I-sarcoma

I-Ewing sarcoma wuhlobo lwesimila esikhula ethanjeni noma esicutshini esithambile sisuka ohlotsheni oluthile lweseli. Amathambo emilenze, izingalo, izinyawo, izandla, isifuba, i-pelvis, umgogodla, nogebhezi konke kungathinteka yi-Ewing sarcoma. I-Ewing sarcoma ingabuye ibonakale ezicutshini ezithambile ze-trunk, izingalo, imilenze, ikhanda, intamo, i-retroperitoneum (indawo engemuva kwezicubu ezihlanganisa udonga lwesisu futhi ivikele iningi lezitho ezisesiswini), nezinye izindawo.

Intsha kanye nabantu abadala abasebasha yibona abavame ukuthinteka kakhulu yi-Ewing sarcoma (intsha kuya phakathi kweminyaka yama-20s).

I-peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma yodonga lwesifuba), i-Ewing sarcoma (i-Ewing sarcoma esicutshini ngaphandle kwethambo), kanye nomndeni we-Ewing sarcoma womdlavuza wonke amagama asetshenziswa ukuchaza i-Ewing sarcoma.

I-Ewing sarcoma iyithumba elingajwayelekile lamathambo elithinta kakhulu intsha. Kungase futhi kwenzeke esicutshini esithambile ngaphandle kwethambo (extraosseous Ewing sarcoma). I-tumor Ewing sarcoma ihlotshaniswa nokubizwa nge-primitive neuroectodermal tumor (PNET). Abacwaningi bathole ukuthi lezi zifo ezimbi zihlanganyela izici ezifanayo zomzimba futhi zixhunywe nenkinga efanayo ye-chromosomal (i-reciprocal translocation ebhalansile). Ngenxa yalokho, lezi zimila zivame ukubizwa ngokuthi umndeni wakwa-Ewing of tumors (EFT). I-Ewing sarcoma yethambo, i-extraosseous Ewing sarcoma, i-primitive neuroectodermal tumor, kanye ne-Askin's tumor konke kufakwe ngaphansi kwegama elilodwa lesambulela (isimila sodonga lwesifuba). I-Ewing sarcoma yethambo ibangela ngaphezu kwama-70% omdlavuza kulo mndeni. I-Ewing sarcoma yigama elikhethiwe ngoba, ngaphandle kwamalebula amaningana, iyithumba elilodwa nge-molecularly. Ithambo elide emilenzeni (i-femur) kanye namathambo ayisicaba ku-pelvis nasesifubeni yiwona avame ukuthinteka yi-Ewing sarcoma yethambo. I-Ewing sarcoma isifo esiyingozi esingasakazeka (i-metastasize) emaphashini, kwamanye amathambo, nomnkantsha wamathambo, okwenza kube usongo olukhulu empilweni yomuntu. Asikho isizathu esaziwayo salezi zimila.

UDkt James Ewing uqale wachaza i-Ewing sarcoma ezincwadini zezokwelapha ngo-1921. I-Ewing sarcoma iyithumba lesibili elivamile lamathambo ezinganeni, elibalelwa ku-2% wazo zonke izifo zomdlavuza ezinganeni.

Ukuze uthole imininingwane nge-Ewing sarcoma, sicela uvakashele:

https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq

Izimpawu nezimpawu ze-Ewing sarcoma

Izimpawu nezimpawu ze-Ewing sarcoma zihlanganisa:

• Ubuhlungu, ukuvuvukala noma ukuzwela eduze kwendawo ethintekile
• Ubuhlungu be-Bone
• Ukukhathala okungachazeki
• Imfiva engaziwa imbangela
• Ukwehlisa isisindo ngaphandle kokuzama

Isimila esivela emndenini wakwa-Ewing wezimila singabangela ukungakhululeki, ubuhlungu, nokuvuvukala endaweni ethintekile yomzimba. Ekuqaleni, ubuhlungu bungase bufike futhi buhambe (ngezikhathi ezithile), kodwa maduze buzovumelana. Kungase futhi kube ndikindiki nobuthakathaka endaweni ethintekile. Imfiva, ukukhathala, ukwehla kwesisindo somzimba, amazinga aphansi okujikeleza kwamangqamuzana abomvu egazi (i-anemia), kanye namazinga aphezulu okujikeleza kwamangqamuzana amhlophe egazi kuyizimpawu ezingaba khona kubantu abathile (i-leukocytosis). Ngokuvamile, inqwaba ezwakalayo iba khona.

