气管上段腺样囊性癌的临床病理

Clinicopathological Features of Adenoid Cystic Carcinoma of the Upper Trachea

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。

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 .