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Unyango lwe-Ewing Sarcoma

Ulwazi ngokubanzi malunga ne-Ewing Sarcoma

IINGONGOMA EZIPHAMBILI

• Ukuthenga i-sarcoma luhlobo lwethumba elenza kwithambo okanye kwizicubu ezithambileyo.

• I-cell sarcoma engafaniyo enokuvela kwisithambo okanye kwithishu ethambileyo.

• Iimpawu kunye neempawu ze-Ewing sarcoma zibandakanya ukudumba kunye nentlungu kufutshane nethumba.

• Uvavanyo oluvavanya ithambo kunye nezicubu ezithambileyo zisetyenziselwa ukufumanisa isifo kunye nenqanaba le-Ewing sarcoma.

• I-biopsy yenziwa ukufumanisa i-Ewing sarcoma.

• Izinto ezithile zichaphazela isifo (ithuba lokuchacha kwakhona).

Ukuthenga i-sarcoma luhlobo lwethumba elenza kwithambo okanye kwizicubu ezithambileyo.

I-Earc sarcoma luhlobo lwesisu esivela kuhlobo oluthile lweseli kwithambo okanye kwithishu ethambileyo. Ukufumana i-sarcoma kunokufumaneka kumathambo emilenze, iingalo, iinyawo, izandla, isifuba, i-pelvis, umqolo, okanye ukhakhayi. I-Earc sarcoma nayo ingafumaneka kwithishu ethambileyo yomthi, iingalo, imilenze, intloko, intamo, i-retroperitoneum (indawo ngasemva kwesisu ngasemva kwethishu ebopha udonga lwesisu kwaye igubungela uninzi lwamalungu esiswini), okanye ezinye iindawo.

I-Earc sarcoma ixhaphake kakhulu kulutsha nakubantu abadala abancinci (ulutsha ukuya kwiminyaka ye-20).

I-Ewing sarcoma ikwabizwa ngokuba yipheripheral primitive neuroectodermal tumor, ithumbu le-Askin (Ewing sarcoma eludongeni lwesifuba), i-extrawingseous Ewing sarcoma (Ewing sarcoma in tissue ngaphandle kwethambo), kunye ne-Ewing sarcoma family of tumors.

I-cell sarcoma engafaniyo enokuvela kwisithambo okanye kwithishu ethambileyo.

I-cell sarcoma engafaniyo ejikelezayo ihlala isenzeka emathanjeni okanye kwimisipha encanyathiselwe emathanjeni kwaye inceda umzimba ukuba uhambe. Zimbini iintlobo zeselfoma ejikeleze iiseli ezingaphathwa njenge-Ewing sarcoma:

• I-cell sarcoma engafaniyo kunye ne-BCOR-CCNB3 yokulungelelaniswa kwakhona. Olu hlobo lwethumba lethambo lidla ngokwenzeka esinqeni, kwiingalo, okanye emilenzeni. Inganwenwela nakwamanye amalungu omzimba. Kolu hlobo lwe-sarcoma ejikeleze iseli, i-BCOR yemfuza idityaniswe kwi-CCNB3 yemfuza. Ukufumanisa isifo se-sarcoma ejikelezileyo, iiseli zethumba zijongwa kolu tshintsho.

• I-sarcoma yeseli ejikeleze ngokungafaniyo kunye nokulungiswa kwakhona kweCIC-DUX4. Olu hlobo lwethumba elithambileyo leethisisi luqhele ukwenzeka esiqwini, ezingalweni, okanye emilenzeni. Ixhaphake kakhulu emadodeni nakubantu abadala abancinci abaphakathi kweminyaka engama-21 nama-40 ubudala. Kolu hlobo lwe-sarcoma ejikeleze iseli, uhlobo lweCIC ludityaniswe kuhlobo lwe-DUX4. Ukufumanisa isifo se-sarcoma ejikelezileyo, iiseli zethumba zijongwa kolu tshintsho.

Iimpawu kunye neempawu ze-Ewing sarcoma zibandakanya ukudumba kunye nentlungu kufutshane nethumba.

Ezi kunye nezinye iimpawu kunye neempawu zinokubangelwa yi-Ewing sarcoma okanye ngezinye iimeko. Jonga kugqirha womntwana wakho ukuba umntwana wakho unayo nayiphi na kwezi zinto zilandelayo:

• Intlungu kunye / okanye ukudumba, zihlala ezingalweni, emilenzeni, esifubeni, ngasemva, okanye kwi-pelvis.

• Iqhuma (elinokuziva lithambile kwaye lifudumele) ezingalweni, emilenzeni, esifubeni, okanye esinqeni.

• Umkhuhlane ngaphandle kwesizathu esaziwayo.

• Ithambo elophuka ngaphandle kwesizathu esaziwayo.

Uvavanyo oluvavanya ithambo kunye nezicubu ezithambileyo zisetyenziselwa ukufumanisa isifo kunye nenqanaba le-Ewing sarcoma.

Iinkqubo ezenza imifanekiso yamathambo kunye nezicubu ezithambileyo kunye neendawo ezikufutshane zinceda ukufumanisa i-Ewing sarcoma kwaye zibonise ukuba umhlaza usasazeke kangakanani. Inkqubo esetyenziselwa ukufumanisa ukuba iiseli zomhlaza zisasazekile ngaphakathi nangaphakathi kwamathambo kunye nezicubu ezithambileyo okanye kwamanye amalungu omzimba kuthiwa yistage.

