39例儿童头颈部横纹肌肉瘤临床特点及治疗转归

Clinical features of 39 children with head and neck rhabdomysarcoma in a single medical center,and treatment outcomes

目的探讨儿童头颈部横纹肌肉瘤的(RMS)临床特点及治疗转归。方法回顾性分析北京同仁医院2004年11月至2010年11月收治的39例原发于头颈部的RMS患儿临床资料及治疗随访结果,其中男23例,女16例,年龄3个月至14岁,中位年龄6.0岁。结果 39例患儿临床主要表现为眼球突出及眼睑肿胀(56%,22/39),鼻塞及鼻出血占28%(11/39),面颊包块占15%(6/39)。39例患儿中,原发部位以眼睑及眼眶为主(56%,22/39),其次为鼻咽部及筛窦(28%,11/39)。37例有明确病理分型,大多数患儿为胚胎型(89%,33/37)。39例患儿中位随访时间为38个月(10～80个月),放弃治疗4例,获随访35例。35例患儿中,行单纯手术4例,单纯化疗1例,手术+化疗12例,手术+放疗2例,手术+化疗+放疗13例(其中8例行125I放射性粒子植入术治疗),手术+化疗+放疗+自体外周血造血干细胞移植(APBSCT)2例,化疗+APBSCT 1例。其中7例复发,5例死亡,总生存率86%(30/35),完全缓解率达66%(23/35),部分缓解率达20%(7/35),8例粒子植入术治疗患儿6例达无瘤生存。结论 RMS患儿临床表现主要为眼球突出及眼睑肿胀;眼部及鼻咽是儿童头颈部RMS多发部位;病理分型以胚胎型为主;包括化疗、手术、125I粒子组织间植入及APBSCT等多种综合治疗方法可有效提高RMS患儿缓解率。

Objective To study the clinical features and treatment outcomes of head and neck rhabdomysarcoma（RMS） in children.Methods The clinical data and results of follow-up visits for 39 children with head and neck RMS were retrospectively reviewed.The children（23 males and 16 females） with a median age of 6 years old（ranged 3 months to 14 years） were admitted to the Beijing Tongren Hospital between November,2004 and November,2010.Results The 39 children mainly presented with exophthalmos and eyelid swelling（56%,22/39）,rhinostegnosis and nasal bleeding（28%,11/39） and check mass（15%,6/39）.Common primary sites were the eyelid and orbit（56%,22/39）,followed by the nasopharynx and ethmoid antrum（28%,11/39）.Thirty-seven of the 39 patients showed a definite pathologic type and the embryo type was the most common（89%,33/37）.Follow-up visits were carried out for 35 children,with a median follow-up time of 38 months（10-80 months）.Of the 35 children,4 cases received surgery alone,1 case received chemotherapy alone,12 cases received surgery plus chemotherapy,2 cases received surgery plus radiochemotherapy,13 cases received surgery,chemotherapy and radiochemotherapy（8 cases received 125I particles implants）,2 cases received surgery,chemotherapy,radiochemotherapy and autologous peripheral blood stem cells transplantation（APBSCT）,and 1 case received chemotherapy and APBSCT.Seven cases relapsed and 5 cases died of brain metastasis.The total survival rate was 86%（30/35）,the complete remission rate was 66%（23/35）,and the partial remission rate was 20%.In the 8 cases receiving 125I particles implants,6 survived without tumor.Conclusions Exophthalmos and eyelid swelling are the main presentations in children with head and neck RMS.Common primary sites of this disease are the eye and nasopharynx.The most common pathologic type is embryo type.Comprehensive treatment,including chemotherapy,surgery,125I particles implants and APBSCT therapy,can improve outcome.