
pediatric ependemoma

pediatric ependimomakal swabhaavathil ependemomayude muthirnna roopathinu samaanamaan, avaa ventricular sisttathil lining cheiyunna radial gliel cellukalil ninnu undaakumenn karuthappedunnu. ennirunnaalum, jeenukaleyum chromosomukaleyum palappozhum badhikkunna muthirnna ependimomakalil ninnum avaa palappozhum kaanapedunna thalachorinte pradeshathu ninnum rogikalude roganirnayam ennivayil ninnum vyathyasthamaan. neurophybromatosis type II (enn. eph. 2) polulla chila paarambarya rogangalulla kuttikalkk eee tharam tumours kooduthalaayi baadikkunnathaayi kandethiyittundengil oru uracha janithaka bandham sthaapikkappedaanundu. chharddi, thalavedana, prakopippikkal, alasata, nadatthatthile mattangal ennivayulppede mattu niravadhi pediatric braine tumourukalude lakshanagal pole pediatric ependimomakalude vikaasavumaayi bandhapetta lakshanagal vyathyasthamaan. cheria kuttikalum aakramanathmaka tumour tharangalulla kuttikalum saadhaaranayaayi anukoolamaaya phalangal anubhavikkunnillenkilum, tumours poornnamaayum neekkam cheiyunnathu atijeevanathinum aavarthanathinum ettavum prakadamaaya roganirnaya gadakamaanu.
adisthaana jeevashasthram.
uthbhava koshangal.
radial gliel koshangalil ninnaan ependimomakal undaakunnathenn vishwasikkappedunnu. ependimomakalil ninnu uruthirinja tumersphereukal neuronal stem sell markerukal sidi133, nesttin, radial gliel nirddishta markerukal aarsi 2, braine lipid binding protein (blbp/fabpy 7) enniva prakadippikkunna phenotype polulla radial-gliel pradarshippikkunnu. radial gliel swabhaavasavisheshathakalulla tumersphereukal oorthootoopik mouse senograafttukalil tumours undaakkunnu, ithu ependimomakalude uthbhava koshamaayi radial glialine soochippikkunnu.
anantharaavakaasham.
neurophybromatosis type II (enf 2), turcott syndrome bii, menz 1 syndrome ennivayulppede niravadhi janithaka syndrokal ependemomayude vikaasavumaayi bandhappettirikkunnu. ennirunnaalum, famili syndromsumaayi bandhapetta jeane mutationukal ependemomayude edaykkideyulla kesukalil apoorvamaayi maathrame kaanappedunnulloo. udaaharanathinu, ependimomakalil enf 2 mutationukal apoorvvamaayi maathrame nireekshikkappettitlu, koodaathe menn 1 mutationukal ependemoma aavarthanangalude cheria kesukalil maathrame kandethiyittulloo.
oongoojenik murivukal.
"erbb2", "erbb4", "human telomerase reverse traanscriptes (" "tert" ") jeane expression enniva tumour sell vyaapanathe prolsaahippikkukayum aakramanathmaka tumour swabhaavathinu kaaranamaakukayum cheiyunnu". epidermal groth factor resepterinte ("iii. jii. eph. aarr") uyarnna aavishkaaram prathikoolamaaya falavumaayi bandhappettirikkunnu. kainettokor proteinukalude amithamaya aavishkaaravum mettalothiyoninukalude daun-regulation ependimomakalude aavarthanavumaayi bandhappettirikkunnu. kitt risapttar tyrosine kines phospho-kittum pediatric ependimomakalil undennu kandethi, aaa tumourukalumaayi bandhapetta angiogenesisil ithu ulppettirikkaam.
chromosomal mattangal.
praayapoorthiyaaya ependimomakalil kaanaatha niravadhi genomic vaikalyangal pediatric ependimomakalil undennu thaarathamya genomic hybridisation (cgh) pareekshanangal theliyichittundu. koodaathe, kendra naadeevyavasthaykkulle vividha sthalangalil ninnulla ependimomakale (nattellu, supertraattantoriyal, infraattantoriyal) avayude chromosomal, immunohistokemikkal, jeane expression vyathyaasangal upayogichu verthirichariyaan kazhiyum.
