喉低级别肌纤维母细胞肉瘤临床病理观察

Low-grade myofibroblastic sarcoma of larynx:a clinicopathological analysis

目的探讨喉低级别肌纤维母细胞肉瘤(LGMS)的临床病理特征、诊断及鉴别诊断。方法对1例喉LGMS进行形态学及免疫组化观察,并复习相关文献。结果患者男性,59岁。临床表现为声嘶、呼吸困难及吞咽疼痛。肿瘤位于喉室左侧黏膜下,组织学显示呈束状、旋涡状或不规则形排列;瘤细胞呈胖梭形,胞质界限不清、淡嗜酸性,核两端逐渐变尖,多形性明显;部分区域血管丰富并见灶性坏死。免疫组化示SMA、calponin和desmin(+),而CK、S-100和h-caldesmon(-)。结论发生于喉的LGMS极为罕见,其形态学诊断易与其他梭形细胞肿瘤(如肉瘤样癌及恶性纤维组织细胞瘤等)相混淆。本瘤易复发但很少转移,治疗以手术为主,应注意密切随访。

Objective To investigate the clinicopathologic features,diagnosis and differential diagnosis of low-grade myofibroblastic sarcoma（LGMS） of the larynx.Methods One case of LGMS was studied morphologically and immunohistochemically,followed by review of related literature.Results The patient was a 59-year-old man,who presented with hoarseness,odynopgagia and dyspnea.Histologically,the tumor was located within the submucosa of the left laryngeal ventricle and arranged in fascicular,whirlpool or patternless pattern.The tumor cells were showed as plump spindle-shaped with ill-defined cell borders,slightly eosinophilic cytoplasms and tapered nuclei.In addition,significant nuclear pleomorphism,hypervascularity and focal coagulative necrosis were seen in some parts of the tumor.Immunohistochemically,the tumor cells were positive for SMA,calponin,and desmin,but negative for CK,S-100 and h-caldesmon.Conclusions It is extremely rare that LGMS occurs in the larynx.When making diagnisis,it should be distinguished LGMS from those of other spindle-shaped cell tumors such as sarcomatoid carcinoma and malignant fibrous histiocytoma（MFH）.It can recur but rarely metastasise.Surgical resection is the mainstay of therapy and close follow-up is recommended.