况。超声检查由1986年进行检查的同一位放射科医生进行。20例患者中,7例发生严重出血,需要住院治疗(5例肾病变直径大于3.5厘米)。2例患者肾血管平滑肌脂肪瘤的确切直径无法确定,因此接受了肾切除术。3例患者成功接受了出血性血管平滑肌脂肪瘤的选择性栓塞治疗。1例患者死亡。1例患者出血自行缓解,无法进行治疗。4例患者的病变在1986年至1991年间增大。基于这些结果,血管平滑肌脂肪瘤的大小与出血风险之间存在关联。直径大于3.5厘米的肾血管平滑肌脂肪瘤有严重出血的重大风险。一些血管平滑肌脂肪瘤会进展。必须每6个月进行定期随访。对于直径大于3.5厘米的血管平滑肌脂肪瘤,建议采取积极的治疗方法。选择性栓塞是首选的初始治疗方法。

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