1q chromosominte amplification 6q, 17p, 22q ennivayude nashtavumaanu pediatric ependimomakalile ettavum saadhaaranamaaya samkhyaaparamaaya chromosomal mattangal. pediatric janasamkhyayil chromosome 1q (1q21.1-32.1) kooduthalaayi kaanapedunnu, ithu intra craniel ependimomakalile tumour aavarthanavumaayi bandhappettirikkunnu. koodaathe, aavarthana rahitavum mothatthilulla athijeevanathinumaayi chromosome 1q25-nte nettam swathanthramaaya roganirnaya moolyamullathaayi kandethiyittundu. eee sthalathu tumour suppressor jeeninte saannidhyathe pinthunaykkunna, edaykkideyullathum kudumbaparavumaaya kesukalil 22 kyu nashtam kandethiyittundu. ennirunnaalum, pediatric kesukalekkaal 22 kyu nashtam muthirnnavarude roopathilaanu kooduthalaayi kaanappedunnath. enf 2" 22q12.2 ll sthithicheyyunnathinaal, ithu ependemomayude vikasanathil ulppettirikkunnuvennu anumanikkappettu. enf 2" leeii mutationukal sushumna roopam ozhikeyulla edaykkideyulla ependimomakalil apoorvvamaayi maathrame kaanappedunnulloovenkilum, "enf 2" intacting jeenaaya "skip 1" pediatric ependimomakalil ganyamaayi niyanthrikkappedunnu, ithu ependimomakalude thudakkathile "enf 2" paathaykk oru punk vahikkunnu.
oongojinukalum tumour suppressor jeenukalum.
vividatharam oongojinukalum tumour suppressor jeenukalum pediatric ependimomakalil parivartthanam cheyyappedukayo allengil vyathiyanangal undaakukayo cheiyunnuvennu kandethiyittundu. kitt risapttar tyrosine kines phospho-kittum pediatric ependimomakalude vikasanathil oru punk vahikkumennu nirddeshikkappettittund, ekadesham 8 sathamaanam pediatric ependimomakalil "nochu 1" mutationukal kandethiyittundu, koodaathe "menn 1" mutationukal idaykkide pediatric ependimomakalil kaanapedunnu. "emm. emm. pii. 2", "emm. emm. pii. 14" ennivayum intra craniel kesukalil tumour valarchayilum purogathiyilum oru punk vahikkunnu. pediatric ependimomakalile saadhaaranayaayi maarunna chromosome pradeshangalil "tpr", "cbi 1" ennee rund candidate jeenukal thiricharinjittundu, chromosomukal 1 kyu 25, chromosome 22 kyu 12-kyu 13. chromosome 1 kyu-ll "ezz 100 eiii 6", "ezz 100 eiii 4" ennee rund adhika candidate jeenukalude aavishkaaravum supertraattantoriyal tumour vikasanathinum 3 vayassinu munbu sambhavikkunna tumorukalkkum anuyojyamaanennu kandethi, ennirunnaalum eee jeenukal ettiyolajiyil enthaanu vahikkunnathenn krithyamaayi vyakthamalla.
tumour purogathi.
radial gliel cellukalil ninnu ependimomakal undaakaamenn nirddeshikkappettittund, ithu ependimomakalude rogakaarithvathinu nochu, soonik hedge hogg (eshh), pii53 thudangiya neural stem sell attakuttappanikalude pathakal pradhaanamaanennu soochippikkunnu.
nochu signalling pathayum transcription gadakangalude hooks kudumbavum yathaakramam supertraattantoriyal, spinal ependimomakalil niyanthrikkappedunnu. nochu ligandukal, resepterukal, taargett jeenukal ("hess 1", "hayee 2", "mike") ennivayude amithamaya aavishkaaravum nochu repressorinte (efbxdablue 7) daun-regulation pediatric ependemomayil kaanapedunnu. nochu paatha thadayunnathu tumourinte valarchaye "inn vitro" badhikkunnu. nochu taargett erbb 2 mikka ependimomakalilum apa-regulated aanu, ithu mosham falavumaayi bandhappettirikkunnu.
