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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 report a case of giant renal angiomyolipoma combined with vena cava tumor thrombus. Through the literature review, to explore the diagnosis and treatment of malignant renal angiomyolipoma (AML). Methods: One case of AML complicated with inferior vena cava tumor thrombus was reported and analyzed. The clinical manifestations, clinical and pathological features, diagnosis and differential diagnosis and surgical treatment of AML with malignant tendency were analyzed and discussed through literature review. Results: The patients underwent both CT and MRI examination of both lower extremity swollen, suggesting that AML with MayoIII posterior hepatic vena cava suppository. Radical nephrectomy and vena cava tumor thrombectomy, postoperative recovery was good. Histopathology confirmed as AML and intravenous tumor emboli. CONCLUSIONS: AML associated with tuberculous sclerosis complex (TSC) is usually a benign tumor of mesenchymal origin. Rarely, the tumor is aggressive when it invades the renal vein or inferior vena cava and forms a tumor plug. This malignant potential is considered to be a biological property of epithelial-like angiomyolipoma (EAMLs). Surgical resection of the tumor and removal of the tumor suppository are the only effective ways to treat this type of AML. Our research emphasizes rigorous preoperative evaluation, careful intraoperative procedures, careful histopathological examination and close postoperative follow-up in order to achieve a better prognosis.