Uyini i-DSRCT? Izimpawu, Izimbangela, Nokwelashwa

I-DSRCT imelela i-Desmoplastic Small Round Cell Tumor, uhlobo oluyimfihlo nolubhubhisayo lomdlavuza oluthinta ikakhulukazi abantu abasebancane. Lo mdlavuza ongajwayelekile uvamise ukuvela esiswini, ikakhulukazi e-peritoneum (ukugqoka komgodu wesisu), nakuba ngezinye izikhathi ungabonakala kwezinye izingxenye zomzimba.

Nakuba i-DSRCT iyimfihlo, ithinta abantu abangaphansi kuka-200 emhlabeni jikelele unyaka ngamunye, ukuqonda lesi simo kungakusiza ukuthi uqaphele izimpawu ezingase zikhona futhi wazi ukuthi nini kufanele ufune ukunakekelwa kwezokwelapha. Izimo eziningi zenzeka kubasakhulayo nabantu abadala abasebancane, ngamadoda ethinta cishe izikhathi ezine kunabesifazane.

Uyini i-DSRCT?

I-DSRCT iyisofthi tissue sarcoma eyiyo enhlanganweni yama-cancers ebizwa ngokuthi yi-small round cell tumors. I-tumor ithatha igama layo ezimpawu ezimbili eziyinhloko: inezicubu ezincane, ezizungezile ze-cancer, futhi izungezwe yithishu ehlanganisiwe ebizwa ngokuthi yi-desmoplastic stroma.

Lo mdlavuza ngokuvamile ukhula njengezicucu eziningi kulo lonke umgodu wesisu kunokuba kube yi-tumor eyodwa. Izicucu zingaba ngobuningi obuningi futhi zivame ukusabalala emaphethelweni e-peritoneal, kungakho ngezinye izikhathi kubizwa ngokuthi "i-peritoneal sarcomatosis."

Okwenza i-DSRCT ibe yiyona ngokwayo ukwakheka kwayo okuqondile kwe-genetic. Izicubu ze-cancer zinezici ezithile ze-chromosomal translocation ezidala i-fusion protein engavamile, eholela ekukhuleni kwe-tumor nokuziphatha okubhubhisayo.

Ziyini izimpawu ze-DSRCT?

Izimpawu zokuqala ze-DSRCT zingaba zimfihlelekile futhi zingase zithuthuke kancane kancane ezinyangeni noma ezinyangeni ezimbalwa. Abantu abaningi baqala ukubheka lezi zibonakaliso njengezinkinga ezincane zokugaya ukudla noma izinkinga ezihlobene nokucindezeleka.

Izimpawu ezivamile ongazihlangabezana nazo zihlanganisa:

• Ubuhlungu obuhlala njalo besisu noma ukungakhululeki okungapheli ngokuphumula
• Ukukhula okukhulu kwesisu noma ukugcwala okuyanda ngokuhamba kwesikhathi
• Ukuzizwa ugcwele ngisho nangemva kokudla okuncane
• Ukulahlekelwa isisindo okungachazeki naphezu kokuthanda ukudla okuvamile noma okwandayo
• Ukuphefumula nokugabha, ikakhulukazi ngemva kokudla
• Ukushintsha kwemikhuba yokuya esihlalweni, kufaka phakathi ukuvimbela noma ukukhuluphala kokudlula
• Ukukhathala okungapheli ngokuphumula
• Ukulahlekelwa ukudla

Ezimweni eziphambili, ungase uqaphele i-mass ebonakalayo esiswini sakho ongayizwa ngesikhumba sakho. Abanye abantu nabo bahlangabezana nokuqina kokuphefumula uma i-fluid iqokelela emgodini wesisu, isimo esibizwa ngokuthi yi-ascites.

Kubalulekile ukukhumbula ukuthi lezi zimpawu zingase zenzeke ngezimo eziningi ezahlukene, eziningi zazo zivame kakhulu futhi azibi njenge-DSRCT. Noma kunjalo, uma uhlangabezana nezimpawu eziningi zalezi zimpawu ngokuqhubekayo, kufanele ukuxoxe nazo nodokotela wakho.

