郭慧　谭丽珊　吴立琦

【摘要】 目的：探討鼻腔鼻窦横纹肌肉瘤的临床病理特征、镜下形态特点、免疫表型，并复习文献探讨其诊断、鉴别诊断的要点、组织发生及其生物学行为。方法：所选病例为2011年2月-2016年2月韶关市第一人民医院收治的鼻腔鼻窦横纹肌肉瘤患者2例，均经病理检查、免疫组化检查确诊为鼻腔鼻窦横纹肌肉瘤。采用回顾性分析的方法，对患者一般资料进行调查。结果：1例40岁（女），以右侧鼻塞伴有流涕一月余为主诉入院；1例58岁（女），以右侧鼻塞，痰中带血5月余为主诉入院。2例入院后均实施血常规、生化全套、鼻腔CT平扫、MRI等检查。病理检查显示2例均为腺泡状横纹肌肉瘤。2例均实施鼻内窥镜切除肿物、放疗、化疗等综合治疗。随访13～64个月，1例出现颅内转移，死亡；1例无其它脏器转移，目前生存状况良好。镜下1例肿瘤细胞被纤维间隔成灶状和小条索状排列，1例细胞密集分布于黏膜下，部分细胞透亮。免疫组化示肿瘤细胞desmin，myoglobin，MyoD1，myogenin均（+），NSE，CgA，Syn，S-100，HMB45，CK，LCA均（-）。结论：鼻腔鼻窦横纹肌肉瘤早期临床症状不典型，误诊、漏诊率高，且病情进展快，预后差，需采取积极措施，进行尽早诊断、尽早治疗。

【关键词】 鼻腔鼻窦； 横纹肌肉瘤； 病理分析； 免疫组化

【Abstract】 Objective：To investigate the clinical and pathological features，microscopic appearance and immunophenotype of rhabdomyosarcoma in sinonasal cavity，and to review the literature to explore the diagnosis，distinguish diagnosis，histogenesis and biological behavior.Method：From February 2011 to February 2016，2 patients with rhabdomyosarcoma in sinonasal cavity were admitted to Shaoguan city first people's Hospital and were confirmed by pathological examination and immunohistochemical examination.By retrospective analysis method，the general data of patients were investigated.Result：1 case of 40 years old（female），right nasal obstruction and runny nose over January as the chief complaint of admission；1 case at the age of 58（female），stuffy and blood in the sputum on the right nose for 5 months as the chief complaint of admission.2 cases after admission underwent the implementation of a full set of blood routine，biochemical，nasal CT scan，MRI examination.The pathological examination showed that 2 cases were alveolar rhabdomyosarcoma.2 tumor cases were implemented endoscopic tumor resection，radiotherapy，chemotherapy and other comprehensive treatment.The follow-up of 13-64 months，1 case was intracranial metastasis and death，the survival condition of 1 case without metastasis was good.Microscopic tumor cells of 1 case were fibrous septa into focal and small cords，1 case cells distributed in mucosa，some cells bright.Immunohistochemical staining showed that tumor cells desmin，myoglobin，MyoD1，myogenin were all（+），NSE，CgA，Syn，S-100，HMB45，CK，LCA were all（-）.Conclusion：nasal rhabdomyosarcoma has early atypical clinical symptoms，high misdiagnosis and misdiagnosis rate，rapid progression and poor prognosis，positive measures should be taken for diagnosis and treatment as soon as possible.

【Key words】 Nasal cavity and paranasal sinuses； Rhabdomyosarcoma； Pathological analysis； Immunohistochemistryendprint

First-authors address：The First Peoples Hospital of Shaoguan，Shaoguan 512000，China

doi：10.3969/j.issn.1674-4985.2017.25.034

作为临床上一种常见软组织恶性肿瘤：横纹肌肉瘤的患病率较高，多发于儿童及青少年[1]。一般来说，该病主要来自胚胎间叶组织，多发于头颈部及泌尿系统。鼻腔、鼻窦横纹肌肉瘤在临床上较为常见，且该部位发病较为隐匿，加上早期临床症状不典型，极易造成误诊、漏诊，影响治疗效果及预后[2]。……