Epithelioid angiomyolipoma of the kidney - A report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component
Epithelioid angiomyolipoma of the kidney - A report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component
复制标题
DOI：
10.1097/00000478-199710000-00001
复制
发表时间：
1997-10-01
影响因子：
5.6
通讯作者：
Young, RH
中科院分区：
文献类型：
--
作者：
Eble, JN;Amin, MB;Young, RH
我们报告了5例血管平滑肌脂肪瘤，患者年龄在20 ~ 48岁（平均36岁），主要成分为上皮样平滑肌细胞。肿瘤往往造成的问题，在诊断，特别是在与肾细胞癌的区别。2例患者患有结节性硬化症。随访5年以上的2例患者均健在。上皮样平滑肌细胞典型地形成片状，在两个肿瘤中被透明索穿过。上皮样细胞从中等大小、多角形、细胞核轻度多形性、胞浆嗜酸性到巨大细胞、核仁明显。出血，坏死，泡沫状巨噬细胞集群存在于三个肿瘤。其中两个肿瘤很容易找到核分裂像，但在其他肿瘤中则不存在。两个肿瘤中存在明显的典型血管平滑肌脂肪瘤成分。其他仅包含散在的厚壁血管或少量脂肪细胞，提示典型的血管平滑肌脂肪瘤。所有肿瘤的低或高分子量细胞角蛋白或上皮膜抗原均为阳性。4例肿瘤上皮样区均检测到肌动蛋白。与gp 100-cl基因产物相关的黑素瘤相关抗原HMB-45和HMB-50存在于所有肿瘤中。另一种黑色素瘤相关抗原CD 63（NKI/C3）也存在于所有肿瘤中，这一发现表明血管平滑肌脂肪瘤与黑色素瘤有共同的特征，而不仅仅是前黑色素体结构。
We report five angiomyolipomas with a prominent component of epithelioid smooth muscle cells that occurred in patients from 20 to 48 (mean, 36) years of age. The tumors often posed problems in diagnosis, particularly with regard to distinction from renal cell carcinoma. Two patients had tuberous sclerosis. Two patients with more than 5 years' follow-up are alive and well. The epithelioid smooth muscle cells typically formed sheets that in two tumors were traversed by hyaline cords. The epithelioid cells ranged from medium sized and polygonal with slightly pleomorphic nuclei and eosinophilic cytoplasm to giant cells with prominent nucleoli. Hemorrhage, necrosis, and clusters of foamy macrophages were present in three tumors. Mitotic figures were easy to find in two of the tumors, but they were absent in the others. Obvious elements of typical angiomyolipoma were present in two tumors. The others contained only scattered, thick-walled blood vessels or a few fat cells suggestive of typical angiomyolipoma. None of the tumors was positive for low-or high-molecular-weight cytokeratins or epithelial membrane antigen. Actin was detected in the epithelioid areas in four tumors. Melanoma-associated antigens related to the gp100-cl gene product, HMB-45 and HMB-50, were present in all the tumors. Another melanoma-associated antigen, CD63 (NKI/C3), also was present in all the tumors, a finding suggesting that angiomyolipoma has features in common with melanoma beyond premelanosomal structures.