Ingxenye emaphakathi (isifunda se-diaphyseal) yamathambo amade ezingalo nemilenze, ikakhulukazi ithambo elide lomlenze, livame ukuthinteka yi-Ewing sarcoma (femur). Amathambo ayisicaba, njengalawo asesinqeni, odongeni lwesifuba, kanye nekholomu yomgogodla, avame ukuthinteka yilezi zicubu (ama-vertebrae). I-Ewing sarcoma ingakhula kunoma yiliphi ithambo emzimbeni, okuhlanganisa unyawo, isandla, umhlathi ongezansi (mandible), ugebhezi, nezinye izindawo. I-trunk nesifuba yizindawo ezivame kakhulu izimila ezithambile. I-pelvis, nokho, iyindawo evame kakhulu yokwethulwa, ibalwa cishe ngama-25% amacala. I-Ewing sarcoma yenza amathambo abe buthaka, okungaholela ekuqhekekeni.

Lezi zimila zivame ukuba nolaka futhi zinamandla okusakazeka (i-metastasize) kwezinye izingxenye zomzimba, kuhlanganise namanye amathambo namaphaphu. Umnkantsha ungase uhileleke ezimweni ezingavamile.

Izimpawu zalezi zimila zincike endaweni yazo. Isimila emlenzeni, ngokwesibonelo, singabangela ukuxhuga, isimila emaphashini singabangela izinkinga zokuphefumula kanye nokunqwabelana koketshezi ezingqimbeni zamaphaphu nesifuba (i-pleural effusion), kanye nesimila ngaphakathi. ikholomu yomgogodla ingabangela ubuthakathaka noma ukukhubazeka kwemisipha ethintekile (i-paraplegia).

Izinkinga ze-Ewing sarcoma

I-Ewing sarcoma ayinambangela eyaziwayo, futhi uhlobo lwamaseli angaphansi alukatholwa. Iningi lezehlakalo kucatshangwa ukuthi zenzeke ngokungahleliwe futhi ngaphandle kwesizathu esicacile (ngokuphindaphindiwe).

Amaseli e-Ewing sarcoma ngokuvamile achazwa ngokushintshashintsha kokwakheka kwawo kofuzo okwaziwa ngokuthi i-reciprocal translocation, ngokocwaningo lwe-chromosomal (cytogenetic). Izingcezu zama-chromosome amabili ahlukene ziyahlukana futhi "zishintshe izindawo" ekudlulisweni okulinganayo. Ulwazi lofuzo lomuntu ngamunye luphathwa ama-chromosome, atholakala kuyi-nucleus yamangqamuzana omuntu. Amapheya e-chromosomal yabantu anenombolo 1 kuya ku-22, kanye nepheya yama-23 yama-chromosome ocansi ahlanganisa i-chromosome eyodwa engu-X neyodwa Y kwabesilisa nama-chromosome amabili ka-X kwabesifazane. Ikhromozomu ngayinye inengalo emfushane nende, echazwa ngezinhlamvu “p” kanye “q,” ngokulandelana. Ama-Chromosome aphinde ahlukaniswe abe amabhendi anezinombolo amaningi.

Izingalo ezinde (q) ze-chromosome 11 kanye no-22 yizindawo ze-chromosomal ezibandakanyeka ku-Ewing sarcoma (11q24-22q12). Lezi zingcezu ziyahlukanisa futhi zishintshanise izindawo. Ezimweni eziningi, lokhu kubangela ukuhlangana okuphambene kwezakhi zofuzo ezimbili, ngokuvamile izakhi zofuzo ze-EWS ne-FLI. Izakhi zofuzo zakha (encode) amaprotheni akhonza imisebenzi eyahlukene emzimbeni. Izakhi zofuzo ze-EWS kanye ne-FLI zihlangana ngendlela engavamile, okuholela “ekuhlanganiseni” kofuzo olukhiqiza umkhiqizo wamaprotheni ongashintshile. Nakuba imisebenzi ethile noma imiphumela yale phrotheni engavamile ingaziwa, abacwaningi bakholelwa ukuthi ingaba nomthelela noma ibe nomthelela ekuthuthukisweni kwe-Ewing sarcoma. Imbangela yokudluliselwa kwe-chromosomal phakathi kwama-chromosome 11 no-22 ayaziwa futhi. Nokho, ngokwezinye izilinganiso, lokhu kudluliselwa kungatholakala kumaphesenti angaphezu kwangu-85 ezimila emndenini wakwa-Ewing. Ufuzo lwe-EWS lungase luhlangane nofuzo ngaphandle kwe-FLI ngezikhathi ezingavamile; lezi ngokuvamile izakhi zofuzo ezivela emndenini ofanayo njenge-FLI1, nofuzo lwe-ERG oluyisibonelo esivame kakhulu.

