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Abstract：
Angiomyolipoma (AML) is a benign tumor that mainly occurs in the kidneys. Simultaneous involvement of the kidney and local regional lymph nodes is very rare and might be misdiagnosed as a metastasizing malignant cancer. In the present study, a 50-year-old woman was referred to our hospital after a routine health screening ultrasound. Sporadic multiple renal AML with lymph node involvement was suspected based on the clinical manifestations and radiologic features. Partial nephrectomy was performed and a para-inferior vena cava lymph node was removed. The pathologic results confirmed multiple AML with lymph node invasion. We also reviewed the English-language literature regarding renal AML with lymph node involvement. We found that middle-aged women were likely to develop this disease and that loin pain was the main presenting feature. Most patients had no history of tuberous sclerosis complex. Radical nephrectomy was the predominant treatment. No local recurrence or distant metastasis occurred in any patients after radical nephrectomy or partial nephrectomy. In conclusion, renal AML with lymph node involvement is rare but can occur in both patients with tuberous sclerosis complex and those with multiple sporadic AML. Partial nephrectomy should be the first-line treatment, after which further treatment is not necessary.
摘要：
血管平滑肌脂肪瘤（AML）是一种良性肿瘤，主要发生在肾脏。肾脏和局部淋巴结同时受累非常罕见，可能被误诊为转移性恶性肿瘤。在本研究中,一名50岁的妇女在接受常规健康筛查超声检查后被转诊到我们医院。根据临床表现和放射学特征，怀疑有淋巴结受累的散发性多发性肾脏AML。进行部分肾切除术，并切除下腔静脉旁淋巴结。病理结果证实多发性AML伴淋巴结浸润。我们还回顾了有关淋巴结受累的肾AML的英语文献。我们发现中年妇女很可能患上这种疾病，腰痛是主要的表现特征。大多数患者没有结节性硬化症病史。根治性肾切除术是主要的治疗方法。根治性肾切除术或部分肾切除术后无局部复发或远处转移。总之,伴有淋巴结受累的肾AML很少见,但在结节性硬化症患者和多发性散发性AML患者中均可发生.肾部分切除术应该是一线治疗，之后，不需要进一步治疗。
