恶性蝾螈瘤2例报道及文献复习  

Malignant triton tumor(A report of 2 cases and literature review)

作　　者：徐芳[1] 李果[1] 刘勇[1] 李惠清[1] 李冬红[1] 肖健云[1] 邱元正[1] 

机构地区：[1]中南大学湘雅医院耳鼻咽喉头颈外科,耳鼻咽喉重大疾病研究湖南省重点实验室,湖南长沙410008

出　　处：《中国耳鼻咽喉颅底外科杂志》2012年第6期451-454,459,共5页Chinese Journal of Otorhinolaryngology-skull Base Surgery

摘　　要：目的探讨恶性蝾螈瘤(malignant triton tumor,MTT)的临床特点,以提高诊断治疗水平。方法结合文献回顾分析2例恶性蝾螈瘤患者的临床资料。结果 2例患者分别发生于扁桃体和咽旁间隙。1例患者经咽侧间隙与口内联合径路行左咽侧肿块切除术;术后辅予放、化疗。随访5年以上,患者预后好,目前张口稍受限,生活自理。1例3岁幼儿患者放弃治疗。结论恶性蝾螈瘤是恶性周围神经鞘膜瘤的一种亚型,临床上少见,极具侵袭性,易复发转移,肿瘤的根治性切除并辅以放、化疗可延长MMT术后生存时间。Objective To investigate the clinical characteristics of malignant triton tumor, so as to improve the diagnostic level. Methods The clinical data of 2 patients with malignant triton tumor was analyzed retrospectively with the literature review. Results The tumors occurred in the left tonsil and fight parapharyngeal space respectively. One patient received from resection form the left lateral pharyngeal space via jaw and neck approach, combined with radiotherapy and chemotherapy postoperatively. The patient was followed up for more than 5 years after operation with a fine prognosis. This patient has slight limitation of mouth opening only and the daily life is independent. One three-year-old patient abandoned any treatment. Conclusion Malignant triton tumor is a rare subtype of malignant peripheral nerve sheath tumor with high invasiveness, and easy recurrence and metastasis. Pathological examination is the only means for diagnosis. Radical tumor resection combined with postoperative radiotherapy and chemotherapy may prolong MMT survival time.

关 键 词：恶性蝾螈瘤 周围神经鞘膜瘤 侵袭性 扁桃体 咽旁间隙 

分 类 号：R739.9[医药卫生—肿瘤]