I-Ewing sarcoma ingenzeka njengomphumela wokufika sekwephuzile kokwelashwa kwangaphambili kolunye uhlobo lomdlavuza ezimeni ezingavamile kakhulu.

Ukuxilongwa kwe-Ewing sarcoma

Ukuhlolwa okuphelele komtholampilo, ukuhlonzwa kwezimpawu ezijwayelekile kanye nokungajwayelekile komzimba, umlando ogcwele wesiguli, kanye nohlu lokuhlola okukhethekile kusetshenziswa ukuxilonga isimila emndenini wakwa-Ewing womdlavuza. Ukuhlolwa kwe-microscopic yamaseli wesimila kanye nezicubu ezilimele (i-histopathology) kanye nokuhlaziywa kwamangqamuzana ukuze kubhekwe ukudluliselwa kwe-EWS-FLI1 kuyizibonelo zokuhlola okunjalo.

Ukuhlolwa Komtholampilo Nokulandelela

Ama-X ray angase atholwe kuqala, ikakhulukazi uma kukhona isigaxa esiphuphumayo. Izithombe zesimila noma indawo ethintekile zitholwa kusetshenziswa ama-X-ray. Amasu okuthwebula athuthuke kakhulu angenziwa ukuze kuhlolwe usayizi wesimila, indawo, kanye nokunwetshwa (isb., kusicubu esithambile noma umnkantsha wethambo), ukuze kutholakale ukuthi isimila sesisabalele yini kwezinye izingxenye zomzimba (isb, amaphaphu namaphaphu). amanye amathambo), kanye nokusiza izinqubo zokuhlinzwa zesikhathi esizayo. Ukuskena kwe-Computerized tomography (CT), imaging resonance magnetic (I-MRI), kanye nezikena zamathambo ziyizibonelo zezindlela zokuthwebula izithombe. I-biopsy yomnkantsha ingakwazi ukubona ukuthi ubungozi buqhubekele emnkantsheni noma cha.

Ukukhishwa kokuhlinzwa (i-biopsy) kanye nokuhlolwa kwe-microscopic kwengxenye yezicubu ezithintekile kungasetshenziswa ukuhlonza i-Ewing sarcoma. I-CD99 iyiphrotheni ethile engaphezulu ekhonjwe iningi lomdlavuza emndenini wakwa-Ewing. Ukuba khona kwaleli phrotheni kungasiza ekuxilongeni i-Ewing sarcoma.

I-Polymerase chain reaction ingenye isivivinyo esisetshenziselwa ukukhomba i-Ewing sarcoma (PCR). I-PCR, noma i-polymerase chain reaction, iyindlela yesayensi eqhathaniswe “nokukopisha.” Ivumela ososayensi ukuthi banwebe futhi bakopishe ukulandelana kwe-DNA ngokuphindaphindiwe. Ngenxa yalokho, bangakwazi ukuhlola i-DNA eduze futhi bathole izakhi zofuzo kanye noshintsho lwezakhi zofuzo ngokushesha okukhulu, njengokudluliswa kwe-Reciprocal Translocation okuyisici se-Ewing sarcoma. Lokhu kuhlolwa kutholakala kuphela ukuze kufundwe.

Ukwelashwa kwe-Ewing sarcoma

Odokotela abasebenza ngokukhethekile ekuxilongeni nasekulapheni umdlavuza ezinganeni (odokotela bezingane), odokotela be-oncologists abadala, ochwepheshe ekusetshenzisweni kwemisebe ukwelapha umdlavuza (ama-radiation oncologists), odokotela abahlinzayo (odokotela bamathambo), abahlengikazi be-oncology, nabanye ochwepheshe bangadingeka ukuze ukuphatha abantu abane-Ewing sarcoma (kuye ngokuthi isizinda sesimila esiyinhloko).