Yini ebangela i-DSRCT?

Imbangela eqondile ye-DSRCT isalokhu ingaziwa kakhulu, okungase kuzwakale kuyadumaza uma uzama ukuqonda ukuthi kungani lo mdlavuza ukhula. Lokho esikwaziyo ukuthi i-DSRCT ivela ekuguqukeni okuqondile kwe-genetic okwenzeka ngokungahleliwe ezicubwini ezithile.

Lolu shintsho lwe-genetic luhilela i-translocation phakathi kwe-chromosomes 11 ne-22, lidala i-fusion gene engavamile ebizwa ngokuthi yi-EWSR1-WT1. Le gene ye-fusion ikhiqiza i-protein ephazamisa ukukhula kwesicubu nokwahlukaniswa okuvamile, okuholela ekukhuleni kwezicubu ze-cancer.

Ngokungafani nezinye i-cancers, i-DSRCT ayibonakali ihlobene nokuthi:

• Izimo ze-genetic ezifuywe noma umlando womndeni
• Ukudalulwa komkhathi noma izici zokuphila
• Ukuphathwa ngaphambili kwe-radiation noma i-chemotherapy
• Ukutheleleka kwe-viral
• Ukudla noma imikhuba yokuzivocavoca

Ukuhlukahluka kwe-genetic okubangela i-DSRCT kubonakala sengathi kuyisenzakalo esingahleliwe esenzeka ngesikhathi sokuhlukaniswa kwesicubu. Lokhu kusho ukuthi ukuthuthukisa i-DSRCT akuyona into engathintelwe ngokwenza izinqumo ezahlukene noma ukuziphatha.

Nini kufanele ubona udokotela ngezimpawu ze-DSRCT?

Kufanele ucabange ukuya kudokotela uma uhlangabezana nezimpawu zesisu ezihlala njalo isikhathi esingaphezu kwamasonto amabili, ikakhulu uma ziya ziba zimbi. Nakuba lezi zimpawu zivame kakhulu ukubangelwa izimo ezivamile, kuhlale kungcono ukuba ziqaphelwe.

Funani ukunakekelwa kwezokwelapha ngokushesha uma uhlangabezana nokuthi:

• Ubuhlungu besisu obukhulu, obuzumayo
• Ukukhula kwesisu okusheshayo ezinsukwini kunokuba kube ngamasonto
• Ukugabha okukuvimbela ekugcineni ukudla noma izinto eziphuzwayo
• Izibonakaliso zokuvimbela kwamathumbu, njengokungakwazi ukukhulula igesi noma ukuya esihlalweni
• Ukulahlekelwa isisindo esikhulu okungachazeki (ngaphezulu kwamakhilogremu angu-4.5 ngaphandle kokuzimisela)
• Ukuphefumula okunzima kanye nokukhula kwesisu

Khumbula ukuthi udokotela wakho ulapho ukuze akusize uhlukanise izimpawu ezikukhathazayo. Bangenza ukuhlolwa okufanele ukuze baqonde ukuthi yini ebangela izimpawu zakho futhi bakunikeze uhlelo lokwelapha olufanele.

Ziyini izici ezingase zikubeke engozini ye-DSRCT?

I-DSRCT inezici ezimbalwa eziqondiwe ezingase zikubeke engozini, okuyintokozo futhi kuyadumaza kubaphenyi bezokwelapha. Umdlavuza ubonakala ukhula ngokungahleliwe kunokuba uhlangene nezici ezingalawuleki ezingase zikubeke engozini.

