CAJ | 학술논문

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。
목적：탐토궁경선양낭성암（adenoid cystic carcinoma， ACC）림상병리급면역조화특정。방법수집북경협화의원2003년1월지2013년12월병리수거고급회진수거고중진단위궁경선양낭성암병례공4례；병채용면역조화방법대석사조직표본진행검측，총결기림상병리특정、치료급예후。결과4례궁경선양낭성암환자평균년령61.5세，다위절경후부녀（3／4），취진주요증상위음도류혈（3／4），종류류형다위외생성종물（3／4）。병이학특정방면，3례환자표현위궁경선양낭성암합병침습성린암，1례위궁경선양낭성암단일조분；선양낭성암적주요생장방식위사상；면역조직화학표체방식지지기도관선상피급기상피량충조성성분： CK7재도관선상피중양성표체， P63、 SMA재기상피중양성표체；선양낭성암특정성표지물C-MYB재소유병례중균강양성표체。3례환자완전절제자궁，1례환자행궁경추절，림상분기균위Ⅰ기；술후균행방사치료，2례환자동시행화학치료；4례환자평균수방시간위21.25개월，균위무병생존상태。결론궁경선양낭성암시일충한견적특수류형선암，상합병기타류형적궁경종류，면역조화표형여기타부위적선양낭성암상동，단예후상대교차，술후가보이방료급화료，조기발현병치료가이제고환자생존솔。
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 .