Izici ezihlukahlukene, njengendawo eyinhloko yesimila, ubungako besimila esiyinhloko (isiteji), kanye nezinga lokonakala (ibanga); kungakhathaliseki ukuthi isimila sisabalele kuma-lymph nodes noma ezindaweni ezikude; iminyaka yomuntu nempilo jikelele; kanye/noma ezinye izakhi, zingaba nomthelela ezinkambisweni ezithile zokwelapha kanye nokungenelela. Odokotela kanye namanye amalungu ethimba lokunakekelwa kwezempilo kufanele benze izinqumo mayelana nokusetshenziswa kokungenelela okuqondile ngokubonisana eduze nesiguli, ngokusekelwe emininingwaneni yecala laso, ingxoxo ephelele yezinzuzo nezingozi ezingase zibe khona, okuthandwa yisiguli, nokunye. izici ezifanele.

Ngenxa yokuthi i-Ewing sarcoma ingabangela ukukhathazeka, ingcindezi, nokucindezeleka okukhulu kwengqondo, abantu abanalesi sifo nemindeni yabo bayanxuswa ukuthi bafune ukwelashwa ngemva kokuxilongwa nangaphambi kokwelashwa. Abantu abathintekile kanye nemindeni yabo kufanele bafune ukwelulekwa ngokochwepheshe kanye nosizo lwezengqondo, futhi bahlanganyele emaqenjini okusekela.

Imithi eminingi elwa nomdlavuza (i-chemotherapy) isetshenziswa ngokuhlanganiswa namasu okuhlinzwa kanye/noma ngemisebe ukwelapha abantu abanomdlavuza emndenini wakwa-Ewing. Indawo eyinhloko yesimila yelashwa ngokuhlinzwa kobuhlungu kanye nezicubu ezilimele noma i-radiotherapy. I-Chemotherapy ibulala amangqamuzana omdlavuza endaweni yokuqala kanye namaseli omdlavuza asesabalele kwezinye izingxenye zomzimba. Ezimweni eziningi, i-systemic chemotherapy inikezwa kuqala, bese kuba ukuhlinzwa noma ngemisebe. Ukwelapha okuxubile kubonakale kusebenza kakhulu kunokuhlinzwa noma ukwelapha ngemisebe kuphela. Imisebe ivame ukusetshenziselwa ukwelapha izimila ezingasebenzi futhi, ezimweni ezingavamile, umdlavuza we-metastatic.

Ngenxa yokuthi imithi ehlukene inezindlela ezihlukene zokwenza ekuqedeni amaseli wesimila kanye/noma ukuwavimba ukuthi aphindaphindeke, odokotela basebenzisa izindlela zokwelapha eziningi ze-chemotherapeutic. I-Doxorubicin, i-vincristine, i-cyclophosphamide, i-dactinomycin, i-ifosfamide, ne-etoposide eminye yemithi yokwelapha ngamakhemikhali evame ukusetshenziswa ukwelapha iziguli ze-Ewing sarcoma.

I-Chemotherapy ku-Ewing sarcoma

Lena eminye yemithi ejwayelekile ye-chemo esetshenziselwa ukwelapha i-Ewing sarcoma:

• ICyclophosphamide

• I-Doxorubicin

• I-Etoposide

• I-Ifosfamide

• UVincristine

I-Chemo ye-Ewing sarcoma inikezwa njengenhlanganisela yemithi. I-combo evame ukusetshenziswa kakhulu i-vincristine, i-doxorubicin, ne-cyclophosphamide, eshintshana ne-ifosfamide ne-etoposide. Ungakuzwa lokhu kubizwa I-VDC/IE.

I-Chemotherapy yindlela yokwelapha esebenzisa amakhemikhali anamandla ukubulala amangqamuzana omdlavuza. I-Chemotherapy ngokuvamile iqukethe imithi emibili noma ngaphezulu enikezwa njenge-IV, ithebhulethi, noma inhlanganisela yakho kokubili.

I-Chemotherapy ngokuvamile isetshenziselwa ukwelapha i-Ewing sarcoma. Imithi ingase ibangele ukuba isimila sinciphe, kwenze kube lula ukususa ukonakala ngokuhlinzwa noma ukukukhomba ngokwelashwa ngemisebe.

Ukwelashwa ngamakhemikhali kungase kuqhutshekwe ngemva kokuhlinzwa noma ukwelashwa ngemisebe ukuze kucekelwe noma yimaphi amangqamuzana omdlavuza asele.

I-Chemotherapy ingase isetshenziselwe ukunciphisa ubuhlungu futhi ibambezele ukuqhubeka komdlavuza osusakazekele kwezinye izingxenye zomzimba.

