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纤毛肝前肠囊肿,一种罕见的细支气管前肠畸形:7例的组织学、组织化学和免疫组织化学研究

The ciliated hepatic foregut cyst, an unusual bronchiolar foregut malformation: a histological, histochemical, and immunohistochemical study of 7 cases.

作者信息

Chatelain D, Chailley-Heu B, Terris B, Molas G, Le Caë A, Vilgrain V, Belghiti J, Degott C, Flejou J F

机构信息

Department of Pathology, Hôpital Beaujon, Clichy, and INSERM U319, Université Paris 7, France.

出版信息

Hum Pathol. 2000 Feb;31(2):241-6. doi: 10.1016/s0046-8177(00)80227-0.

DOI:10.1016/s0046-8177(00)80227-0
PMID:10685641
Abstract

The ciliated hepatic foregut cyst is an unusual solitary cystic lesion of the liver. In a series of 7 cases of hepatic ciliated cysts, we performed a histological, histochemical, and immunohistochemical study to better define the histogenesis of this rare entity. The patients were 4 women and 3 men, aged 39 to 75 years. Four patients presented with abdominal pain. In 3 cases the cyst was discovered incidentally on ultrasonography. The cysts measured from 1 to 4 cm in diameter. Microscopically, the lining of the columnar epithelium was composed of ciliated cells and mucin secreting goblet cells. The wall was composed of bands of smooth-muscle fibers surrounded by an outer fibrous capsule. The goblet cells stained with PAS, alcian blue, and high-iron diamine. The immunohistochemical study showed that endocrine cells were present within the cyst epithelium, positive for chromogranin, synaptophysin, bombesin, and calcitonin, and negative for serotonin, somatostatin, glucagon, insulin, gastrin, and pancreatic polypeptide. In all the cases, immunoreactivity of some cells for CC10 strongly suggested the presence of Clara cells. Our study shows that the epithelium lining ciliated hepatic foregut cysts has histological, histochemical, and immunohistochemical features similar to those observed in the bronchiolar epithelium. This lesion is a developmental ventral foregut abnormality that could arise from a bronchiolar bud of the tracheobronchial diverticulum.

摘要

纤毛肝前肠囊肿是一种罕见的肝脏孤立性囊性病变。在一组7例肝纤毛囊肿病例中,我们进行了组织学、组织化学和免疫组织化学研究,以更好地明确这种罕见病变的组织发生。患者包括4名女性和3名男性,年龄在39至75岁之间。4例患者表现为腹痛。3例囊肿是在超声检查时偶然发现的。囊肿直径为1至4厘米。显微镜下,柱状上皮内衬由纤毛细胞和分泌粘蛋白的杯状细胞组成。囊壁由平滑肌纤维束组成,周围有一层纤维性外膜。杯状细胞对PAS、阿尔辛蓝和高铁二胺染色阳性。免疫组织化学研究显示,囊肿上皮内存在内分泌细胞,嗜铬粒蛋白、突触素、蛙皮素和降钙素呈阳性,而血清素、生长抑素、胰高血糖素、胰岛素、胃泌素和胰多肽呈阴性。在所有病例中,一些细胞对CC10的免疫反应性强烈提示存在克拉拉细胞。我们的研究表明,纤毛肝前肠囊肿的上皮内衬具有与细支气管上皮相似的组织学、组织化学和免疫组织化学特征。这种病变是一种发育性腹侧前肠异常,可能起源于气管支气管憩室的细支气管芽。

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