恶性倾向肾巨大血管平滑肌脂肪瘤伴下腔静脉瘤栓1例报告并文献复习

发表时间：2014-07-07 浏览次数：1164次

引用：

闵捷，于德新，张志强等.恶性倾向肾巨大血管平滑肌脂肪瘤伴下腔静脉瘤栓1例报告并文献复习[J].临床泌尿外科杂志.2013,28(4):263-320.

关键词：

恶性倾向肾血管平滑肌脂肪瘤；下腔静脉；肾肿瘤；

作者：

闵捷，于德新，张志强，谢栋栋，王毅，张涛，丁德茂，陈磊，邹慈

作者单位：

安徽医科大学第二附属医院泌尿外科；

出版年份：

2013

期刊页数：

263-320

收录者：

知网

摘要：

目的:报告1例巨大肾血管平滑肌脂肪瘤合并腔静脉瘤栓病例。通过文献复习,探讨恶性倾向肾血管平滑肌脂肪瘤(renal angiomyolipoma,AML)的诊断与治疗。方法:报道和分析1例巨大AML伴下腔静脉瘤栓的病例资料,并通过查阅文献,分析和探讨具有恶性倾向的AML的临床表现、临床与病理学特性、诊断与鉴别诊断及外科治疗。结果:患者以双下肢肿胀就诊并行CT及MRI检查,提示AML伴肝后下腔静脉瘤栓(MayoIII)形成。行根治性肾切除和腔静脉瘤栓取出术,术后恢复良好。组织病理学证实为AML及静脉内肿瘤栓子。结论:散发的或结节性硬化症(tuberous sclerosis complex,TSC)相关的AML通常为间叶组织来源的良性肿瘤。罕见的,当瘤体侵犯肾静脉或下腔静脉并形成瘤栓时具有侵袭性。这种恶性潜能被认为是上皮样血管平滑肌脂肪瘤(EAMLs)的生物学特性,手术切除肿瘤并取出瘤栓是可能治疗这类AML的惟一有效方法。我们的研究强调了严密的术前评估、精细的术中操作、审慎的组织病理学检查及密切的术后随访,以求获得更好的预后。 Objective: To present an uncommon giant renal angiomyolipoma with embolus in the Inferior vena cava and review literatures,evaluating its diagnosis and management. Method: One case cf giant renal:ngiornyoli- poma with inferior vena cava tumor embolus was reported. Clinical manifestation, clinical and pathological charac- tensucs.diagnosis and differential diagnosis.surgical treatment of angiomyolipoma vaere analyzed and discussed by iiteratures searching and reviewing. Result:The patient presented to the urology department with bilateral lower extremity edema. CT and MRI showed renal angiomyolipcma with tumor thrombus extending:。the love of Mayo 11I. Right radical nephreetomy with inferior、ena eava thrombectomy was performed with no severe complication Histopathological examination demonstrated that right renal angiomyolipoma with tumor thrombus. Conclusion; Renal angiomyoiipomas(AMI，)are mostly benign mesenchymal tunuors that occur either sporadic川y or associated with tuberousselcrosis. Rarely, the tumor may extend into the renal vein and inferior vena cava indicating aggres- live behaviors. Malignant potential is，to our knowledge, considered to be the characteristics of the、。一called cpi- thelioid angiomyolipomas;E八III、)which can only be demonstrated by immunoh istochemistry examination. Sur- gical treatment of angicmyolipoma with I'tO: thrombus]、the only potentially curative treatment. C)ur finding em phasizes the importance of strict preoperative。、一。luation.skillful operation,fine histological examination and closer follow-up to lead a better prognosis.

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