头颈部软骨黏液样纤维瘤7例临床病理特征分析  

Clinicopathological characteristics of chondromyxoid fibroma of the head and neck: an analysis of seven cases

作　　者：刘晋红[1] 成兰云[2] 邓晋芳[1] 石怀银[1] LIU Jin-hong1 , CHENG Lcm-yun2 , DENG Jin-fang1 , SHI Huai-yin1(1. Department of Pathology, the Chinese PEA General Hospital, Beijing 100083, China; 2. Department of Pathology, Zhuozhou City＇s Hospital Hebei Province, Zhuozhou 072750, Chin)

机构地区：[1]解放军总医院病理科,北京100853 [2]涿州市医院病理科,河北涿州072750

出　　处：《诊断病理学杂志》2018年第3期181-185,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨头颈部软骨黏液样纤维瘤(CCMF)的临床病理学特征及其鉴别诊断。方法收集7例发生在头颈部的软骨黏液样纤维瘤患者的临床及病理资料,观察临床表现及组织形态特征,并结合文献复习。结果 7例中男性4例,女性3例,年龄17~58岁,中位年龄35岁。发生于颅底骨2例、乳突2例、颈椎体、颌骨和右眼颅眶各1例。临床以疼痛、肿胀为主要症状。影像学检查均显示相应部位的类圆形占位性病变。手术见肿瘤呈灰白色半透明样、质软,血供不丰富,并见局部骨质破坏。镜下肿瘤呈分叶状结构,富含黏液软骨样基质,瘤细胞为短梭形及星芒状,小叶中央细胞稀疏,周边细胞较密集,并见多核巨细胞、钙盐沉积及出血。免疫组化显示肿瘤细胞vimentin和S-100(+)。7例中4例失访,其余3例分别随访5年、4年8个月和2个月,至今无复发。结论软骨黏液样纤维瘤是一种很少见的软骨源性良性肿瘤,发生在头颈部更少见,因此诊断应结合临床资料和影像学表现,并需与骨肿瘤相鉴别,尤其应与恶性软骨肿瘤相鉴别。Objective To study the clinicopathological features and differential diagnosis of chondromyxoid fibroma（ CMF） of the head and neck. Methods The clinicopathological data nere collected in 7 cases of CMF of the head and neck. The clinieal presentation and morpholgical features were analyzed with review of related literature. Results In the7 cases,4 male and 3 female were included,ages 17 to 58 years,and the median age was 35 years. 2 cases of CMF located in skull base bone,2 in mastoid process,1 in cervical vertebrae,1 in jaw bone and 1 in the right side of cranio-orbital region. Pain and swelling were common presenting symptoms. The imaging of CMF presented as a round-like mass. Most of the CMF was domed,grey and semitransparent soft mass,with poor vascular supply and local bone destruction. The histopathological features of CMF included lobulated structure,rich in mucus chondroid matrix. The tumor cells were elongated or star-like shape,high cellularity in the center,low cellularity in the circum. Multinuclear giant cell,calcium deposition and hemorrhage were common. The tumor cells demonstrated immunoreactivity for vimentin and S-100. At the end of study,4 out of 7 cases were lost to follow-up and the remaining 3 cases were followed up for 5 years,4 years and 8 months,2 months respectively; so far there was no recurrence. Conclusions Chondromyxoid fibroma is a rare chondrogenic benign tumor,while even rare in the head and neck. It should be distinguished from bone tumor,especially malignant cartilage tumor. Therefore,histological and radiographic appearance plays important roles in diagnosis of this tumor.

关 键 词：软骨黏液样纤维瘤 头颈部 临床病理特征 鉴别诊断 

分 类 号：R738.3[医药卫生—肿瘤]