摘要
目的探讨头颈部非典型软骨肿瘤/软骨肉瘤Ⅰ级的临床及病理特征、鉴别诊断、治疗方法及预后。方法采用光学显微镜及免疫组织化学LSAB法结合临床表现、治疗和预后资料,对25例发生于头颈部的非典型软骨肿瘤/软骨肉瘤Ⅰ级进行整体分析。结果 25例患者中,男性11例、女性14例;发病年龄21~80岁,平均51岁。肿瘤分别位于鼻腔、鼻窦11例,鼻咽部2例,喉部3例,颈静脉孔4例,上颌骨3例,面神经1例,颞骨1例。患者多以鼻塞、鼻出血、疼痛就诊。CT显示鼻腔、鼻窦等相应部位占位性病变,其中大部分有骨质破坏。免疫组织化学染色显示肿瘤表达波形蛋白（Vim）、S100蛋白。随访18例病例,经手术治疗均健在,4例复发。结论头颈部非典型软骨肿瘤/软骨肉瘤Ⅰ级较为少见,多发生于鼻腔、鼻窦和颅底,形态学上易与软骨瘤相混淆,需要结合临床资料、CT检查、形态学等综合鉴别诊断。（中国眼耳鼻喉科杂志,2015,15：280-284）
Objective To investigate the clinicopathologic features,differential diagnosis,treatment and prognosis of atypical cartilaginous tumour / chondrosarcoma grade I of the head and neck. Methods The clinical,pathologic and immunohistochemical features,treatment and prognosis of 25 cases with atypical cartilaginous tumour /chondrosarcoma grade I in head and neck were evaluated. Results Among the 25 cases studied,there were 11 males and14 females. The age of patients ranged from 21 to 80 years old（ mean 51 years）. The lesions arose in the nasal cavity（ 11cases）,nasopharyngeal portion（ 2 cases）,the throat（ 3 cases）,jugular foramen（ 4 cases）,upper jaw（ 3 cases）,facial nerve（ 1 case） or temporal bone（ 1 case）. Most of the patients presented with nasal obstruction,rhinorrhagia or pain. CT scan often showed the presence of soft tissue lesion and most of which accompanied with bone destruction.Immunohistochemical study showed that the tumor strongly expressed S100 protein and vimentin. Eighteen patients were followed up and all lived alive after the surgery,in which 4 cases got recurrence. Conclusions Atypical cartilaginous tumour / chondrosarcoma grade I of the head and neck is very rare,which was usually confused with the chondroma. It is usually occured in the nasal cavity and the base of the skull. The clinical manifestation,CT scan,pathologic and immunohistochemical features are all helpful in the differential diagnosis.（ Chin J Ophthalmol and Otorhinolaryngol,2015,15： 280-284）
出处
《中国眼耳鼻喉科杂志》
2015年第4期280-284,共5页
Chinese Journal of Ophthalmology and Otorhinolaryngology
关键词
非典型软骨肿瘤/软骨肉瘤Ⅰ级
头颈部
诊断
鉴别
治疗
预后
Atypical cartilaginous tumour/chondrosarcoma gradeⅠ
Head and neck
Diagnosis
Differentiation
Treatment
Prognosis
