Park Hang Kyu, Zhang Shaozeng, Wong Michael K K, Kim Hyung L
Department of Urologic Oncology, Roswell Park, Cancer Institute, Buffalo, New York 14263, USA.
Int J Urol. 2007 Jan;14(1):21-5. doi: 10.1111/j.1442-2042.2006.01665.x.
Epithelioid angiomyolipomas (AML) of the kidney are malignant tumors with aggressive clinical behavior.
We reviewed cases of epithelioid AML recently diagnosed at our institution to highlight the spectrum of clinical presentations.
In all cases, renal lesions seen on computed tomography were suspicious for renal cell carcinoma (RCC). Histologically, these tumors can resemble RCC. The diagnosis of epithelioid AML was established by positive staining for melanoma and smooth muscle cell markers, and presence of perivascular epithelioid cells. One patient presented with a renal tumor extending into the inferior vena cava to the level of the hepatic veins. Two patients developed recurrent, metastatic disease following nephrectomy. One patient with tuberous sclerosis and multiple, bilateral AML developed an enhancing renal tumor that did not contain any fat densities. A partial nephrectomy was performed and pathology revealed epithelioid AML adjacent to conventional AML.
These tumors are distinguished from RCC by positive immunostaining for melanoma markers and smooth muscle cell markers. They resemble conventional RCC on imaging. Epithelioid AML may be locally aggressive and metastasize.
肾上皮样血管平滑肌脂肪瘤（AML）是具有侵袭性临床行为的恶性肿瘤。
我们回顾了近期在我院诊断的上皮样AML病例，以突出临床表现的范围。
在所有病例中，计算机断层扫描显示的肾脏病变疑似肾细胞癌（RCC）。组织学上，这些肿瘤可能类似于RCC。上皮样AML的诊断通过黑色素瘤和平滑肌细胞标志物的阳性染色以及血管周上皮样细胞的存在来确立。一名患者的肾肿瘤延伸至下腔静脉至肝静脉水平。两名患者在肾切除术后出现复发、转移性疾病。一名患有结节性硬化症和多发双侧AML的患者出现了一个强化的肾肿瘤，其中不含任何脂肪密度。进行了部分肾切除术，病理显示上皮样AML与传统AML相邻。
这些肿瘤通过黑色素瘤标志物和平滑肌细胞标志物的阳性免疫染色与RCC相鉴别。它们在影像学上类似于传统RCC。上皮样AML可能具有局部侵袭性并发生转移。
