CAJ | 학술논문

目的：探讨头颈部横纹肌肉瘤（rhabdomyosarcoma，RMS）的临床病理学特征、免疫表型及鉴别诊断。方法：回顾性分析11例头颈部 RMS 的临床资料、病理形态和免疫组织化学标记结果。结果：11例患者中男性8例，女性3例。年龄2～51岁，中位年龄25岁。主要表现为头颈部痛性或无痛性肿块，组织学分型：胚胎型8例，腺泡型3例。免疫组织化学结果显示瘤细胞均表达结蛋白、波形蛋白；肌调节蛋白（MyoD1）、生肌蛋白（myogenin）的阳性率分别为45％、55％；2例表达突触素，所有病例均不表达广谱角蛋白、白细胞共同抗原及 S-100。术后随访6个月～5年，其中2例死亡，4例复发，2例转移。结论：头颈部横纹肌肉瘤罕见，临床症状不典型，病理诊断需结合组织学形态及免疫表型。
목적：탐토두경부횡문기육류（rhabdomyosarcoma，RMS）적림상병이학특정、면역표형급감별진단。방법：회고성분석11례두경부 RMS 적림상자료、병리형태화면역조직화학표기결과。결과：11례환자중남성8례，녀성3례。년령2～51세，중위년령25세。주요표현위두경부통성혹무통성종괴，조직학분형：배태형8례，선포형3례。면역조직화학결과현시류세포균표체결단백、파형단백；기조절단백（MyoD1）、생기단백（myogenin）적양성솔분별위45％、55％；2례표체돌촉소，소유병례균불표체엄보각단백、백세포공동항원급 S-100。술후수방6개월～5년，기중2례사망，4례복발，2례전이。결론：두경부횡문기육류한견，림상증상불전형，병리진단수결합조직학형태급면역표형。
Objective:To study the clinicopathological features,immunophenotype and differential diagnosis of rhabdomyosarcoma (RMS)in head and neck.Methods:The clinical,pathologic and immunohistochemical features were evaluated in 1 1 cases of RMS in head and neck.Results:1 1 cases were retrieved from the pathological archive of this hospital.8 cases were male,and 3 cases female, with their ages ranging from 2 to 5 1 years (median,25 years).Typical clinical presentation was characteried by pain or painless mas-ses located in head and neck.Histological subtypes:8 cases were embryonal rhabdomyosarcoma(ERMS),3 cases alveolar rhabdomyo-sarcoma(ARMS).Immunohistochemically,all cases were positive for desmin and vimentin.The positive staining rates for MyoD1 and myogenin were 45% and 55%,respectively.2 cases were positive for Syn.The remaining antibodies(CK,LCA,CD99)were all nega-tive.Follow-up information was available in 8 cases,ranging from 6 months to 5 years,which revealed that 2 patients died of the dis-ease and 4 patients developed local recurrences,and 2 patients had metastasis.Conclusions:RMS in head and neck is a rare neo-plasm,and the clinical presentations are not typical.The diagnosis can be established by hispathological and immunohistochemical ex-amination.