首页|肝内胆管导管内嗜酸性乳头状肿瘤的临床病理特征及文献复习

肝内胆管导管内嗜酸性乳头状肿瘤的临床病理特征及文献复习

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目的:探讨肝内胆管的导管内嗜酸性乳头状肿瘤(IOPN)的临床特征及组织病理学特点、免疫表型与鉴别诊断.方法:回顾性分析1例肝内IOPN的临床病理特征,采用免疫组化方法检测AE1/AE3、EMA、MUC1、MUC2、MUC5AC、MUC6、AFP、HepPar-1、CA199、CDX-2、CK19、CK7、CK20、p53、Ki-67在IOPN中的表达模式,并进行文献复习.结果:患者男性,55岁,CT显示肝左内叶囊性为主占位.囊腔大小约8.0 cm×8.0 cm×3.0 cm,镜下肿瘤组织位于囊腔内,与周围边界欠清,呈乳头状结构生长,肿瘤细胞大小较一致,核呈圆形或椭圆形,存在轻-中度异型性,染色质粗糙,可见小核仁,未见病理性核分裂像,肿瘤细胞胞质丰富,可见明显嗜酸性颗粒,局灶囊壁见肿瘤细胞侵犯.免疫组化标记结果显示,肿瘤细胞AE1/AE3、EMA、MUC6、HepPar-1、CK19及CK7阳性表达;个别肿瘤细胞MUC1阳性表达;部分肿瘤细胞MUC5AC阳性表达;p53为野生型;肿瘤细胞MUC2、AFP、CA199、CDX-2及CK20均呈阴性表达.结论:肝内IOPN是一种罕见的原发肝脏肿瘤,临床及影像学检查不能明确诊断,需与多种肿瘤鉴别,最终确诊需依赖组织形态学特点及免疫表型,治疗方法以手术切除为主,无浸润病例预后佳,浸润型复发风险较高.
Clinicopathological features and literature review of intraductal oncocytic papillary neoplasm in the intrahepatic bile duct
Objective:To investigate the clinical characteristics,histopathological manifestation,immunophenotype and differential diagnosis of intraductal oncocytic papillary neoplasm(IOPN)in the intrahepatic bile duct.Methods:The clinicopathological characteristics were analyzed retrospectively in one case of IOPN.Immunohistochemistry was used to detect the expression of AE1/AE3,EMA,MUC1,MUC2,MUC5 AC,MUC6,AFP,HepPar-1,CA199,CDX-2,CK19,CK7,CK20,p53 and Ki-67 in IOPN,and related literatures were reviewed.Results:The patient was a 55-year-old male.CT revealed a cystic space occupying lesion in the left inner lobe of the liver.The size of the cyst was approximately 8.0 cm×8.0 cm×3.0 cm.Microscopy exposed that the tumor tissue was located within the cyst cavity,with inexplicitly defined boundary between the tumor and surrounding tissues,and grew in a papillary structure.Tumor cells showed relatively consistent size,with round or oval nuclei,mild-moderate heteromorphism,rough chromatin,and small nucleoli.No pathological mitosis was observed.The cytoplasm of tumor cells was abundant and eosinophilic granules were visible.Tumor cells invaded the focal cyst wall.Immunohistochemical markers showed positive expression of AE1/AE3,EMA,MUC6,HepPar-1,CK19 and CK7 on tumor cells.MUC1 was positively expressed in individual tumor cells,and MUC5AC was positively expressed in partial tumor cells.p53 was in wild type.MUC2,AFP,CA199,CDX-2 and CK20 were all negatively expressed on tumor cells.Conclusion:Intrahepatic IOPN is a rare primary liver growth,for which clinical and imaging examinations can not provide a definitive diagnosis and must be differentiated from various tumors.Final confirmation relies on histomorphological findings and immunophenotypes.The primary treatment depends on surgical resection,with good prognosis in non-invasive cases and a higher recurrence risk in invasive types.

intrahepatic bile ductintraductal oncocytic papillary neoplasmimmunophenotypepathological diagnosisdifferential diagnosis

田晓蕾、王冠男、邢雪雪、梅晶晶、汪向明

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皖南医学院 研究生学院,安徽 芜湖 241002

皖南医学院第一附属医院弋矶山医院肝胆外科,安徽芜湖 241001

华东师范大学附属芜湖医院芜湖市第二人民医院 病理科,安徽 芜湖 241001

皖南医学院第一附属医院弋矶山医院病理科,安芜湖 241001

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肝内胆管 导管内嗜酸性乳头状肿瘤 免疫表型 病理诊断 鉴别诊断

皖南医学院重点项目科研基金

WK2022ZF14

2024

皖南医学院学报
皖南医学院

皖南医学院学报

CSTPCD
影响因子:0.695
ISSN:1002-0217
年,卷(期):2024.43(5)
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