Ukucwangcisa unyango, kubalulekile ukwazi ukuba umhlaza unwenwele kwamanye amalungu omzimba. Uvavanyo kunye neenkqubo zokufumanisa, ukufumanisa isifo, kunye nenqanaba le-Ewing sarcoma zihlala zenziwa ngaxeshanye.

Ezi mvavanyo zilandelayo kunye neenkqubo zinokusetyenziselwa ukufumanisa isifo okanye isigaba se-Ewing sarcoma:

• Uvavanyo lomzimba kunye nembali: Uvavanyo lomzimba ukukhangela iimpawu zempilo ngokubanzi, kubandakanya nokujonga iimpawu zesifo, ezinje ngamaqhuma okanye nayiphi na into ebonakala ingaqhelekanga. Imbali yemikhwa yempilo yesigulana kunye nezifo zangaphambili kunye nonyango ziya kuthathwa.

• I-MRI (imaging resonance imaging): Inkqubo esebenzisa umazibuthe, amaza erediyo kunye nekhompyuter ukwenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni, ezinje ngendawo elakhiwe kuyo eli thumba. Le nkqubo ikwabizwa ngokuba yimifanekiso yenyukliya yokujonga umfanekiso (i-NMRI).

Imagnetic resonance imaging (MRI) yesisu. Umntwana ulele phezu kwetafile etyibilika kwisikena se-MRI, esithatha imifanekiso engaphakathi komzimba. Iphedi esiswini somntwana inceda ukwenza imifanekiso icace gca.

• Ukuskena i-CT (ukuskena i-CAT): Inkqubo eyenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni, ezinje ngendawo apho lakhiwe khona ithumba okanye isifuba, sithathwe kwiimbombo ezahlukeneyo. Imifanekiso yenziwe yikhompyuter edityaniswe kumatshini we-x-ray. Idayi inokufakwa ngaphakathi emthanjeni okanye iginywe ukunceda amalungu okanye izicwili zibonise ngokucacileyo. Le nkqubo ikwabizwa ngokuba yi-computed tomography, ikhompyuter ye-tomography, okanye ikhompyuter ye-axial tomography.

Ikhompyuter ye-tomography (CT) yesisu. Umntwana ulele phezu kwetafile etyibilika ngesikena se-CT, esithatha imifanekiso ye-x-ray engaphakathi esiswini.

• Ukuskena i-PET (positron emission tomography scan) Inkqubo yokufumana iiseli zethumba ezinobungozi emzimbeni. Inani elincinci leswekile ene-radioactive (iswekile) itofelwe emthanjeni. Iskena se-PET sijikeleza ujikeleze umzimba kwaye senze umfanekiso wendawo esetyenziswa kuyo iswekile emzimbeni. Iiseli zethumba ezinobungozi zibonakala ziqaqambile emfanekisweni kuba ziyasebenza kwaye zithatha iswekile eninzi kuneseli eziqhelekileyo. Iskena se-PET kunye ne-CT scan zihlala zisenziwa ngexesha elinye. Ukuba kukho nawuphi na umhlaza, oku kwandisa amathuba okuba kufumaneke.

Ukuskena i-Positron emission tomography (PET). Umntwana ulele phezu kwetafile etyibilika kwisikena sePET. Ukuphumla kwentloko kunye nomtya omhlophe kunceda umntwana ukuba alale. Inani elincinci leswekile ene-radioactive (iswekile) itofwe emthanjeni womntwana, kwaye iskena senza umfanekiso apho iswekile isetyenziswa khona emzimbeni. Iiseli zomhlaza zibonakala ziqaqambile emfanekisweni kuba zithatha iswekile eninzi kuneseli eziqhelekileyo.

• Ukuskena amathambo: Inkqubo yokujonga ukuba ngaba kukho iiseli ezahlula ngokukhawuleza, ezinje ngeeseli zomhlaza, emathanjeni. Inani elincinci kakhulu lezinto ezisasazeka ngemitha lifakwa emthanjeni kwaye lihamba ngokuhamba kwegazi. Izinto ezinemitha eqhumayo ziqokelela emathanjeni anomhlaza kwaye zifunyenwe siskena.

Ukuskena amathambo. Inani elincinci lezinto ezinemitha eqhumayo lifakwe kumthambo womntwana kwaye lihamba ngegazi. Izinto ezinemitha yeathomathizi eziqokelela emathanjeni. Njengokuba umntwana elele phezu kwetafile eslayidayo phantsi kweskena, kufunyanwa into enemitha ye-radio kunye nemifanekiso yenziwe kwikhompyuter.

• Umnqweno wethambo kunye ne-biopsy: Ukususwa komongo wethambo kunye neqhekeza elincinci lethambo ngokufaka inaliti engenanto kumqolo. Iisampulu zisuswe kuwo omabini amathambo. Ugqirha wezifo ujonga umongo wethambo kunye nethambo phantsi kwemicroscope ukubona ukuba umhlaza usasazekile.

Umnqweno wethambo lomnqweno kunye ne-biopsy. Emva kokuba indawo encinci yolusu iphonyiwe, inaliti yomongo wethambo ifakwa kwithambo lesinqe somntwana. Iisampulu zegazi, ithambo, kunye nomongo wethambo ziyasuswa ukuze zihlolwe phantsi kwemicroscope.

