头颈部心外横纹肌瘤

Extracardiac rhabdomyoma in the head and neck region

横纹肌瘤是罕见的来源于横纹肌的良性肿瘤,按其发生部位分为心脏横纹肌瘤与心外横纹肌瘤。心外横纹肌瘤90%发生在头颈部。根据肿瘤细胞的分化程度,心外横纹肌瘤分为成人型横纹肌瘤、胎儿型横纹肌瘤与生殖器型横纹肌瘤。头颈部心外横纹肌瘤在临床上主要表现为缓慢生长的无痛性包块,包块呈单发或多发,病程可达数年。组织学检查是诊断横纹肌瘤的主要方法,胞浆中的横纹结构与肌源性蛋白的特殊染色是诊断横纹肌瘤的主要依据。手术切除是治疗头颈部心外横纹肌瘤的有效方法。

Rhabdomyoma （RM） is a rare benign mesenchymal tumour with skeletal muscle differentiation that is classified into cardiac and extracardiac types based on location. 90% of the extracardiac RM occurs in the head and neck region. Extracardiac RM is further classified into adult, fetal and genital types, depending on the degree of differentiation. The main clinical features of the extracardiac RM in the head and neck is a single or multiple slowly growing asymptomatic mass, the course can be up to several years. Histological examination is the principal method for the diagnosis of the extracardiac RM, Cytoplasmic cross striations identified with PAH and the strongly positive of the muscle specific actin and desmin identified with immunohistochemical staining are the main evidence for diagnosis of the extracardiac RM. Complete excision is the recommended treatment.