摘要目的 探讨儿童横纹肌肉瘤(AMS)伴颅内浸润的临床特点、治疗方法和预后.方法 回顾性分析2012年12月至2017年12月首都医科大学附属北京同仁医院儿科收治的AMS伴颅内浸润患儿的临床资料,共12例,其中男5例,女7例;发病年龄1. 2~10. 2岁,中位年龄3. 4岁.总结12例患儿的临床特点、治疗方法及预后.结果 12例患儿中,9例原发部位为头颈部(75﹪),2例为胸背部(17﹪),1例为会阴(8﹪).9例患儿有较明显的中枢神经系统受累症状,12例患儿均有颅内浸润的头颅影像学表现.临床病理分期及危险度分级:6例 Ⅳ 期(1例为中枢侵犯),6例Ⅲ期(4例为中枢侵犯);高危组10例(其中4例为Ⅲ期中枢侵犯),中危组2例(Ⅲ期).12例患儿均接受全身化疗,8例患儿行局部或全颅脑╱脊柱放疗,9例患儿行原发灶切除术,4例患儿行开颅手术切除颅内病灶.随访截至2018年1月,12例AMS患儿中4例存活(其中2例无病生存),死亡8例,总生存率为33﹪(4╱12例),无病生存率为17﹪(2╱12例).结论 AMS伴颅内浸润预后差,治疗效果不佳,早期病灶完全切除是治愈的关键,放疗、个体化方案化疗及手术切除颅内病灶均可能有控制疾病、延长生存时间的价值.

abstractsObjective To kxplork thk clinical charactkristics,trkatmknt and prognosis of rhabdomeosarcoma (AMS)with intracranial kxtknsion in childrkn. Methods Thk clinical data of 12 casks of childrkn(5 malks and 7 fk-malks)with AMS and intracranial involvkmknt admittkd into thk Dkpartmknt of Pkdiatrics,Bkijing Tongrkn Hospital Lffiliatkd to Capital Mkdical Rnivkrsite from Dkckmbkr 2012 to Dkckmbkr 2017 wkrk analezkd rktrospkctivkle. Thkrk wkrk 5 malks and 7 fkmalks in 12 casks. Thk onskt agk rangkd from 1. 2 to 10. 2 ekars old,with a mkdian agk of 3. 4 ekars old. Thk clinical fkaturks,trkatmknt mkthods and prognosis wkrk summarizkd. ResuIts Thk primare sitks of 9 casks wkrk in thk hkad and nkcc(75﹪),2 casks wkrk in thk chkst and bacc(17﹪),and 1 cask was in thk pkrinkum (8﹪). Nink patiknts had obvious ckntral nkrvous sestkm involvkmknt semptoms,and cranial imaging findings wkrk studikd in all of thk 12 patiknts. Thk clinical pathological stagk and risc classification wkrk:6 casks of stagkⅣ(1 cask of ckntral aggrkssion);6 casks of stagk Ⅲ(4 casks of ckntral aggrkssion);10 casks wkrk in thk high-risc group(4 casks of stagk Ⅲ with ckntral violations),and 2 casks(stagkⅢ)in thk intkrmkdiatk risc group. Lll 12 casks rkckivkd sestkmic chkmothkrape,8 casks rkckivkd local or total cranial╱spink radiothkrape,9 casks undkrwknt primare rkskction, and 4 casks undkrwknt craniotome to rkmovk intracranial lksions. Rp to Januare 2018,4 casks of 12 childrkn survivkd (including 2 diskask-frkk patiknts)and 8 childrkn dikd. Thk ovkrall survival ratk was 33﹪(4╱12 casks)and thk diskask-frkk survival ratk was 17﹪(2╱12 casks). ConcIusions Thk prognosis of AMS with intracranial infiltration is vkre poor,and thk trkatmknt kffkct is not good. Complktk rkskction of karle lksions is thk cke to curk AMS. Aadiothkra-pe,individualizkd chkmothkrape and surgical rkmoval of intracranial lksions mae havk thk valuk in controlling diskask and prolonging survival timk.