pediatric ependemomayil pii53 ("tipi53") mutationukal palappozhum nireekshikkappedunnil, radiation therapy prathirodhatthilum tumour purogathiyilum pii53 paatha oru punk vahikkaan nirddeshikkappedunnu, orupakshe "mdm 2"-nte amithamaya aavishkaarathiloode. koodaathe, pii53-nte oru homologue aaya pii73 ("tipi73")-nte appregulation, pii53 pathway jeane pii14rf/pii16/ink4eiii ("cdnn2eiii") illaathaakkal ennivayum pediatric ependemomasil kandethiyittundu.
jii. aii. aii. 1", "jii. aii. aii 2", "ezz. tii. keey. 36" thudangiya ezz. echu. paathayude gadakangalude amithamaya aavishkaaram ependimomakalile ezz. echu. paathayude niyanthranam illaathaakkunnathine soochippikkunnu. koodaathe, ependemomayile "igfbp2", "igfbp3", "igfbp5" ennivayude amithamaya aavishkaaram pediatric ependimomakalude rogakaarikalil eshh, insulin polulla valarchaa ghadakam (igf) signalinginulla oru pankine soochippikkunnu.
purogathi nirakk.
endothelial sell kitt expression pilocytic aastrosaittoma allengil ependemomayude roganirnayathil cheruppavumaayi bandhappettirikkunnu. kuttikkaalatthe ependemomayil telomerase pravartthanam kaanapedunnu. koodaathe, telomerase veendum sajeevamaakkukayum telomiyarukalude paripalanam purogathikku aavashyamaayi varikayum cheiyunnu. telomerasinte prathipravartthana proteinaaya nucleolinte kuranja expression, phalatthinte ettavum pradhanappetta jaiva pravachanamaanennu kandethi, avide kuranja expression kooduthal anukoolamaaya roganirnayavumaayi bandhappettirikkunnu.
klinikkal biology.
avatharanam.
roganirnayathinu maasangalkk munbu rogalakshanangal <aii. dii. 1> avatharippikkunnu, praayam, tumour grade, sthaanam ennivaye aashrayich vyathyaasappedaam. vardhicha intracranial marddam chharddi, thalavedana, prakopippikkal, alasata, nadatthatthile mattangal, 2 vayassinu thaazheyulla kuttikalil, bhakshana prasnangal, aniyanthrithamaaya kanninte chalanagal, hydrocephalus enniva palappozhum sradheyamaanu. ekadesham 20 sathamaanam pediatric rogikalilum apasmaram sambhavikkunnu. sii. ezz. eph ozhukkinu nirnayakamaaya oru sthalathaanu tumour sthithicheyyunnathenkil vaijnjaanika pravartthanatthinte nashtavum pettennulla maranavum polum sambhavikkam. saadhaaranayaayi nattellil sambhavikkunna muthirnna ependimomakalil ninnu vyathyasthamayi pediatric ependimomakal palappozhum pinbhaagathe thalayotiyile fosayilaanu sambhavikkunnath. sidi skaanukalil kuranja saandrathayulla pindangalayi ependimomakal kaanapedunnu, koodaathe tii 2-waited emri chithrangalil hyperincense aanu.
pathology.
2007leeii vargeekaranathil subependimoma (grade I), mixopapillary ependemoma (grade I), ependemoma (grade II), anaplastic ependemoma (grade III) enniva praadhamika vargeekaranangalaayi pattikappeduthiyittundum ependimomakalude gradinginecurichu kaaryamaaya charchakal thudarunnu. eee vargeekarana padhathi ependemoma grouppinullile naalu upavibhaagangale kooduthal nirddeshikkunnu. ennirunnaalum, vyathyastha rogalakshanangalulla niravadhi amgeekritha upavibhagangalaaya ependimomakalundu. pathukke valarunnathum sushumna nadiyude conus medullaris-kauda ikvina-philam terminal mekhalayilekku parimithappeduthunnathumaa mixopapillary ependemoma (mepn), intra craniel, infraatteeriyal (posteeriyar phosa), intra craniel supertraatteeriyal, spinal ependemoma, subpendymomas enniva ithil ulppedunnu. tissue-specifixity efakttukalkku adisthaanamaaya tanmatra savisheshathakalkk lokkeshan adhishtitha vargeekaranam ettavum prasakthamaanennu reportukal theliyichittundu.