• IX-reyi: IX-reyi luhlobo lombane wamandla onokuthi uhambe emzimbeni nakwifilimu, wenze umfanekiso weendawo ezingaphakathi emzimbeni, ezinje ngesifuba okanye indawo eyakhele ithumba.

• Gcwalisa ubalo lwegazi (i-CBC): Inkqubo apho kuthathwa khona isampulu yegazi kwaye ihlolwe oku kulandelayo:

• Inani leeseli ezibomvu zegazi, iiseli ezimhlophe zegazi kunye neeplatelets.

• Isixa sehemoglobin (iprotein ethwala ioksijini) kwiiseli ezibomvu zegazi.

• Inxalenye yesampulu yegazi eyenziwe ziiseli ezibomvu zegazi.

• Izifundo ze-chemistry yegazi: Inkqubo apho kuvavanywa isampulu yegazi ukulinganisa izixa zezinto ezithile, ezinjenge-lactate dehydrogenase (LDH), ekhutshelwa egazini ngamalungu kunye nezicubu zomzimba. Inani elingaqhelekanga (eliphezulu okanye elisezantsi kunesiqhelo) lwento elinokuba ngumqondiso wesifo.

I-biopsy yenziwa ukufumanisa i-Ewing sarcoma.

Iisampulu zamathambo ziyasuswa ngexesha le-biopsy ukuze zibonwe phantsi kwemicroscope ngugqirha wezilwanyana ukujonga iimpawu zomhlaza. Kuyanceda ukuba i-biopsy yenziwa kwiziko elinye apho unyango luya kunikwa khona.

• I-biopsy yenaliti : Ukwenza ialiti biopsy, izicubu ziyasuswa kusetyenziswa inaliti. Olu hlobo lwe-biopsy lunokwenziwa ukuba kunokwenzeka ukuba kususwe iisampulu zeethishu ezinkulu ngokwaneleyo ukuba zisetyenziswe kuvavanyo.

• I-biopsy ye-incisional : Kwi-biopsy ye-incisional, isampuli yethishu isuswe ngokusikwa kulusu.

• I-biopsy ekhethekileyo: Ukususwa kwesigaxa esipheleleyo okanye indawo yeethishu engabonakali njengeqhelekileyo.

Iingcali (i-pathologist, i-oncologist ye-radiation, kunye no-surgeon) oya kunyanga isigulana bahlala besebenza kunye ukwenza isigqibo ngeyona ndawo ibalaseleyo yokubeka inaliti okanye ukusikwa kwento. Ukukhethwa kwendawo ye-biopsy kubalulekile. Indawo ye-biopsy engakhethwanga ngokufanelekileyo inokubangela ukuba kwenziwe utyando olubanzi ukususa ithumba okanye indawo enkulu ephathwa ngonyango lwemitha.

Ukuba kukho ithuba lokuba umhlaza usasazeke kwii-lymph node ezikufutshane, inye okanye enye i-lymph node ingasuswa kwaye ihlolwe iimpawu zomhlaza.

Olu vavanyo lulandelayo lunokwenziwa kwizicubu ezisusiweyo:

• Uhlalutyo lwe-cytogenetic: Uvavanyo lwelebhu apho ii-chromosomes zeeseli kwisampulu yethishu zibalwa kwaye zijongwa kulo naluphi na utshintsho, njengokwaphuka, ukulahleka, ukulungiswa ngokutsha, okanye ii-chromosomes ezongezelelweyo. Utshintsho kwii-chromosomes ezithile kunokuba luphawu lomhlaza. Uhlalutyo lweCytogenetic lusetyenziselwa ukunceda ekuchongeni umhlaza, ukucwangcisa unyango, okanye ukufumanisa ukuba lusebenza njani unyango.

• I-Immunohistochemistry: Uvavanyo lwaselebhu olusebenzisa ii-antibodies ukukhangela ii-antigen ezithile (iimpawu) kwisampulu yesicwili somguli. Amachiza omzimba ahlala enxulunyaniswa ne-enzyme okanye idayi ye-fluorescent. Emva kokuba ii-antibodies zibophelela kwi-antigen ethile kwisampulu yethishu, i-enzyme okanye idayi iyasebenza, kwaye i-antigen iya kuthi emva koko ibonwe phantsi kwemicroscope. Olu hlobo lovavanyo lusetyenziselwa ukufumanisa isifo somhlaza kunye nokunceda uxelele olunye uhlobo lomhlaza kolunye uhlobo lomhlaza.

• Ukuhamba kwe-cytometry: Uvavanyo lwelebhu olulinganisa inani leeseli kwisampulu, ipesenti yeeseli eziphilayo kwisampulu, kunye neempawu ezithile zeeseli, ezinje ngobungakanani, imilo, kunye nobukho beempawu zethumba (okanye ezinye) umphezulu weseli. Iiseli ezivela kwisampulu yegazi lesigulana, umongo wethambo, okanye ezinye izicwili zinamabala edayi ye-fluorescent, ibekwe kulwelo, emva koko idlulise nganye nganye kwitampu yokukhanya. Iziphumo zovavanyo zisekwe kwindlela iiseli ezazinebala ledayi ye-fluorescent zisabela njani kwimbonakalo yokukhanya.

Izinto ezithile zichaphazela isifo (ithuba lokuchacha kwakhona).

Izinto ezichaphazela ukuxela kwangaphambili (ithuba lokuchacha) zahlukile ngaphambi nasemva konyango.

