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经腹腔镜部分肝切除术治疗的长期逐渐增大的多囊性胆管错构瘤

Multicystic biliary hamartoma with long-term gradual enlargement treated by laparoscopic partial hepatectomy.

作者信息

Nishiwada Satoshi, Tanaka Tetsuya, Kirihataya Yuki, Takei Takeshi, Sadamitsu Tomomi, Takano Masato, Sawai Masayoshi, Yoshimura Atsushi

机构信息

Department of Surgery, Minami-Nara General Medical Center, 8 - 1 Fukugami Oyodo-cho, Yoshino, Nara, 638 - 8551, Japan.

Department of Pathology, Minami-Nara General Medical Center, 8 - 1 Fukugami Oyodo-cho, Yoshino, Nara, 638 - 8551, Japan.

出版信息

Clin J Gastroenterol. 2025 Jun;18(3):527-534. doi: 10.1007/s12328-025-02124-z. Epub 2025 Apr 5.

Abstract

Multicystic biliary hamartoma (MCBH) is an extremely rare liver tumor characterized by a well-circumscribed, multicystic honeycomb appearance on imaging. Herein, we report a case of long-term gradually enlarging MCBH that underwent laparoscopic hepatectomy. A 69-year-old man presented with mildly elevated carcinoembryonic antigen levels and underwent computed tomography (CT) 11 and 5 years ago, at which time the physicians did not note any particular abnormal findings. The current contrast CT for screening demonstrated a cystic lesion in segment 2 of the liver. A retrospective review of CT images showed that the cystic lesion had gradually increased over time. The patients successfully underwent laparoscopic partial hepatectomy and recovered without any complications. The surgically resected specimen grossly presented an aggregated nodule of small cysts. Pathological findings showed multiple cysts of various sizes covered with cuboidal cells without dysplasia resembling bile duct epithelium, with lumens containing a stone component consisting of brown bile. Around the cyst, there was vascular fibrous tissue, smooth muscle bundles, normal hepatocytes, and chronic inflammation. Based on these findings, we finally diagnosed MCBH. Although MCBH is histologically classified as a benign tumor, physicians may have to pay careful attention to the potential for enlargement in actual clinics.

摘要

多囊性胆管错构瘤(MCBH)是一种极其罕见的肝脏肿瘤,在影像学上表现为边界清晰的多囊性蜂窝状外观。在此,我们报告一例长期逐渐增大的MCBH病例,该病例接受了腹腔镜肝切除术。一名69岁男性癌胚抗原水平轻度升高,11年前和5年前接受了计算机断层扫描(CT)检查,当时医生未发现任何特殊异常发现。此次用于筛查的增强CT显示肝脏2段有一个囊性病变。对CT图像的回顾性分析显示,该囊性病变随时间逐渐增大。患者成功接受了腹腔镜部分肝切除术,术后恢复良好,无任何并发症。手术切除标本肉眼可见为小囊肿聚集形成的结节。病理结果显示多个大小不一的囊肿,被立方体细胞覆盖,无发育异常,类似胆管上皮,管腔内含有由棕色胆汁组成的结石成分。囊肿周围有血管纤维组织、平滑肌束、正常肝细胞和慢性炎症。基于这些发现,我们最终诊断为MCBH。尽管MCBH在组织学上被归类为良性肿瘤,但在实际临床中医生可能仍需密切关注其增大的可能性。

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