头颈部低度恶性肌纤维母细胞肉瘤

[摘要]目的 探讨低度恶性肌纤维母细胞肉瘤( low - grade myofibroblastic sarcoma, LGMS)的临床表现，病理组织学特征、治疗及预后。方法 回顾性分析13例头颈部LGMS患者的临床资料，并进行光镜检查及免疫组化染色。结果 光镜下肿瘤细胞呈梭形或星形,排列成束状或席纹状。免疫组化染色结果示瘤细胞弥漫性广泛表达Vim，而Act和SMA表达程度不一,但均不表达CD34、S-100蛋白。术后随访2-16个月，其中12例术后均无复发，另1例声带LGMS 5个月后复发再次手术。结论 LGMS是低度恶性肉瘤，手术为首选治疗，明确诊断后局部进行广泛切除并随访。河北医科大学第四医院耳鼻喉科王占龙

关键词：低度恶性肌纤维母细胞肉瘤，头颈部肿瘤，临床特点

low - grade myofibroblastic sarcoma tumor of the head and neck

WANG Zhan-long¹，WANG Zhan-dong ²,LIU Sheng-hui¹，ZHAO Rui-li¹

(1 Department of Otolaryngology- Head and Neck Surgery，Forth hospital of Hebei medical university，shijiazhuang 050011，China; 2 Department of Pathobiology，Forth hospital of Hebei medical university，shijiazhuang 050011，China)

Corresponding author: Corresponding author: ZHAO Rui-li (Email: [e-mail])

[abstract] Objective To study the clinical and pathology characteristics，treatment，prognosis of  LGMS in head and neck.Methods A retrospective analysis was made on the clinical data of 13 cases.Results Microscopically，the tumor cells were spindle-shsped and arranged in fasciculus or whirlpool. Immunohistochemically，the tumor cells were diffusely strong positive for vim and not uniform for Act and SMA，but negative for CD34 and S-100. The patients were followed up for 10 days to 16 months after operation.Twelve patients were alive with no evidence of diseases，one patient was alive recurred at 5 months after operation.Conclutions LGMS is a low grade malignant tumor，the main treatment of LGMS is to resect the tumor thoroughly，close follow-up is necessary in the treatment to aviod recurrences.

[keywords] low - grade myofibroblastic sarcoma tumor ; head and neck tumor ; clinical characteristics