Ngaphambi kokuba kunikwe naluphi na unyango, ukuxela kwangaphambili kuxhomekeke:

• Nokuba ithumba lisasazekile kwii-lymph node okanye kwiindawo ezikude zomzimba.

• Apho emzimbeni laqala khona ithumba.

• Nokuba ithumba lenziwe kwithambo okanye kwithishu ethambileyo.

• Likhulu kangakanani ithumba xa kufunyaniswa ukuba eli thumba lifunyenwe.

• Nokuba eli thumba lenze nawaphi na amathambo aphukileyo.

• Nokuba inqanaba le-LDH egazini liphezulu kuneqhelekileyo.

• Nokuba ithumba lineenguqu ezithile zofuzo.

• Nokuba isigulana singaphantsi kweminyaka eli-15.

• Isini sesigulana.

• Nokuba isigulana sinonyango lomhlaza owahlukileyo.

• Nokuba ithumba lisandula ukufumanisa ukuba likhe lafunyaniswa okanye laphinda labuya (buyela).

Emva kokuba unyango lunikiwe, isifo sesifo sichaphazeleka:

• Nokuba ithumba lisuswe ngokupheleleyo ngoqhaqho.

• Nokuba ithumba liphendule kunyango lwe-chemotherapy okanye unyango ngemitha.

Ukuba umhlaza ubuyela emva konyango lokuqala, ukuxela kwangaphambili kuxhomekeke:

• Nokuba umhlaza ubuyile ngaphezulu kweminyaka emibini emva konyango lokuqala.

• Nokuba umhlaza ubuyile apho ubuqale khona ukubumba okanye kwamanye amalungu omzimba.

Amanqanaba e-Ewing Sarcoma

IINGONGOMA EZIPHAMBILI

• Iziphumo zovavanyo lokuqonda isifo kunye nesiteji zisetyenziselwa ukufumanisa ukuba iiseli zomhlaza zisasazekile na.

• I-sarcoma ye-Ewing ichazwa njengendawo yokuhlala, imastastatic, okanye ephindaphindayo.

• Indawo ye-Ewing sarcoma

• Imetastatic Ewing sarcoma

• Ukuphinda sarcoma kwe-Ewing

• Zintathu iindlela umhlaza onwenwa ngayo emzimbeni.

• Umhlaza unokusasazeka ukusuka apho waqala khona kwamanye amalungu omzimba.

Iziphumo zovavanyo lokuqonda isifo kunye nesiteji zisetyenziselwa ukufumanisa ukuba iiseli zomhlaza zisasazekile na.

Inkqubo esetyenziselwa ukufumanisa ukuba ngaba umhlaza sele usasazekile ukusuka apho uqale khona ukuya kwamanye amalungu omzimba kuthiwa yi-staging. Akukho nkqubo yokubeka emgangathweni ye-Ewing sarcoma. Iziphumo zovavanyo kunye neenkqubo ezenziweyo zokuchonga kunye nenqanaba le-Ewing sarcoma zisetyenziselwa ukuchaza amathumba njengawendawo okanye imastastatic.

I-sarcoma ye-Ewing ichazwa njengendawo yokuhlala, imastastatic, okanye ephindaphindayo.

I-sarcoma ye-Ewing ichazwa njengendawo yokuhlala, imastastatic, okanye ephindaphindayo.

Indawo ye-Ewing sarcoma

Umhlaza ufunyanwa kwithambo okanye kwithishu ethambileyo apho iqale khona kwaye inokuthi isasaze kwiithishu ezikufuphi, kubandakanya ne-lymph node ezikufutshane.

Imetastatic Ewing sarcoma

Umhlaza usasazekile ukusuka ethanjeni okanye kwithishu ethambileyo apho waqala khona kwamanye amalungu omzimba. Kwi-Ewing tumor yethambo, umhlaza uhlala usasazeka ukuya emiphungeni, kwamanye amathambo, kunye nomongo wethambo.

Ukuphinda sarcoma kwe-Ewing

Umhlaza uphinde wabuya (ubuye) emva kokuba unyangiwe. Umhlaza ungabuyela kwithambo okanye kwithishu ethambileyo apho iqale khona okanye kwelinye ilungu lomzimba.

Zintathu iindlela umhlaza onwenwa ngayo emzimbeni.

Umhlaza unokusasazeka ngezicubu, inkqubo ye-lymph, kunye negazi:

• Izicubu. Umhlaza usasazeka ukusuka apho waqala ngokukhula waya kwiindawo ezikufuphi.

• Inkqubo yeLymph. Umhlaza usasazeka ukusuka apho waqala ngokungena kwinkqubo ye-lymph. Umhlaza uhamba ngemithambo ye-lymph ukuya kwamanye amalungu omzimba.

• Igazi. Umhlaza usasazeka ukusuka apho waqala ngokungena egazini. Umhlaza uhamba ngemithambo yegazi uye kwamanye amalungu omzimba.

Umhlaza unokusasazeka ukusuka apho waqala khona kwamanye amalungu omzimba.

Xa umhlaza usasazeka kwelinye ilungu lomzimba, ubizwa ngokuba yi-metastasis. Iiseli zomhlaza ziyaphuma apho ziqale khona (ithumba lokuqala) kwaye zihamba ngenkqubo ye-lymph okanye igazi.

• Inkqubo yeLymph. Umhlaza ungena kwinkqubo ye-lymph, uhamba kwiinqanawa ze-lymph, kwaye wenze i-tumor (i-metastatic tumor) kwenye indawo yomzimba.