saadhaarana eppendymal koshangale cancer koshangalayi parivartthanam cheiyunna oongoojenik sambhavangalil ninnaan ependymomakal undaakunnath. praadhamika koshangal radial gliayaanennu sameepakaala thelivukal soochippikkunnu. histologicalaayi samanamaaya ependemoma tumerukalkkidayil janithaka mattangal valare vaividhyapoornamaanu.
roganirnaya savisheshathakal.
thaarathamya genomic hybridisation (sii. jii. echu) pareekshanangal kaanikkunnath, pediatric tumorukalkku muthirnna ependimomakalil kaanaatha niravadhi genomic apaakathakalundenn kaanikkunnu, koodaathe muzhuvan chromosome asanthulithaavasthayudeyum uyarnna vyaapanamund. mattu pediatric cns tumourukalil ninnu ependimomakale verthirichariyaan epithelial membran aantijan sahaayikkumenn theliyikkappettittund. neuraxis emr imaging, lumber csf saittolaji vilayiruthal enniva tumour vyaapanam nirnnayikkunnathinulla vyaapakamaayi amgeekarikkappetta reethikalaanu.
deferential diagnosis.
oru tumour undennu samshayikkappettal, meduloblastomas, defuse astrocytomas, pilocytic astrocytomas, ependemomas enniva posteeriyar phosa tumourukalaayi deferential diagnosisil thudarunnu. ennirunnaalum, gaalaktin-3 nuu pilocytic aastrocytomakalum ependimomakalum maathrame positteev aayi paadulloo. ependemomayude upavibhaagathe tanmatra maargangaliloode churukkanum kazhiyum. udaaharanathinu, mixopapillary ependiomakalkku "hoxby5", "plaa2jii5", "ittih2" ennivayude uyarnna padaprayogam undennu kandethiyittundu. transcription gadakangalude "soaks" kudumbatthile moonnu amgangalkku meduloblastomasum ependimomakalum thammil vivechanaparamaaya shakthiyundenn oru jeane expression profiling pareekshanam theliyichittundu. histolaji illaathe, magnetic resonance imajingil shareeraghadana vyathyaasangalillaathil grade II umm grade III anaplastic ependimomakalum verthirichariyaan prayaasamaan.
pravachana savisheshathakal.
pothuve, pediatric ependimomakal muthirnnavarude ependimomakalekkaal anukoolamallaatha roganirnayangalumaayi bandhappettirikkunnu, koodaathe cheruppakkaaraaya pediatric rogikalude ependimomakal prayamaaya pediatric rogikalude ependimomakalekkaal anukoolamalla (avalokanam cheythath). pinbhaagathe phosayil sambhavikkunna tumorukalkku anukoolamaaya roganirnayam kuravaanennu theliyikkappettittund. pediatric intracranial ependemomasil "tert" enna padaprayogam telomerase pravarthanavumaayum tumour purogathiyumaayum bandhappettirikkunnu, koodaathe athijeevanavumaayi prathikoolamaayi bandhappettirikkunnu. aarr. tii. keey-1 kudumbatthile amgangal parasparabandhitharallenkilum, pediatric ependemomayude kesukalil protein nucleolinum "emm. emm. pii. 2", "emm. emm. pii. 14" ennivayude aavishkaaravum purogathiyillaattha athijeevanavumaayi vipareethamaayi bandhappettirikkunnave kandethiyittundu. tumour microenvation, vividha imaging reethikal upayogichu nannayi verthirichariyaan kazhiyunna tumourukalil polum, tumourumaayi vipareethamaayi bandhappettirikkunnave kandethi.|purogathiyillaathathum mothatthilulla athijeevanavum. chromosome <aii. dii. 1> illaathaakkunnathu pediatric ependimomakalil athijeevanathinu adhika aanukoolyam nalkumennu chila thelivukal soochippikkunnu.
chikitsa.
pediatric ependimomakalkkulla chemotherapy rejimentukal mithamaaya prayojanam maathrame nalkiyittulloo, koodaathe vichedanam aavarthanathinum atijeevanathinum ettavum prakadamaaya gadakamaayi thudarunnu.
