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  

Malignant-tendency giant renal angiomyolipoma extending into the inferior vena cava as a tumor thrombus(A case report and literature review)

作　　者：闵捷[1] 于德新[1] 张志强[1] 谢栋栋[1] 王毅[1] 张涛[1] 丁德茂[1] 陈磊[1] 邹慈[1] 

机构地区：[1]安徽医科大学第二附属医院泌尿外科,合肥230601

出　　处：《临床泌尿外科杂志》2013年第4期263-267,共5页Journal of Clinical Urology

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 of giant renal angiomy01ipoma with inferior vena cava tumor embolus was reported. Clinical manifestation, clinical and pathological characteristics, diagnosis and differential diagnosis , surgical treatment of angiomyolipoma were analyzed and discussed by literatures searching and reviewing. Resuit ： The patient presented to the urology department with bilateral lower extremity edema. CT and MRI showed renal angiomyolipoma with tumor thrombus extending to the leve of Mayo III. Right radical nephrectomy with inferior vena cava thrombectomy was performed with no severe complication. Histopathological examination demonstrated that right renal angiomyolipoma with tumor thrombus. Conclusion.. Renal angiomyolipomas（AMLs） are mostly benign mesenchymal tumors that occur either sporadically or associated with tuberoussclerosis. Rarely, the tumor may extend into the renal vein and inferior vena cava indicating aggres- sive behaviors. Malignant potential is, to our knowledge,considered to be the characteristics of the so-called epithelioid angiomyolipomas（EAMLs）, which can only be demonstrated by immunohistochemistry examination. Surgical treatment of angiomyolipoma with IVC thrombus is the only potentially curative treatment. Our finding em- phasizes the importance of strict preoperative evaluation, skillful operation, fine histological examination and closer followup to lead a better prognosis.

关 键 词：恶性倾向肾血管平滑肌脂肪瘤 下腔静脉 肾肿瘤 

分 类 号：R737.11[医药卫生—肿瘤]