Izici eziyinhloko ezingase zikubeke engozini ezitholakele zihlanganisa:

• Ubudala: Izimo eziningi zenzeka kubantu abaneminyaka engu-10 kuya kwengu-30, ngokwanda okukhulu kweminyaka yobudala ekuqaleni kweminyaka yobudala.
• Ubulili: Amadoda athinteka cishe izikhathi ezine kunabesifazane
• Ayikho iphethini elidluliselwa ezizukulwaneni: I-DSRCT ayibonakali igijima emindenini

Ngokungafani nezinye i-cancers eziningi, i-DSRCT ayihlobene nokubhema, ukusetshenziswa kotshwala, ukudla, ukuzivocavoca, ukudalulwa komsebenzi, noma ukwelashwa kwezokwelapha okudlule. Lokhu empeleni kungase kube ukududuza ukwazi, njengoba kusho ukuthi cishe akukho lutho obungalwenza ngokuhlukile ukuze ulivimbele.

Ukuba yimfihlo kwalo mdlavuza kusho nokuthi ngisho nabantu abasemaqenjini abekwe engozini enkulu (amadoda asebancane) banesilinganiso esincane kakhulu sokuthuthukisa i-DSRCT. Ingozi yonke ihlala ingaphansi kuka-1 kuya kwabantu abayizigidi eziyisigidi ngonyaka.

Ziyini izinhlanganisela ezingase zenzeke ngenxa ye-DSRCT?

I-DSRCT ingase iholele ezinhlanganisela eziningi, ikakhulukazi ngenxa yendlela ekhula futhi isabalala ngayo kulo lonke umgodu wesisu. Ukuqonda lezi zinhlelo ezingase zenzeke kungakusiza ukuthi uqaphele ukuthi nini izimpawu zingase zibe zimbi kakhulu.

Izinhlanganisela ezivamile zihlanganisa:

• Ukuvimbela kwamathumbu: Ama-tumors angavimba amathumbu, avimbele ukugaya ukudla okuvamile
• I-Ascites: Ukubunjwa kwe-fluid emgodini wesisu okubangela ukukhula nokungakhululeki
• Ukondleka okungalungile: Ngenxa yokuthanda ukudla okubi, ukuphefumula, nobunzima bokudla
• Ama-blood clots: I-cancer ingase ikhulise ingozi yama-clots akhiqizwa e-blood vessels
• Izinkinga zezinso: Uma ama-tumors ecindezela noma ehlanganisa isimiso sokuphuma kwesisu

Ezimweni eziphambili, i-DSRCT ingase isabalale ngaphandle komgodu wesisu ezinye izitho, ikakhulukazi isibindi, amaphaphu, noma ama-lymph nodes. Noma kunjalo, lo mkhuba wokusabalala kude awuvamile njengokusabalala kwendawo ngaphakathi kwesisu.

Kubalulekile ukwazi ukuthi ukunakekelwa okuhambisana okunamuhla kungalawula ngempumelelo izinhlanganisela eziningi zalezi, kusize ukugcina ikhwalithi yokuphila ngesikhathi sokwelashwa. Iqembu lakho lezokwelapha lizokulandelela lezi zinkinga futhi lizixazulule ngokushesha uma zithuthuka.

I-DSRCT ihlonishwa kanjani?

Ukuhlonishwa kwe-DSRCT ngokuvamile kuhilela izinyathelo eziningi, njengoba odokotela bedinga ukuqeda izimo ezivamile kuqala. Inqubo ngokuvamile iqala ngomlando wakho wezokwelapha nokuhlolwa komzimba wesisu sakho.

Udokotela wakho cishe uzokubeka ukuhlolwa kwezithombe ukuze uthole umbono omuhle wento eyenzeka ngaphakathi kwesisu sakho. I-CT scan yesisu ne-pelvis ngokuvamile iyisifundo sokuqala sokuthola izithombe esenziwa, njengoba ingabonisa ubukhulu, indawo, nobuningi bama-masses akhona.

Ukuhlolwa okwengeziwe kungase kuhlanganise:

• Ama-MRI scans ukuze uthole izithombe eziningi zezicubu ezithambile
• Ama-PET scans ukuze uhlole umsebenzi we-cancer kulo lonke umzimba
• Ukuhlolwa kwegazi ukuze uhlole impilo yakho yonke nomsebenzi wezitho
• Ama-tumor markers, nakuba lawa awuchazi i-DSRCT

Ukuhlonishwa okuqinisekile kudinga i-biopsy, lapho isibonelo esincane sethishu sisuswa futhi sihlolwe ngaphansi komkhiqizo. I-pathologist izobheka izicubu ezincane ezizungezile futhi enze ukuhlolwa okukhethekile ukuze iqinisekise i-EWSR1-WT1 gene fusion echaza i-DSRCT.

