上皮样血管平滑肌脂肪瘤的临床病理特征

目的:探讨上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的临床病理学特征.方法:回顾性分析17例病理诊断为EAML的组织学形态、免疫组织化学特点、临床及随访资料.结果:男5例,女12例,年龄21~62(平均31.2)岁,其中14例发生于肾脏(3例伴有子宫平滑肌瘤,2例伴有宫内孕);2例发生于肝脏,1例发生于腹腔;肿瘤最大径3~15 cm,镜下瘤细胞呈上皮样巢、片状分布,可见围绕血管呈放射状排列的结构;细胞体积大,卵圆形、梭形,胞质丰富,异型明显,核分裂少见,其中2例伴有出血、坏死.免疫组织化学HMB-45,Melan-A,SMA阳性,vimentin,ER和PR表达率分别为41%(7/17),23%(4/17)以及35%(6/17).17例EAML均行肿瘤根治或肿瘤切除术,术后随访5~52个月,1例2年后肝肺转移死亡,其余16例均健在,无复发及转移.结论:EAML多发生于女性,可能与激素水平有关;肾脏最常见,其次为肝、腹腔;病理形态多样,免疫组织化学对其诊断有重要价值,手术效果好,有转移可能.

Clinicopathologic features of epithelioid angiomyolipoma

Objective: To investigate the clinicopathologic features of epithelioid angiomyolipoma (EAML). Methods: The morphological, immunohistochemical characteristics, clinical and follow-up data of 17 patients with EAML were retrospectively reviewed. Results: There were 5 males and 12 females of 17 cases, age ranged from 21 to 62 (average 31.2) years old, and 14 cases occurred in the kidney (3 cases with uterine leiomyoma and 2 cases with intrauterine pregnancy), 2 cases occurred in liver, 1 case occurred in abdominal cavity. The tumors maximum diameter was 3 to 15 cm. Microscopically, the tumor cells arranged in nests or sheets, cuff-shaped around the blood vessels. The cells were larger, ovoid or spindle, with abundant and eosinophilic cytoplasm, a big nucleus and obvious nucleoli, and a few mitotic figures. There were two cases with hemorrhage and necrosis of all. Immunohistochemistry showed that the tumor cells were positive for HMB-45, Melan-A and SMA. Vimentin, ER and PR positive rate respectively was 41% (7/17), 23% (4/17), 35% (6/17). All of them underwent radical surgery or surgical resection, and were followed up for 5-52 months after operation. Sixteen cases were alive, without recurrence or metastasis, while one case died of liver and lung metastases 2 years later. Conclusion: EAML mostly occurs in female, which may be associated with hormone levels. Most of them occur in kidney, then in liver and abdominal cavity. It has no specifications in the histopathologic features. Immunohistochemistry plays an important role in the differential diagnosis. Surgical treatment is the main method with a good prognosis, but there are possibility of recurrence or metastasis.