CAJ | 학술논문

目的：探讨气管上段腺样囊性癌的临床病理特征及诊断和鉴别诊断要点。方法收集2000年1月至2014年2月在北京协和医院确诊的4例气管上段腺样囊性癌病例，通过光镜、免疫组织化学及组织化学染色分析其临床病理特征、免疫组织化学特点、诊断及鉴别诊断要点。结果4例气管上段腺样囊性癌患者中，男1例，女3例，平均年龄47岁（38～57岁）；既往均无腺样囊性癌病史，1例患者既往有结节性甲状腺肿手术史。镜下检查4例均为筛状／管状型腺样囊性癌，3例累及甲状腺组织，3例累及神经组织，未见淋巴结受累。免疫组织化学染色示4例P16、 CD117、 BCL-2、 P63、 SMA均阳性， Ki-67指数平均8％， TTF-1和P53均阴性；4例中基底膜样物胶原Ⅳ阳性；组织化学染色示AB／PAS阳性。术后均接受总剂量为48～56 Gy的放疗，随访6～120个月，平均72.5个月，1例术后96个月复发，3例随访期间无复发及转移。结论气管上段腺样囊性癌是罕见的原发于气管的低度恶性肿瘤，肿瘤生长缓慢，就诊时多数已侵及甲状腺组织，需要与原发于甲状腺的恶性肿瘤相鉴别，特别是在甲状腺穿刺及术中冰冻检查时。结合电子喉气管镜下表现、典型的形态学及免疫组织化学和组织化学染色有助于准确诊断。手术难以切除干净，术后放疗对延缓疾病复发有一定帮助。
목적：탐토기관상단선양낭성암적림상병리특정급진단화감별진단요점。방법수집2000년1월지2014년2월재북경협화의원학진적4례기관상단선양낭성암병례，통과광경、면역조직화학급조직화학염색분석기림상병리특정、면역조직화학특점、진단급감별진단요점。결과4례기관상단선양낭성암환자중，남1례，녀3례，평균년령47세（38～57세）；기왕균무선양낭성암병사，1례환자기왕유결절성갑상선종수술사。경하검사4례균위사상／관상형선양낭성암，3례루급갑상선조직，3례루급신경조직，미견림파결수루。면역조직화학염색시4례P16、 CD117、 BCL-2、 P63、 SMA균양성， Ki-67지수평균8％， TTF-1화P53균음성；4례중기저막양물효원Ⅳ양성；조직화학염색시AB／PAS양성。술후균접수총제량위48～56 Gy적방료，수방6～120개월，평균72.5개월，1례술후96개월복발，3례수방기간무복발급전이。결론기관상단선양낭성암시한견적원발우기관적저도악성종류，종류생장완만，취진시다수이침급갑상선조직，수요여원발우갑상선적악성종류상감별，특별시재갑상선천자급술중빙동검사시。결합전자후기관경하표현、전형적형태학급면역조직화학화조직화학염색유조우준학진단。수술난이절제간정，술후방료대연완질병복발유일정방조。
Objective To investigate the clinicopathological features and key points in diagnosis and dif -ferential diagnosis of adenoid cystic carcinoma of the upper trachea .Methods From January 2000 to February 2014 , 4 cases of adenoid cystic carcinoma of the upper trachea were diagnosed in Peking Union Medical College Hospital .Microscopic , immunohistochemical , and histochemical staining results of the patients were reviewed to summarize the clinicohistological and immunohistochemical features as well as key points of diagnosis and differ -ential diagnosis of adenoid cystic carcinoma of the upper trachea .Results The 4 patients of adenoid cystic carci-noma of the upper trachea included 1 male and 3 females, aged 38-57 years (mean age 47 years).None had history of adenoid cystic carcinoma , and one had the history of nodular goiter surgery .Microscopically , 4 cases all appeared as cribriform/tubular type, with thyroid tissue involvement in 3 cases, nervous tissue involvement in 3 cases, and no lymph node involvement .Immunohistochemically , P16, CD117, BCL-2, P63, SMA, and typeⅣ collagen were positive in all 4 patients , average Ki-67 index was 8%, and TTF-1 and P53 were negative in all patients .Histochemically , AB/PAS was positive in all patients .All the 4 cases received postoperative radiothera-py (total dose 48-56 Gy) and were followed up for 6-120 months (mean 72.5 months).One case had relapse after 96 months , and the other 3 had no recurrence or metastasis during the follow-up period .Conclusions Ad-enoid cystic carcinoma of the upper trachea is a rare low-grade malignant tumor .It is slow-growing and the major-ity of patients have invasion of thyroid tissue at presentation .It has to be differentiated from primary thyroid canc-er , especially in fine-needle aspiration cytology and intraoperative frozen section of the thyroid .Accurate diagno-sis can be made based on laryngotracheoscopical findings , typical morphological , immunohistochemical and histo-chemical features .Complete resection of the tumor is hard to achieve , therefore radiotherapy may be useful to prevent recurrence .