Le nqubo yokuhlola ingathatha izinsuku ezimbalwa kuya emakhulwini, okungase kuzwakale kuyadumaza. Khumbula ukuthi le ndlela ephelele iqinisekisa ukuthi uthola ukuhlonishwa okuqondile okungenzeka, okubalulekile ekucwaningeni uhlelo lokwelapha oluhle kakhulu.

Yini ukwelashwa kwe-DSRCT?

Ukwelashwa kwe-DSRCT ngokuvamile kuhilela indlela enezinyathelo eziningi ehlanganisa izinhlobo ezahlukene zokwelapha. Umgomo ukunciphisa ama-tumors ngangokunokwenzeka futhi ulawule isifo isikhathi eside.

Indlela yokwelapha evamile ngokuvamile ihlanganisa:

1. I-Chemotherapy: Izilinganiso eziningi zamankosi e-anti-cancer drugs ezihanjiswa izinyanga eziningi
2. Ukuhlinzwa: Ukususa i-tumor eningi ngangokunokwenzeka, ngokuvamile kuhlanganiswe ne-chemotherapy eshisayo ngqo esiswini
3. I-Radiation therapy: Ama-beams aphezulu amandla aqondiswe ezicubwini ze-cancer ezisele

Isigaba se-chemotherapy ngokuvamile siqala kuqala futhi singathatha izinyanga ezingu-4-6. Ukusetshenziswa kwemithi okuvamile kuhlanganisa i-ifosfamide, i-carboplatin, i-etoposide, ne-doxorubicin. Lezi zidakamizwa zisebenza ngokuhlose izicubu ze-cancer ezihlukanisayo ngokushesha.

Ukuhlinzwa, uma kungenzeka, kuhilela inqubo ebizwa ngokuthi yi-cytoreductive surgery nge-hyperthermic intraperitoneal chemotherapy (HIPEC). Lokhu kuhilela ukususa ama-tumors abonakalayo bese kuhlanzwa umgodu wesisu ngemithi ye-chemotherapy eshisayo.

Kuyo yonke inqubo yokwelashwa, iqembu lakho lezokwelapha lizophinde ligxile ekunakekelweni okuhambisanayo ukuze lisize ukulawula imiphumela emibi futhi ligcine amandla akho nekhalithi yokuphila. Lokhu kungase kuhlanganise imithi yokuphefumula, ukusekelwa kokudla, nokwelashwa ukuvimbela ukutheleleka.

Indlela yokulawula izimpawu ekhaya ngesikhathi sokwelashwa kwe-DSRCT?

Ukulawula izimpawu ekhaya kungakusiza uzizwe ukhululekile futhi ugcine amandla akho ngesikhathi sokwelashwa. Ukulungiswa okuncane kwansuku zonke kungenza umehluko omkhulu endleleni ozizwa ngayo ngokujwayelekile.

Ngezimpawu zokugaya ukudla, ukudla ukudla okuncane, okuvamile kuvame ukusebenza kangcono kunokuzama ukudla izingxenye ezinkulu. Gxila ekudleni okulula ukukugaya futhi kukuthande, ngisho noma okuthandayo okwejwayelekile kungazwakali kuhle njengamanje.

Ukulawula ukukhathala:

• Phumula uma udinga, kodwa zama ukuhlala usebenza kancane uma kungenzeka
• Hamba izinyathelo ezimfushane noma wenze ukwelula okulula njengoba kungenzeka
• Gcina isimiso sokulala esijwayelekile uma kungenzeka
• Cela usizo lwabantu bakubo nabangane emisebenzini yansuku zonke

Ngokuphefumula nokudla okungathandeki, zama ukudla ukudla okungathandeki njenge-crackers, i-toast, noma irayisi. Itiye le-ginger noma izithasiselo ze-ginger zingasiza ngokuphefumula. Hlala uthambile ngokuphuza izinto ezincane usuku lonke.