• Igazi. Umhlaza ungena egazini, uhambe kwimithambo yegazi, kwaye wenze ithumba (metastatic tumor) kwelinye ilungu lomzimba.

I-tumor ye-metastatic yinto efanayo yomhlaza njenge-tumor yokuqala. Umzekelo, ukuba i-Ewing sarcoma isasazeka emiphungeni, iiseli zomhlaza emiphungeni ngokwenene zi-Ewing sarcoma cells. Esi sifo siyi-metastatic Ewing sarcoma, hayi umhlaza wemiphunga.

Unyango ngoKhetho lonyango

IINGONGOMA EZIPHAMBILI

• Kukho iintlobo ezahlukeneyo zonyango lwabantwana abane-Ewing sarcoma.

• Abantwana abane-Ewing sarcoma kufuneka unyango lwabo lucwangciswe liqela lababoneleli ngezempilo abaziingcali kunyango lomhlaza ebantwaneni.

• Unyango lwe-Ewing sarcoma lunokubangela iziphumo ebezingalindelekanga.

• Iindidi ezine zonyango olusetyenziswayo zisetyenziswa:

• Unyango ngamayeza

• Unyango ngemitha

• Ugqirha

• I-dose ephezulu ye-chemotherapy kunye nokuhlangulwa kweseli

• Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.

• Unyango ekujoliswe kulo

• Unyango lwe-Immunotherapy

• Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.

• Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango lomhlaza.

• Iimvavanyo zokulandelela zisenokufuneka.

Kukho iintlobo ezahlukeneyo zonyango lwabantwana abane-Ewing sarcoma.

Iindlela ezahlukeneyo zonyango ziyafumaneka kubantwana abane-Ewing sarcoma. Olunye unyango lusemgangathweni (unyango olusetyenziswayo ngoku), kwaye olunye luvavanywa kuvavanyo lweklinikhi. Uvavanyo lweklinikhi yonyango luphononongo olwenzelwe ukunceda ukuphucula unyango lwangoku okanye ukufumana ulwazi kunyango olutsha kwizigulana ezinomhlaza. Xa uvavanyo lwezonyango lubonisa ukuba unyango olutsha lungcono kunonyango oluqhelekileyo, unyango olutsha kunokuba lunyango olusemgangathweni.

Kuba umhlaza kubantwana nakwishumi elivisayo unqabile, ukuthatha inxaxheba kuvavanyo lweklinikhi kufuneka kuthathelwe ingqalelo. Olunye uvavanyo lwezonyango luvuleleke kuphela kwizigulana ezingaluqalanga unyango.

Abantwana abane-Ewing sarcoma kufuneka unyango lwabo lucwangciswe liqela lababoneleli ngezempilo abaziingcali kunyango lomhlaza ebantwaneni.

Unyango luya kujongwa yi-oncologist yabantwana, ugqirha ogxile ekunyangeni abantwana abanomhlaza. I-oncologist yabantwana isebenza nabanye ababoneleli ngezempilo abaziingcali ekunyangeni abantwana abane-Ewing sarcoma kwaye abagxile kwiindawo ezithile zamayeza. Oku kunokubandakanya ezi ngcali zilandelayo:

• Ugqirha wabantwana.

• Ugqirha oncologist okanye oncologist wamathambo.

• Imitha ye-oncologist.

• Ingcali yomongikazi wabantwana.

• Unontlalontle.

• Ingcali yokuvuselela.

• Ingcali yeengqondo.

Unyango lwe-Ewing sarcoma lunokubangela iziphumo ebezingalindelekanga.

Ngolwazi malunga neziphumo ebezingalindelekanga eziqala ngexesha lonyango lomhlaza, jonga iphepha lethu Iziphumo ezingalunganga.

Iziphumo ebezingalindelekanga ezivela kunyango lomhlaza eziqala emva konyango kwaye ziqhubeke iinyanga okanye iminyaka zibizwa ngokuba ziziphumo ezifike kade. Iziphumo zokunyanga komhlaza zingabandakanya oku kulandelayo:

• Iingxaki zomzimba.

• Utshintsho kwiimvakalelo, iimvakalelo, ukucinga, ukufunda, okanye inkumbulo.

• Umhlaza wesibini (iintlobo ezintsha zomhlaza). Izigulana ezinyangwe i-Ewing sarcoma zinomngcipheko okhulayo we-myeloid leukemia kunye ne-myelodysplastic syndrome. Kukho umngcipheko okhulayo we-sarcoma kwindawo ephathwe ngonyango lwe-radiation.

Ezinye iziphumo ezinokufika emva kwexesha zinokunyangwa okanye zilawulwe. Kubalulekile ukuba uthethe noogqirha bomntwana wakho malunga nefuthe unyango lomhlaza olunokuba nalo emntwaneni wakho. (Jonga isishwankathelo se- malunga neZiphumo eziMva zaNyango zoMhlaza woMntwana ngolwazi oluthe kratya.)

