摘要目的 探讨喉肉瘤样癌及癌肉瘤的临床特征、病理表现、治疗手段及预后.方法 回顾性分析1996年6月至2016年4月中国医学科学院北京协和医学院北京协和医院耳鼻咽喉科7例病理证实的原发于喉的肉瘤样癌或者癌肉瘤临床病例资料.7例患者均为男性,平均发病年龄65.9岁,6例有长期吸烟史,其中2例有喉部放疗病史;未前期治疗的5例患者均以声音嘶哑为首发症状;主要原发部位均位于声门区域,典型喉镜表现为表面基本光滑的息肉样或菜花样新生物;就诊时肿瘤分期Ⅰ期2例,Ⅱ期2例,Ⅲ期1例,另外2例均为喉癌手术+放疗后复发患者,不适合分期.结果患者均手术治疗,均无淋巴结转移.术后病理报告为肉瘤样癌或癌肉瘤,病理显示肿物为癌和肉瘤样成分并存,癌成分为鳞状细胞癌,肉瘤成分表现为杂乱排列的梭形细胞,免疫组化显示肿瘤组织Vimetin强阳性,SMA阳性3例,CD68阳性0例,S-100阳性1例,均无HMB-45及Myglobin表达.随访3个月至20年,其中死亡1例,失访2例,其余4例患者目前均存活.结论 喉部肉瘤样癌或癌肉瘤光镜下都可见到癌及肉瘤成分,肉瘤样癌分子水平研究显示该类肿瘤为单克隆的上皮性肿瘤,伴有间叶趋异进化,手术为主要治疗手段,无前期放疗病史及肉瘤样成分侵入较浅者预后良好.

abstractsObjective To investigate the clinic feature,pathology,therapy and prognosis of the sarcomatoid caricinoma or carcinosarcoma of the larynx.Methods We reviewed the clinical records of 7 patients with laryngeal carcinosarcoma/sarcomatoid caricinoma who were treated at our hospital between June 1996 and August 2016.All patients were men (mean age,65.9 years;range,52 to 94 years).Among 7 patients,6 had a history of smoking;2 underwent radiotherapy;and 5 patients who didn't undergo radiotherapy complained of hoarseness.The glottis was the most frequent site of involvement.Most tumors exhibited a polypold or pedunculated gross morphology.Among the 5 patients who didn't undergo a radiotherapy,2 were in stage Ⅰ,2 in stage Ⅱ,and 1 in stage Ⅲ.The other 2 cases underwent surgeries and radiotherapy were staged.Results All 7 patients received surgeries,without lymph node metastasis.All the tumors were pathologically carcinosarcoma/sarcomatoid carcinoma.With immunohistochemistry examination,Vimetin was positive in all tumors,SMA positive in 3 tumors,S-100 positive in 1 tumors,but CD-68,HMB-45 or Myglobin was negative in all tumors.With follows-up from 3 months to 20 years,of 7 patients,4 survived without recurrent,1 dead,and 2 lost connection.Conclusions Both of the carcinosarcoma and the sarcomatoid carcinoma of larynx contain pathologically carcinoma and sarcoma.Surgery is the best choice for laryngeal sarcomatoid carcinoma,and these patients without a undergoing radiotherapy before surgery or these with little sarcoma in tumors show better prognosis.