目的 探讨食管黏膜原发性Paget病的临床病理特征、诊断与鉴别诊断.方法 分析4例食管黏膜原发性Paget病的临床病理特征、组织学形态及免疫组化标记的表达,并复习相关文献.结果 患者均为男性,年龄61 ～74岁,均发生于食管黏膜,光镜下Paget细胞于食管黏膜内呈单个或小巢状、腺泡状分布,2例伴原位腺癌,其中1例伴远隔部位的食管鳞癌;免疫组化EnVision法染色显示Paget细胞均强表达CKpan、CKL、CK7,不表达CKH、CK5/6、CK14、p63,与食管鳞癌免疫组化标记相反.结论 食管黏膜原发性Paget病非常罕见,可单独发生,或可伴原位腺癌、食管鳞癌,确诊需详细的病理形态学观察、免疫组化染色,并结合临床病史排除继发性、转移性可能,避免误、漏诊.%Purpose To investigate the clinicopathological and diagnostic characteristics of primary Paget disease (PD ) in esophagus. Methods The clinical presentation, histological observation and immunohistochemical staining were analyzed in four cases of primary PD involved esophagus and related literatures were reviewed. Results The patients were all male, aged from 61 to 74 years old. All the tumors were originated from the mucosa of the esophagus. Histologically, the Paget cells showed a single or small nesting and acinar distribution in the esophage-al mucosa. Adenocarcinoma in situ were seen in 2 cases and squamous cell carcinoma was seen in one of them. Immunohisto-chemically, the Paget cells were typically strongly positive for Ckpan, CKL, and CK7, while negative for CKH, CK5/6, CK14, p63. Conclusion Primary esophagus PD is rare. It can develop alone in esophagus or accompanied with adenocarcinoma in situ, invasive squamous cell carcinoma. The correct diagnosis need detailed pathological observation, immunohistochemical ev-idence and medical history.
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