PDF(Pubmed)
Abstract：
Renal angiomyolipomas (AMLs) were first described in the early 1900s by Gravitz, but it was not until 1951 that they were named renal AML. These kidney tumours are rare, occurring in 0.13%-0.44% of the population. These mesenchymal tumours are composed of smooth muscle-like, adipocyte-like and epithelioid cells. Depending on the predominant cell population, it can be further subclassified into classic, epithelioid and AML with epithelial cyst. A 32-year-old woman presented with mild, intermittent, epigastric and right upper quadrant abdominal pain. Abdominal ultrasound revealed an incidental lesion within the inferior vena cava (IVC). A CT scan showed a lesion within the left renal vein extending into the IVC with 40% narrowing and a fat-containing mass in the lower pole of the left kidney of 15 mm suggesting an AML. Thrombectomy was performed. The specimen resulted positive for classic variant renal AML. Initial diagnosis is centred on imagining studies, based in fatty tissue concentration. The AML expresses melanocytic markers. This helps differentiate from renal cell carcinoma. Although AML is considered a benign condition, there is evidence of malignant transformation. Active surveillance is recommended for lesions <4 cm. Nephron sparing surgery is the procedure of choice. Nephrectomy is recommended if there is a high probability of malignancy. Mammalian target of rapamycin (mTOR) inhibitors have been proposed to be an alternative treatment.
摘要：
肾血管平滑肌脂肪瘤（AMLs）在1900年代初由Gravitz首次描述，但直到1951年才被命名为肾AML。这些肾脏肿瘤很少见，发生在0.13%-0.44%的人口中。这些间充质肿瘤由平滑肌样组成，脂肪细胞样和上皮样细胞。取决于主要的细胞群体，它可以进一步细分为经典，上皮样和AML伴上皮囊肿。一名32岁的妇女表现得温和，间歇性,上腹部和右上腹腹痛。腹部超声显示下腔静脉（IVC）内的偶然病变。CT扫描显示左肾静脉内的病变延伸至IVC，狭窄40％，左肾下极有15毫米的含脂肪块，提示AML。进行血栓切除术。样本对经典变异型肾AML呈阳性。初步诊断以想象研究为中心，基于脂肪组织浓度。AML表达黑素细胞标记。这有助于区分肾细胞癌。尽管AML被认为是良性疾病，有恶变的证据.对于<4cm的病变，建议进行主动监测。保留肾单位的手术是选择的程序。如果恶性肿瘤的可能性很高，建议进行肾切除术。哺乳动物雷帕霉素靶(mTOR)抑制剂已被提出作为替代治疗。
