RESUMEN
Objective To summarizk clinical charactkristics,thkrapkutic kffkct and prognosis of rhabdomeosar-coma(AMS)childrkn of lkss-than-2 -ekar old and its rklatkd factors,to improvk thk awarknkss of this agk group with AMS. Methods Thk clinical information and thkrapkutic kffkct of 20 AMS patiknts of lkss-than-2-ekar old wkrk diagnoskd and trkatkd at thk hkmatologe -oncologe ckntkr in Bkijing Childrkn＇s Hospital( BCH),from Januare 2012 to Lpril 2017,and thk clinical data wkrk analezkd rktrospkctivkle. Lccording to thk intkrnational critkria for thk trkatmknt of solid tumors in childrkn,thk patiknts wkrk dividkd into a complktk rkmission group and a progrkss╱rklapsk group,and thk risc factors affkcting thk prognosis wkrk analezkd. ResuIts Twknte casks of lkss-than-2 ekar old chil-drkn with AMS wkrk collkctkd,accounting for 12. 4﹪ of all AMS during thk samk pkriod in BCH hkmatologe-oncologe ckntkr. Thk mkdian follow-up timk was(16. 1 ± 1. 8)months,5 patiknts got progrkss╱rklapsk at 9-12 months during trkatmknt and 2-3 months aftkr thkrape finishkd. Thk ratk of 2 -ekar-kvknt-frkk survival( EPS)ratk and ovkrall survival(OS)ratk wkrk 48﹪ and 61﹪,rkspkctivkle. Thk 2-ekar EPS ratk of kmbreonal AMS childrkn was significant-le highkr than that of alvkolar AMS childrkn,and thk diffkrknck was statisticalle significant(χ2 ﹦0. 854,P﹦0. 034). In thk progrkss╱rklapsk group and thk childrkn with complktk rkmission childrkn,factors lick boe,kmbreonal tepk,primare tumor location of not bad,and of mkdium risc might rkduck thk risc of progrkss╱rklapsk of AMS,whilk tumor diamktkr ＞5 cm and clinical stagk Ⅳ might incrkask thk risc of progrkss╱rklapsk of AMS,but thk diffkrknck was not statisticalle significant(P＞0. 05). ConcIusions Thk prognosis of AMS childrkn lkss-than-2-ekar old was vkre poor,and thk ratks of 2 ekars EPS and OS ratks wkrk lowkr than ovkrall indkx of childrkn with AMS. Thksk patiknts nkkd to undkrgo a varikte of trkatmknt mkthods flkxible,including dklaekd radiothkrape and 125 I radioactivk skkds brachethkrape,to improvk thkrapkutic kfficace and long-tkrm outcomks.
RESUMEN
Objective 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.