Ukuhlinzwa ku-Ewing sarcoma

Inhloso yokuhlinzwa ukuqeda wonke amangqamuzana omdlavuza. Kodwa-ke, lapho uhlela ukusebenza, khumbula ukuthi kuzokuthinta kanjani amandla akho okwenza umsebenzi wakho wansuku zonke.

Ukuhlinzwa kwe-Ewing sarcoma kungabandakanya ukukhipha ingxenye encane yethambo noma isitho sonke. Ubukhulu nendawo yesimila, kanye nokuthi siyancipha ngemva kokwelashwa, kunquma ukuthi odokotela abahlinzayo bangawususa yini wonke umdlavuza ngaphandle kokuthatha umlenze wonke.

Ukwelashwa ngemisebe ku-Ewing sarcoma

Ukubulala amangqamuzana omdlavuza, imisebe Ukwelapha kusebenzisa imishayo enamandla amakhulu njengama-X-ray nama-proton.

Imishayo yamandla ihlinzekwa umshini ohamba ngakuwe njengoba ulele etafuleni ngesikhathi sokwelashwa ngemisebe. Ukunciphisa ithuba lokulimaza amaseli anempilo azungezile, imishayo iqondiswa ngokunembile endaweni ye-Ewing sarcoma.

Ngemva kokuhlinzwa, ukwelashwa ngemisebe kungase kuphakanyiswe ukuze kuqedwe noma yimaphi amangqamuzana omdlavuza asele. Uma i-Ewing sarcoma itholakala endaweni yomzimba lapho ukuhlinzwa kungenzeki khona noma okungaholela emiphumeleni engathandeki yokusebenza, ingasetshenziswa futhi esikhundleni sokuhlinzwa (njengokulahlekelwa amathumbu noma umsebenzi wesinye).

Ukwelashwa ngemisebe kungasiza ekunciphiseni ukungakhululeki futhi kubambezele ukuqhubeka kwe-Ewing sarcoma ethuthukisiwe.

Ukwelapha okuhlosiwe

Ukwelapha okuqondiwe kuwuhlobo lokwelapha oluhlonza futhi luhlasele amangqamuzana omdlavuza athile kusetshenziswa imithi noma amanye amakhemikhali. Izindlela zokwelapha ezihlosiwe mancane kakhulu kune-chemotherapy noma ukwelapha ngemisebe ukubulala amaseli avamile. Okulandelayo yizibonelo zemithi ehlosiwe esetshenziselwa ukwelapha i-Ewing sarcoma.

Amasosha omzimba angama-monoclonal zingamaprotheni amasosha omzimba adalwa elebhu ukwelapha izifo ezahlukahlukene, okuhlanganisa nomdlavuza. Lawa amasosha omzimba angabambelela ekuqondisweni okuthile kumaseli omdlavuza noma amanye amaseli angasiza ukukhula kwamangqamuzana omdlavuza njengokwelashwa komdlavuza. Amasosha omzimba angabese ebulala amangqamuzana omdlavuza, awavimbe ekukhuleni, noma awavimbele ukuthi angasakazeki. I-infusions ye-monoclonal antibodies ilawulwa. Zingasetshenziswa ngokwazo noma ukuletha imithi, ubuthi, noma izinto ezikhipha imisebe kumaseli omdlavuza ngokuqondile. I-Ganitumab iyi-antibody ye-monoclonal esacwaningwa njengokwelashwa kwe-metastatic Ewing sarcoma.

• Ukwelashwa kwe-Kinase inhibitor: Lokhu kwelashwa kuvimbela iphrotheni edingekayo ukuze amangqamuzana omdlavuza ahlukane. I-Cabozantinib iyi-kinase inhibitor efundelwa ukwelapha i-Ewing sarcoma ephindaphindayo.
• I-NEDD8-activating enzyme (NAE) inhibitor therapy: I-NAE inhibitor imithi enamathela ku-NAE futhi ivimbele amangqamuzana omdlavuza ukuba ahlukane. I-Pevonedistat iyi-NAE inhibitor efundelwa ukwelapha i-Ewing sarcoma ephindaphindayo.

immunotherapy

I-Immunotherapy yindlela yokwelapha esebenzisa amasosha omzimba esiguli ukulwa nomdlavuza. Izinto ezenziwe umzimba noma ezenziwe elabhorethri zisetshenziselwa ukuqinisa, ukuqondisa, noma ukubuyisela izivikelo ezingokwemvelo zomzimba ngokumelene nomdlavuza. Lokhu kwelashwa komdlavuza kuwuhlobo lwe-biologic therapy.