Iindidi ezine zonyango olusetyenziswayo zisetyenziswa:

Unyango ngamayeza

I-Chemotherapy yonyango lomhlaza olusebenzisa iziyobisi ukunqanda ukukhula kweeseli zomhlaza, nokuba zibulale iiseli okanye uziyekise ekwahlukaneni. Xa i-chemotherapy ithathwa ngomlomo okanye ifakwe kwi-vein okanye kwimisipha, amachiza angena kwigazi kwaye anokufikelela kwiiseli zomhlaza kuwo wonke umzimba (systemic chemotherapy). Xa i-chemotherapy ibekwa ngqo kwi-cerebrospinal fluid, ilungu, okanye indawo yomzimba efana nesisu, iziyobisi zichaphazela kakhulu iiseli zomhlaza kwezo ndawo (chemotherapy yengingqi). Umdibaniso wekhemotherapy yonyango olusebenzisa ngaphezulu kwesiyobisi esinye somhlaza.

Inkqubo yokudibanisa ichemotherapy yinxalenye yonyango kuzo zonke izigulana ezinethumba. Kuhlala kunyango lokuqala olunikiweyo kwaye kuhlala malunga neenyanga ezi-6 ukuya kwezi-12. I-Chemotherapy ihlala inikwa ukunciphisa ithumba ngaphambi kotyando okanye unyango lwe-radiation kunye nokubulala naziphi na iiseli zesisu ezinokuthi zisasazeke kwamanye amalungu omzimba.

Jonga iziyobisi ezivunyiweyo zeTissue Tissue Sarcoma ngolwazi oluthe kratya.

Unyango ngemitha

Unyango ngemitha lunyango olusebenzisa amandla e-x-reyi okanye ezinye iintlobo zemitha yokubulala iiseli zomhlaza okanye ukuzigcina zikhula. Zimbini iintlobo zonyango lwe-radiation:

• Unyango lwangaphandle lwemitha lusebenzisa umatshini ngaphandle komzimba ukuthumela imitha kumhlaza.

• Unyango lwangaphakathi lwemitha lusebenzisa into enemitha ye-radio etywinwe kwiinaliti, iimbewu, iingcingo, okanye iicatheters ezibekwe ngqo okanye kufutshane nomhlaza.

Unyango lwangaphandle lwemitha lusetyenziselwa ukunyanga i-Ewing sarcoma.

Unyango ngemitha lusetyenziswa xa ithumba lingenakususwa ngotyando okanye xa utyando lokususa ithumba luchaphazela imisebenzi ebalulekileyo yomzimba okanye indlela umntwana ajongeka ngayo. Ingasetyenziselwa ukwenza ithumba libe lincinci kwaye inciphise inani lezihlunu ekufuneka zisuswe ngexesha lotyando. Ingasetyenziselwa ukunyanga naliphi na ithumba elihlala likhona emva kotyando kunye nezihlunu ezisasazeke kwamanye amalungu omzimba.

Ugqirha

Ugqirha luqhele ukwenziwa ukususa umhlaza oshiyekileyo emva konyango okanye ngemitha. Xa kunokwenzeka, lonke ithumba lisuswa ngotyando. Amathambo kunye nethambo elisusiweyo linokufakwa endaweni yegraft, esebenzisa izicwili kunye nethambo elithathwe kwelinye ilungu lomzimba wesigulana okanye umnikeli. Ngamanye amaxesha ukufakelwa, njengamathambo angengowamvelo, kuyasetyenziswa.

Emva kokuba ugqirha ewususile wonke umhlaza onokubonakala ngexesha lotyando, ezinye izigulana zinokunikwa ichemotherapy okanye unyango lweradiation emva kotyando lokubulala naziphi na iiseli zomhlaza ezisele. Unyango olunikezwe emva kotyando, ukunciphisa umngcipheko wokuba umhlaza ubuye kwakhona, lubizwa ngokuba lunyango oluncedisayo.

I-dose ephezulu ye-chemotherapy kunye nokuhlangulwa kweseli

Amanqanaba aphezulu e-chemotherapy anikezelwa ukubulala iiseli zomhlaza. Iiseli ezisempilweni, kubandakanya iiseli ezenza igazi, nazo ziyatshatyalaliswa lunyango lomhlaza. Ukufakelwa kweseli kwistem kunyango endaweni yeeseli ezenza igazi. Iiseli zeziqu (iiseli zegazi ezingafakwanga) ziyasuswa egazini okanye kumongo wethambo wesigulana okanye somnikeli kwaye zibandisiwe kwaye zigcinwe. Emva kokuba isiguli sigqibe i-chemotherapy, iiseli ezigciniweyo ezigciniweyo ziyanyibilika kwaye zibuyiselwe kwisigulana ngokufakwa. Ezi seli ze-stem ziphinde zafakwa zikhula ziye (kwaye zibuyisele) iiseli zegazi lomzimba. I-Chemotherapy nge-stem cell Rescue isetyenziselwa ukunyanga i-Ewing sarcoma yendawo kunye neyesiqhelo.

Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.

Eli candelo lesishwankathelo lichaza unyango olufundwayo kuvavanyo lweklinikhi. Isenokungakhankanyi lonke unyango olutsha olufundwayo. Ulwazi malunga novavanyo lweklinikhi luyafumaneka kwiwebhusayithi yeNCI.

