肝血管平滑肌脂肪瘤21例临床病理观察

目的：研究肝血管平滑肌脂肪瘤（HAML）的形态学特点。方法：收集21例HAML的临床相关资料；采用HE染色、特色染色、免疫组化、电镜等方法进行研究。结果：HAML患者的症状、体征及影响学有其特征，病理形态学上均显示有血管周上皮样细胞，血管有厚壁和薄壁两种，上皮样细胞分为4种形态，形态与免疫表型有一定的相关性，部分上皮样细胞浆PAS染色阳性且抗淀粉酶消化，电镜下可见类似前黑色素水体的特殊晶体样结构及密体。结论：HAML是一种好发于中青年女性的良性肿瘤，其特别为actin、HMB45（＋）。HAML因其特殊形态、及临床特征归入血管周上皮样细胞相关肿瘤。

Hepatic angiomyolipoma: A clinicopathological study of 21 cases

Objective To study the clinical and pathologic characteristics of hepatic angiomyolipoma (HAML). Methods The clinical and pathological data were collected, and routine sections, special staining, immunohistochemistry and electronic microscopy were used in 21 cases of HAML. Results All the cases had characteristics on images, symptoms and physical signs, and histologically showed perivascular epithelial cells (PECs), and thin and thick walls of vessels. PECs had four morphologic variants with the same immunophenotype, and characteristic structures under PAS staining and electronic microscope. Conclusions HAML is a special kind of PEC related benign tumor. Positive reaction for actin and HMB45 is a characteristic feature of HAML.