宫颈腺样囊性癌临床病理特征及免疫组织化学表型

Clinicopathological Features and Immunohistochemical Phenotypes of Adenoid Cystic Carcinoma of the Uterine Cervix

目的探讨宫颈腺样囊性癌(adenoid cystic carcinoma,ACC)临床病理及免疫组化特征。方法收集北京协和医院2003年1月至2013年12月病理数据库及会诊数据库中诊断为宫颈腺样囊性癌病例共4例;并采用免疫组化方法对石蜡组织标本进行检测,总结其临床病理特征、治疗及预后。结果 4例宫颈腺样囊性癌患者平均年龄61.5岁,多为绝经后妇女(3/4),就诊主要症状为阴道流血(3/4),肿瘤类型多为外生性肿物(3/4)。病理学特征方面,3例患者表现为宫颈腺样囊性癌合并侵袭性鳞癌,1例为宫颈腺样囊性癌单一组分;腺样囊性癌的主要生长方式为筛状;免疫组织化学表达方式支持其导管腺上皮及肌上皮两种组成成分:CK7在导管腺上皮中阳性表达,P63、SMA在肌上皮中阳性表达;腺样囊性癌特征性标志物C-MYB在所有病例中均强阳性表达。3例患者完全切除子宫,1例患者行宫颈锥切,临床分期均为Ⅰ期;术后均行放射治疗,2例患者同时行化学治疗;4例患者平均随访时间为21.25个月,均为无病生存状态。结论宫颈腺样囊性癌是一种罕见的特殊类型腺癌,常合并其他类型的宫颈肿瘤,免疫组化表型与其他部位的腺样囊性癌相同,但预后相对较差,术后可辅以放疗及化疗,早期发现并治疗可以提高患者生存率。

Objective To investigate the clinicopathological and immunohistochemical features of adenoid cystic carcinoma （ ACC） of the uterine cervix .Methods Four cases who were diagnozed with ACC of the uter-ine cervix in the period from January 2003 to December 2013 were collected from the pathological databank and consultation database of Peking Union Medical Hospital .Immunohistochemical examination was conducted on the formalin-fixed, paraffin-embedded tissue specimens from the 4 patients.Clinical information, pathological fea-tures, treatment, and outcomes of these patients were summarized .Results The average age of the 4 ACC patients was 61.5 years.The patients were mostly post-menopausal （3/4） and their chief complaint was vaginal bleeding （3/4）.The tumors were primarily protruding masses （3/4）.Histologically, 3 of the 4 cases were ACC combined with invasive squamous cell carcinoma , the other 1 was ACC alone .The growth pattern of ACC in all of the 4 ca-ses was cribriform composed of luminal adenoepithelial and abluminal myoepithelial cells .Immunohistochemical findings supported the identification of 2 components in ACC：positive expression of CK 7 in luminal adenoepithe-lial cells, and positive expressions of P 63 and SMA in abluminal myoepithelial cells .C-MYB, a ACC-specific marker, was found with strong positive expression in all the 4 cases.Three of the 4 patients had total hysterecto-my, the other 1 had conization .All were in stage I in terms of clinical staging .Postoperative radiotherapy was ad-ministered in all the 4 cases, combined with chemotherapy in 2.The mean follow-up period was 21.25 months, finding all the 4 patients in disease-free survival .Conclusions ACC of the uterine cervix is a rare special type of adenocarcinoma , usually coexisting with other types of cervical tumors .The immunohistochemical findings in ACC of the uterine cervix are similar with ACC in other organs , but associated with poorer prognosis .Radiotherapy and chemotherapy after sugery is recommended .Early detection and treatment could improve survival .