pediatric ependimomakalile mosham falavumaayulla "tert" eksprashante bandham chila gaveshakare telomerase inhibition pediatric ependimomakalkku phalapradamaaya anubandha chikilsayaayirikkaamenna nirddeshikkaan prerippichu. koodaathe, praadhamika tumour isolate cellukal upayogikkunna "inn vitro" pareekshanangalil ninnulla daatta soochippikkunnathu telomerase pravartthanatthinte nirodhanam koshangalude vyaapanathe thadayukayum praadhamika tumourukalile uyarnna telomerase pravartthanatthinte nireekshanavumaayi poruthappedunna dna nasippikkunna ejantukalodulla koshangalude samvedanakshamatha vardhippikkukayum cheiyumennaanu. koodaathe, pediatric ependimomakalil appurinic/apyrimedinic endonuclease ("aapu 1") vikirana prathirodham nalkunnuvennu kandethiyathinal, aapu endo pravartthanatthinte inhibittarukalkku vikirana samvedanakshamatha punasthaapikkaanum kazhiyumennu nirddeshikkappettittund.
pediatric ependimomakalude infraattantoriyal grouppinullil, radiotherapy 5 varshathe athijeevanam ganyamaayi vardhippikkunnathaayi kandethi. ennirunnaalum, stereotactic radiosurgery oru munkaala avalokanam, munbu vichedanam, vikiranam ennivaykku vidheyamaaya rogikalkku ithu mithamaaya prayojanam maathrame nalkiyittulloo ennu kaanichu. mattu superprotentariyal tumorukalkku mikacha roganirnayam undengilum, superprotentariyal anaplastic ependimomakal ettavum aakramanathmakamaaya ependemomayaanu, koodaathe poornnamaaya exitiono poost operative vikiranamo neratheyulla aavarthanam thadayunnathil phalapradamaanennu kandethiyitla.
infraattentoriyal ependimomakalude vichedanam kazhinjaal, radiological pree-oopparetteevaayi tharamthirickunna laattaral vezhsas medial tumourukalil sheshikkunna tumourinu kooduthal saadhyathayund. eee kesukalil itrogenic efakttukal ozhivaakkumbol poornnamaaya vichedanam nadatthaan sahaayikkunnathin cerebellomedullary fisher desection polulla nirddishta saankethikavidyakal nirddeshichittundu. aavarthanathinaayulla nireekshana neuro imaging rogikalkku nireekshanatthil maathram adhika athijeevanam nalkunnu.
biochemical markerukal.
roganirnayathinum chikithsayodulla prathikaranatthinum nirnaayaka adayaalangalaanu echtertum vaiechu2exum. uyarnna hert, kuranja YH2X expression enniva therappiyodulla mosham prathikaranavumayi bandhappettirikkunnu. eee adayaalangalude uyarnnatho thaazhnnatho aaya aavishkaramulla rogikal mithamaaya prathikarana grouppukalaayi maarunnu.
veendum aavarthikkuka.
5 vayassinu mukalilulla 5 varshathe rogarahitha athijeevanam 50-60% aanu. mattoru reportil samanamaaya 5 varshathe athijeevanam ekadesham 65 shathamaanamaanennum 51 sathamaanam purogathiyillaattha athijeevanamaanennum kandethi. 10 varshathe rogarahitha athijeevanam 40-50% aanu. cheruppakkaar 5,10 varshathe athijeevana nirakk kuranju. 133 rogikale nireekshicha 2006 leeii oru padanatthil anchu varshathinullil 31 perkku (23.3%) rogam aavarthichathayi kandethi.
chikilsayude deerghakaala prathyaaghaathangal.
mattu keemotheraappikalumaayi thaarathamyappeduthumbol emettalstaatt polulla telomerase inhibittarukalude upayogam valare kuranja vishaamshamullathaayi thonnunnu. mikka telomerase inhibittarukaludeyum ariyappedunna oreyoru paarshwafalamaanu dossil ninnu undaakunna neutropenia. neuro psychological kammi vichedanam, chemotherapy, radiation, endocrinopathy enniva moolavum undakam. koodaathe, pediatric cancerine athijeevichavaril dahananaala sankeernathakalil vardhanavu nireekshikkappettundu.

