喉肉瘤样癌及癌肉瘤临床分析

Clinic features of laryngeal carcinosarcoma and sarconmtoid carcinoma

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。

Objective To investigate the clinic feature, pathology, therapy and prognosis of the sarcomatoid caricinoma or 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.