Maruyama Shigeo, Hirayama Chisato, Yamamoto Satoru, Inoue Masayuki, Umeki Kensuke, Maeta Yoshihiko, Konishi Ichiro, Suzuki Kazunori, Kajitani Shinji, Abe Juro, Kishimoto Hiroyuki, Horie Yasushi
Department of Internal Medicine, Saiseikai Gotsu General Hospital, Shimane, Japan.
J Gastroenterol. 2003;38(6):593-7.
Hepatobiliary cystadenoma was suggested to be uncommon and it is often difficult to make a differential diagnosis. We report a case of a 65-year-old woman who presented with changes in the structure of a cyst that had been observed for the previous 10 years. Diagnostic imaging revealed a 7-cm-diameter cystic lesion with internal septations and papillary projections in her liver. All laboratory test results were normal; however, cystic fluid carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 were raised, at 160 ng/ml andover 200,000 U/ml, respectively. Owing to changes in the structure of the cyst and the difficulty of differential diagnosis from cystadenocarcinoma, a complete surgical excision was performed. The histological findings indicated that the tumor consisted of a multilocular cyst lined by glandular cells (with cuboidal or tall columnar cystoplasm), which were immunohistochemically positive for cytokeratin, CEA, epithelial membrane antigen, and CA 19-9. The underlying stroma was composed of proliferating primitive spindle cells which were immunoreactive for vimentin, alpha-smooth muscle actin, muscle-specific actin, and desmin, and resembled ovarian stroma. From these findings, this tumor was diagnosed as hepatobiliary cystadenoma with mesenchymal stroma. Even though the tumor was previously diagnosed as a simple liver cyst, it was necessary to pay special attention to the changes in the structure of the cyst, using ultra sonography and/or computed tomography, bearing in mind hepatobiliary cystadenoma with mesenchymal stroma. The malignant potential of this tumor is stressed, and complete surgical resection is the recommended therapy.
肝内胆管囊腺瘤被认为较为罕见，且往往难以进行鉴别诊断。我们报告一例65岁女性病例，该患者出现了一个已观察10年的囊肿结构变化。诊断性影像学检查显示其肝脏有一个直径7厘米的囊性病变，内部有分隔及乳头状突起。所有实验室检查结果均正常；然而，囊液癌胚抗原（CEA）和糖类抗原（CA）19-9升高，分别为160 ng/ml和超过200,000 U/ml。由于囊肿结构变化以及与囊腺癌鉴别诊断困难，遂进行了完整的手术切除。组织学检查结果表明，肿瘤由内衬腺细胞（立方或高柱状细胞质）的多房囊肿组成，这些腺细胞细胞角蛋白、CEA、上皮膜抗原和CA 19-9免疫组化呈阳性。其下方的间质由增殖的原始梭形细胞组成，波形蛋白、α-平滑肌肌动蛋白、肌特异性肌动蛋白和结蛋白免疫反应阳性，类似卵巢间质。根据这些发现，该肿瘤被诊断为伴有间质性间质的肝内胆管囊腺瘤。尽管该肿瘤之前被诊断为单纯性肝囊肿，但鉴于伴有间质性间质的肝内胆管囊腺瘤，有必要通过超声和/或计算机断层扫描特别关注囊肿结构的变化。强调了该肿瘤的恶性潜能，建议采用完整的手术切除治疗。
