CIinicaI anaIysis of 12 rhabdomyosarcoma chiIdren with intracraniaI extension / 中华实用儿科临床杂志

ABSTRACT

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 wkrk6 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.