Unyango ekujoliswe kulo

Unyango ekujoliswe kulo lunyango olusebenzisa iziyobisi okanye ezinye izinto ukuphazamisa iinkqubo ezifuna ukuba iiseli zomhlaza zikhule kwaye zikhule. Iindidi zonyango olujolisiweyo olusetyenziselwa ukunyanga umhlaza wabantwana ongaqhelekanga lubandakanya oku kulandelayo:

• Unyango lwe- monoclonal antibody: Iintsholongwane zeMonoclonal zenziwe elebhu zivela kuhlobo olunye lweeseli zomzimba. Ezi antibodies zinokuchonga izinto ezikumhlaza iiseli okanye izinto eziqhelekileyo ezinokunceda iiseli zomhlaza zikhule. Amachiza omzimba ancamathele kwizinto kwaye abulale iiseli zomhlaza, athintele ukukhula kwazo, okanye azigcine zisasazeka. Iintsholongwane zeMonoclonal zinikezelwa ngokufakwa. Zingasetyenziselwa zodwa okanye ukuphatha iziyobisi, ityhefu, okanye izinto ezinemitha yeathom ngqo kwiiseli zomhlaza. IGanitumab ifundelwa unyango lwe-metwingatic Ewing sarcoma.

• Unyango lwe-Kinase inhibitor: I- Kinase inhibitors sisiyobisi esivimba iprotein efunekayo ukuze iiseli zomhlaza zahlule. Bafundiswa ukunyanga i-Ewing sarcoma ephindaphindayo.

• I-NEDD8-activating enzyme (NAE) ye-inhibitor yonyango: I- NAE inhibitors ziziyobisi ezinamathele kwi-NAE kwaye ziyeke iiseli zomhlaza ekuhlukaniseni. I-Pevonedistat ifundwa kunyango lwe-Ewing sarcoma ephindaphindayo.

Unyango lwe-Immunotherapy

I-Immunotherapy lunyango olusebenzisa amajoni omzimba omzimba ukulwa nomhlaza. Izinto ezenziwe ngumzimba okanye ezenziwe elebhu zisetyenziselwa ukonyusa, ukuqondisa, okanye ukubuyisela ukhuseleko lwendalo lomzimba kumhlaza. Olu hlobo lonyango lomhlaza lukwabizwa ngokuba yi-biotherapy okanye unyango lwe-biologic.

• Unyango lwe-inhibitor yokujonga amajoni omzimba Ezi proteni zinceda ukugcina iimpendulo ze-immune zitshekishwe kwaye zinokugcina iiseli ze-T ekubulaleni iiseli zomhlaza. Xa ezi proteni zivaliwe, "iibhuleki" kumajoni omzimba ziyakhutshwa kwaye iiseli ze-T ziyakwazi ukubulala iiseli zomhlaza ngcono. I-Nivolumab kunye ne-ipilimumab ziindidi ze-immune checkpoint inhibitors ezifundwayo ukunyanga i-Ewing sarcoma ephindaphindayo.

• Unyango lwe- T-cell ye- Chimeric antigen receptor (i-CAR): Unyango lwe- CAR T-cell luhlobo lwe-immunotherapy etshintsha iiseli ze-T zesigulana (uhlobo lweseli yamajoni omzimba) ukuze zihlasele iiproteni ezithile ngaphezulu kweeseli zomhlaza. Iiseli ze-T zithathwa kwisigulana kwaye ii-receptors ezizodwa zongezwa kumphezulu wazo kwilabhoratri. Iiseli eziguqulweyo zibizwa ngokuba ziiseli ze-chimeric antigen receptor (CAR) T. Iiseli ze-CAR T zikhula elebhu kwaye zinikwa isigulana ngokufakwa. Iiseli ze-CAR T ziphindaphindeka kwigazi lesigulana kwaye zihlasela iiseli zomhlaza. Unyango lwe-CAR T-cell lufundwa kunyango lwe-Ewing sarcoma ephindaphindayo.

Unyango lwe-CAR T-cell. Uhlobo lonyango apho iiseli ze-T zesigulana (uhlobo lweseli yomzimba yokuzikhusela) ziguqulwa kwilabhoratri ukuze zibophelele kwiiseli zomhlaza kwaye zibabulale. Igazi eliphuma emthanjeni engalweni yesigulana lihamba lingena kwityhubhu lisiya kumatshini weapheresis (ongaboniswanga), osusa iiseli ezimhlophe zegazi, kubandakanya neeseli zeT, kwaye uthumele lonke igazi libuyele kwisigulana. Emva koko, i-gene ye-receptor ekhethekileyo ebizwa ngokuba yi-chimeric antigen receptor (CAR) ifakwe kwiiseli ze-T elebhu. Izigidi zeeseli ze-CAR T zikhuliswa elebhu zize zinikwe isigulana ngokufakwa. Iiseli ze-CAR T ziyakwazi ukubopha kwi-antigen kwiiseli zomhlaza kwaye zibabulale.

Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.

Kwezinye izigulana, ukuthatha inxaxheba kuvavanyo lweklinikhi kunokuba lolona khetho lonyango lufanelekileyo. Uvavanyo lwezonyango luyinxalenye yenkqubo yophando ngomhlaza. Uvavanyo lwezonyango luyenziwa ukufumanisa ukuba unyango lomhlaza olutsha lukhuselekile kwaye luyasebenza okanye lungcono kunonyango oluqhelekileyo.

Uninzi lonyango oluqhelekileyo lwanamhlanje lomhlaza lusekwe kuvavanyo lweklinikhi lwangaphambili. Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zingafumana unyango olusemgangathweni okanye zibe phakathi kwabokuqala ukufumana unyango olutsha.

Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zikwanceda ukuphucula indlela umhlaza oza kunyangwa ngayo kwixa elizayo. Nokuba uvavanyo lwezonyango alukhokeleli kunyango olusebenzayo, bahlala bephendula imibuzo ebalulekileyo kwaye bancedisa ukuqhubela phambili uphando.

Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango lomhlaza.

Olunye uvavanyo lwezonyango lubandakanya kuphela izigulana ezingekafumani unyango. Olunye uvavanyo lokuvavanywa kunyango kwizigulana ezinomhlaza ongakhange ubengcono. Kukwakho nezilingo zeklinikhi ezivavanya iindlela ezintsha zokumisa umhlaza ekubuyeleni (ekubuyeni) okanye ukunciphisa iziphumo ebezingalindelekanga zonyango lomhlaza.

Uvavanyo lwezonyango lwenzeka kwiindawo ezininzi zelizwe. Ulwazi malunga novavanyo lweklinikhi oluxhaswe yi-NCI lunokufumaneka kwiphepha lewebhu le-NCI. Uvavanyo lwezonyango oluxhaswa yiminye imibutho lunokufumaneka kwiwebhusayithi yeClinicalTrials.gov.

Iimvavanyo zokulandelela zisenokufuneka.

Ezinye zeemvavanyo ezenziweyo ukufumanisa umhlaza okanye ukufumanisa inqanaba lomhlaza zinokuphindwa. Olunye uvavanyo luya kuphindwa ukuze kubonwe ukuba lusebenza njani unyango. Izigqibo malunga nokuqhubeka, ukutshintsha, okanye ukuyeka unyango zinokusekwa kwiziphumo zolu vavanyo.

Ezinye zeemvavanyo ziya kuqhubeka ukwenziwa amaxesha ngamaxesha emva kokuba unyango luphelile. Iziphumo zolu vavanyo zingabonisa ukuba imeko yomntwana wakho itshintshile okanye ukuba umhlaza ubuye wabuya (buyela). Olu vavanyo ngamanye amaxesha lubizwa ngokuba luvavanyo olulandelayo okanye ukuhlolwa.

Iinketho zonyango lwe-Ewing Sarcoma

Kweli Candelo

• Indawo yase-Ewing Sarcoma

• I-Metastatic Ewing Sarcoma

• Ukuphinda u-Ewing Sarcoma

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

Indawo yase-Ewing Sarcoma

Unyango oluqhelekileyo lwe-Ewing sarcoma yendawo ibandakanya:

• Unyango ngamayeza.

• Utyando kunye / okanye unyango lwemitha.

• I-dose ephezulu ye-chemotherapy kunye nokuhlangulwa kweseli.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

I-Metastatic Ewing Sarcoma

Unyango oluqhelekileyo lwe-metwingatic Ewing sarcoma lubandakanya:

• Unyango ngamayeza.

• Ugqirha.

• Unyango ngemitha.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Ukuphinda u-Ewing Sarcoma

Akukho lonyango luqhelekileyo lwe-Ewing sarcoma ephindaphindayo kodwa ukhetho lonyango lunokubandakanya oku kulandelayo:

• Ukudibanisa i-chemotherapy.

• Unyango ngemitha yamathumba, njengonyango lokuthomalalisa ukukhulula iimpawu kunye nokuphucula umgangatho wobomi.

• Unyango lwe-radiation olunokuthi lulandelwe ngotyando lokususa amathumba athe saa emiphungeni.

• I-dose ephezulu ye-chemotherapy kunye nokuhlangulwa kweseli.

Ukhetho lonyango olufundelwa i-Ewing sarcoma ephindaphindiweyo lubandakanya oku kulandelayo:

• Ukujonga isampulu yethumba lesigulana kwiinguqu ezithile zofuzo. Uhlobo lonyango ekujoliswe kulo oluya kunikwa isigulana luxhomekeke kuhlobo lotshintsho lwemfuza.

• Unyango olujolise kwi-tyrosine kinase inhibitor (cabozantinib).

• I-Immunotherapy ene-immune checkpoint inhibitor (nivolumab okanye ipilimumab).

• Unyango lwe-T-cell ye-antigen receptor (CAR).

• Unyango olujolise kwi-NEDD8-activating enzyme inhibitor (pevonedistat) kunye nekhemotherapy.

• Uvavanyo lweklinikhi yohlobo olutsha lonyango ekujoliswe kulo.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Ukufunda ngakumbi nge-Ewing Sarcoma

Ngolwazi oluthe kratya kwiZiko leSizwe loMhlaza malunga ne-Ewing sarcoma, jonga oku kulandelayo:

• Ikhasi lasekhaya lomhlaza wamathambo

• Ikhompyuter yeTomography (CT) kunye noMhlaza

• Iithagethi zoMhlaza ezijolise kuko

• Umhlaza wamathambo

Ngolwazi oluthe kratya lomhlaza wabantwana kunye nezinye izixhobo zomhlaza ngokubanzi, jonga oku kulandelayo:

• Malunga noMhlaza

• Umhlaza wabantwana

• Unyango lokuKhangela uMhlaza waBantwanaPhuma kwiNgcazelo yokuzihlangula

• Iziphumo zokugqibela zonyango lweCancer yoMntwana

• Abafikisayo kunye naBantu abaDala abaDala abanomhlaza

• Abantwana abanomhlaza: Isikhokelo sabazali

• Umhlaza kuBantwana nakwishumi elivisayo

• Ukuqokelela

• Ukujamelana noMhlaza

• Imibuzo yokubuza ugqirha wakho malunga neCancer

• Abasindileyo kunye nabaNonopheli