Sharma Sharad, Dean Amanda G, Corn Ayumi, Kohli Vivek, Wright Harlan I, Sebastian Anthony, Jabbour Nicolas
Nazih Zuhdi Transplant Institute, 3300 North West Expressway, Oklahoma 73112, USA.
Hepatobiliary Pancreat Dis Int. 2008 Dec;7(6):581-9.
Ciliated foregut cysts of the liver are rare, with only 96 cases diagnosed since the first description in 1857. They are being increasingly diagnosed recently; the majority of the cases have been reported in the last 15 years. Although they bear a close resemblance to the simple cyst of the liver which has essentially a benign course, ciliated hepatic foregut cysts (CHFCs) can progress to malignancy with devastating consequences. It is imperative that this group of conditions be diagnosed and treated adequately.
This review includes discussion of the data from all the 96 reported cases from English and non-English literature. Analysis of the incidence rates, embryogenesis, growth, clinical features, risk of malignancy and the prognosis are highlighted systematically. The roles of various diagnostic modalities including ultrasound, CT, MRI, fine needle aspiration cytology (FNAC), immunohistochemistry and surgery are further discussed.
The mean age of patients with CHFC was 48+/-12 years. The male/female ratio was 1.1:1. The majority of patients with CHFC (62%) were asymptomatic, and the common mode of presentation was right upper abdominal pain. The cysts occurred in the left lobe in 51 patients, with sole location in segment IV in 44, and in the right lobe in 26. The average size of the cysts was 3.6+/-2.12 cm. The majority of the cysts were unilocular, and only 7 cases were multilocular. Cyst contents were described as viscous or mucinous in 73 patients, whereas bilious fluid was noted in 3. Large cysts having squamous carcinoma were cited in 3 patients, and 2 had extensive squamous metaplasia without malignancy. Others had benign histopathology.
Clinicians have become increasingly aware of CHFC. Imaging alone is not diagnostic per se, but when considered in the context of the global picture does provide important clues to the diagnosis. FNAC is diagnostic by the presence of the ciliated columnar aspirate but lacks sensitivity. Infantile presentation is usually accompanied by biliary communication and mandates a different surgical approach. The demonstration of malignant transformation in 3 cases and its fatal course emphasizes the need for surgical resection in all cases once the diagnosis is made.
肝脏纤毛前肠囊肿罕见，自1857年首次描述以来，仅确诊96例。近年来其诊断率不断上升；大多数病例是在过去15年中报告的。尽管它们与本质上病程良性的肝单纯囊肿极为相似，但纤毛性肝前肠囊肿（CHFC）可进展为恶性，后果严重。必须对这组疾病进行充分的诊断和治疗。
本综述讨论了英文和非英文文献中所有96例报告病例的数据。系统地重点分析了发病率、胚胎发生、生长、临床特征、恶性风险和预后。进一步讨论了包括超声、CT、MRI、细针穿刺细胞学检查（FNAC）、免疫组化和手术等各种诊断方法的作用。
CHFC患者的平均年龄为48±12岁。男女比例为1.1:1。大多数CHFC患者（62%）无症状，常见表现为右上腹疼痛。囊肿发生在左叶51例，仅位于IV段44例，右叶26例。囊肿平均大小为3.6±2.12 cm。大多数囊肿为单房，仅7例为多房。73例患者的囊肿内容物描述为粘性或粘液性，3例为胆汁样液体。3例患者有伴有鳞状细胞癌的大囊肿，2例有广泛鳞状化生但无恶性病变。其他患者组织病理学为良性。
临床医生对CHFC的认识日益提高。单纯影像学本身不能确诊，但结合整体情况考虑确实可为诊断提供重要线索。FNAC通过吸出物中存在纤毛柱状上皮细胞而具有诊断价值，但缺乏敏感性。婴儿期表现通常伴有与胆管相通，需要采用不同的手术方法。3例出现恶性转化及其致命病程强调一旦确诊，所有病例均需手术切除。
