摘要目的 探讨食管腺样囊性癌的临床病理特点及鉴别诊断.方法 总结10例发生于食管的腺样囊性癌,分析其临床病理学、免疫组织化学特征及鉴别诊断.结果 患者中男性9例,女性1例,发病年龄59～76岁.肿瘤部位:4例位于食管中段,4例位于食管中下段,2例位于食管下段.低倍镜下肿瘤均为推挤性的生长方式,结构以筛状、实性混合为主,局灶呈小管状,瘤细胞核深染,核质比高,部分示导管和肌上皮分化的特征,部分间质显示黏液透明样变.8例伴有实性细胞巢中央的坏死,1例表面鳞状上皮伴中-重度不典型增生,3例伴有小灶鳞状细胞癌成分.免疫组织化学显示瘤细胞p63 (10/10)、S-100蛋白(9/10)、calponin(2/10)、平滑肌肌动蛋白(2/10)、CD117(10/10)、细胞角蛋白7腺管样成分(10/10)阳性.结论 食管腺样囊性癌作为独立的肿瘤实体,有其独特的形态学特征,免疫表型呈S-100蛋白表达以及恒定的表达CD117,需与基底细胞样鳞状细胞癌、黏液表皮样癌、小细胞癌鉴别.

abstractsObjective To study the clinicopathologic features and differential diagnosis of adenoid cystic carcinoma in the esophagus.Methods Ten cases of primary adenoid cystic carcinoma of the esophagus were retrieved from the archival file.The clinicopathologic and immunohistochemical features were studied.The differential diagnosis was analyzed.Results The male-to-female ratio was 9:1.The age of patients ranged from 59 to 76 years.There were 4 cases with tumor located in mid esophagus,4 cases with tumor located in mid to lower esophagus and the remaining 2 cases in lower esophagus.Low-power histologic examination showed mainly expansive growth pattern,with cribriform,solid and focal tubular architectures identified.The tumor cells showed nuclear hyperchromasia.Both ductal and myoepithelial differentiation was demonstrated.The stroma showed myxoid degeneration in areas.Comedo-type necrosis was observed in 8 cases and moderate to severe squamous dysplasia was present in one case.Three cases showed focal areas of squamous cell carcinoma.Immunohistochemical study showed that the tumor cells were positive for p63 (10/10),CD117 (10/10) and S-100 protein (9/10).There was focal staining for calponin (2/10) and smooth muscle actin (2/10).The ductal structures expressed CK7 (10/10).Conclusions Adenoid cystic carcinoma of the esophagus demonstrates unique morphologic features with expression of S-100 protein and consistent expression of CD117.The above characteristics help to distinguish this entity from basaloid squamous cell carcinoma,mucoepidermoid carcinoma and small cell carcinoma of the esophagus.