Angiomyolipoma with epithelial cysts (AMLEC): a rare but distinct variant of angiomyolipoma.
Angiomyolipoma with epithelial cysts (AMLEC): a rare but distinct variant of angiomyolipoma.
复制标题
DOI：
10.1186/1746-1596-2-11
复制
发表时间：
2007-03-21
影响因子：
2.6
通讯作者：
Parwani AV
中科院分区：
文献类型：
--
作者：
Armah HB;Yin M;Rao UN;Parwani AV
血管平滑肌脂肪瘤伴上皮囊肿（AMLEC）是一种新近发现的血管平滑肌脂肪瘤（AML）的囊性变异。迄今为止，2个病例系列报告了15例AMLEC病例。我们报告第16例，一名39岁女性。她的左肾肿瘤是偶然发现的。行肾部分切除术。在组织学上，肿瘤由三种成分组成：1）上皮囊肿内衬立方至鞋钉细胞; 2）致密的上皮下苗勒样AML间质，伴有混合性慢性炎症; 3）上皮下间质外血管畸形的肌肉型AML。免疫组化，上皮下基质染色最强烈的HMB-45和Melan-A，而肌肉为主的AML地区的平滑肌肌动蛋白和结蛋白染色最强烈。雌激素受体（ER）、孕激素受体（PR）和CD 10在上皮下基质中染色最强。囊肿衬里是阳性的泛细胞角蛋白，但阴性的HMB-45，Melan-A，ER，PR和CD 10。术后12个月，患者存活，无疾病证据，计划每年进行随访CT扫描。
Angiomyolipoma with epithelial cysts (AMLEC) is a recently described distinct cystic variant of angiomyolipoma (AML). To date 15 cases of AMLEC have been reported in 2 case series. We report the 16th case in a 39-year-old female. Her left kidney tumor was discovered incidentally. Partial nephrectomy was performed. Histologically, the tumor was composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) compact subepithelial mullerian-like AML stroma with admixed chronic inflammation; and 3) muscle-predominant AML with dysmorphic blood vessels exterior to the subepithelial stroma. Immunohistochemically, the subepithelial stroma stained most intensely with HMB-45 and Melan-A, whilst the muscle-predominant AML areas stained most intensely with smooth muscle actin and desmin. Estrogen receptor (ER), progesterone receptor (PR), and CD10 stained most intensely in the subepithelial stroma. The cyst lining was positive for pancytokeratin, but negative for HMB-45, Melan-A, ER, PR, and CD10. The patient is alive with no evidence of disease, 12 months postoperatively, and yearly follow-up CT scans are planned.
登录
查看更多内容
影响因子：
5.6
作者：
Cibas, ES;Goss, GA;Fletcher, CDM
通讯作者：
Fletcher, CDM
影响因子：
5.6
作者：
Petit, A;Castillo, M;Mallofré, C
通讯作者：
Mallofré, C
影响因子：
5.3
作者：
Green, AJ;Sepp, T;Yates, JRW
通讯作者：
Yates, JRW
影响因子：
5.6
作者：
Fine, SW;Reuter, VE;Argani, P
通讯作者：
Argani, P
影响因子：
5.6
作者：
Martignoni, G;Pea, M;Mariuzzi, GM
通讯作者：
Mariuzzi, GM