Nagata S, Aishima S, Fukuzawa K, Takagi H, Yonemasu H, Iwashita Y, Kinoshita T, Wakasugi K
Department of Surgery, Nakabaru Hospital, Fukuoka, Japan.
J Clin Pathol. 2008 Jun;61(6):777-80. doi: 10.1136/jcp.2007.054684.
An unusual primary adenomatoid tumour arising in the normal liver is described. Hepatectomy was performed, and the patient is alive and free of disease 1 year postsurgery. Grossly, the tumour showed a haemorrhagic cut surface with numerous microcystic structures. Histological examination revealed cystic or angiomatoid spaces of various sizes lined by cuboidal, low-columnar, or flattened epithelioid cells with vacuolated cytoplasm and round to oval nuclei. The epithelioid cells were entirely supported by proliferated capillaries and arteries together with collagenous stroma. Immunohistochemical studies showed that the epithelioid cells were strongly positive for a broad spectrum of cytokeratins (AE1/AE3, CAM5.2, epithelial membrane antigen and cytokeratin 7) and mesothelial markers (calretinin, Wilms' tumour 1 and D2-40). These cells were negative for Hep par-1, carcinoembryonic antigen, neural cell adhesion molecule, CD34, CD31 and HMB45. Atypically, abundant capillaries were observed; however, the cystic proliferation of epithelioid cells with vacuoles and immunohistochemical profile of the epithelioid element were consistent with hepatic adenomatoid tumour.
本文描述了一例发生于正常肝脏的罕见原发性腺瘤样肿瘤。患者接受了肝切除术，术后1年存活且无疾病复发。大体上，肿瘤切面呈出血性，有众多微囊结构。组织学检查显示，肿瘤内有大小不一的囊性或血管瘤样腔隙，内衬立方状、低柱状或扁平的上皮样细胞，细胞质呈空泡状，细胞核圆形至椭圆形。上皮样细胞完全由增生的毛细血管、动脉以及胶原性间质支持。免疫组织化学研究表明，上皮样细胞对多种细胞角蛋白（AE1/AE3、CAM5.2、上皮膜抗原和细胞角蛋白7）和间皮标志物（钙视网膜蛋白、肾母细胞瘤1和D2-40）呈强阳性。这些细胞对肝细胞抗原-1、癌胚抗原、神经细胞黏附分子、CD34、CD31和HMB45呈阴性。非典型的是，观察到大量毛细血管；然而，具有空泡的上皮样细胞的囊性增生以及上皮样成分的免疫组织化学特征与肝腺瘤样肿瘤相符。