Gcina ulandelela izimpawu zakho nemiphumela emibi ukuze ukwazi ukuyixoxa neqembu lakho lezokwelapha. Bavame ukulungisa imithi noma banikeze ukwelashwa okuhambisanayo okwengeziwe ukuze bakusize uzizwe kangcono.

Kufanele ulungiselele kanjani iziqu zakho zokubona udokotela?

Ukulungiselela iziqu zakho zokubona udokotela kungakusiza ukwenza okuningi ngesikhathi sakho neqembu lakho lezokwelapha futhi kuqinisekise ukuthi uthola yonke imibuzo yakho iphendulwe. Ukulungiselela okuncane kuhamba ibanga elikhulu ekusikweni uzizwe ulawula ukunakekelwa kwakho.

Ngaphambi kwesikhathi ngasinye sokubona udokotela, bhala izimpawu zakho zamanje, kufaka phakathi ukuthi zaqala nini nokuthi zishintshile kanjani. Qaphela noma yiziphi izimpawu ezintsha noma imiphumela emibi oyithola ngokwelashwa.

Lungiselela uhlu lwemibuzo ofuna ukuyibuza:

• Yini eboniswa yimiphumela yami yokuhlola yakamuva?
• Ukwelashwa kwami kusebenza kanjani?
• Yimiphi imiphumela emibi engifanele ngiyilandele?
• Ingabe kunezindlela zokulawula izimpawu zami zamanje kangcono?
• Nini ukwelashwa kwami okulandelayo kuhlelwe?

Letha uhlu oluphelele lwazo zonke imithi oyithathayo, kufaka phakathi imithi yokuthengwa ngaphandle komthetho kanye nezithasiselo. Cabanga ukuletha ilungu lomndeni noma umngani ukuze akusize ukukhumbula lokho okuxoxwe ngakho nokunikeza ukwesekwa ngokomzwelo.

Ungangabazi ukucela incazelo uma ungaqondi lutho. Iqembu lakho lezokwelapha lifuna ukuqinisekisa ukuthi uqonda ngokugcwele isimo sakho nehlelo lokwelapha.

Yini into ebalulekile mayelana ne-DSRCT?

I-DSRCT iyimfihlo kodwa iyisifo esibi somdlavuza esithinta ikakhulukazi abantu abasebancane. Nakuba ukuhlonishwa kungase kuzwakale kuyadumaza, ukuthuthuka ekwelapheni kuye kwathuthukisa imiphumela kubantu abaningi eminyakeni yamuva.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi awuwedwa kule ndlela. Iqembu lakho lezokwelapha linolwazi lokwelapha lo mdlavuza omfihlekile futhi lizosebenza nawe ukuze lenze uhlelo lokwelapha oluhle kakhulu esimweni sakho esithile.

Ukuqaphela izimpawu ngokushesha nokufuna ukunakekelwa kwezokwelapha ngokushesha kungenza umehluko emiphumeleni yokwelapha. Uma uhlangabezana nezimpawu zesisu ezihlala njalo, ikakhulu uma ungumuntu osemncane, ungangabazi ukuxoxa nazo nodokotela wakho.

Khumbula ukuthi ukuba nezimpawu ezikukhathazayo akusho ukuthi une-DSRCT. Lo mdlavuza uyimfihlo kakhulu, futhi izimpawu zakho zivame kakhulu ukubangelwa isimo esivamile, esingaphathwa. Noma kunjalo, ukuhlolwa kukunika ukuthula kwengqondo futhi kuqinisekisa ukuthi uthola ukunakekelwa okufanele kungakhathaliseki ukuthi imbangela iyini.

Imibuzo evamile ebuzwayo mayelana ne-DSRCT

Ingabe i-DSRCT ilidluliselwa ezizukulwaneni?

Cha, i-DSRCT ayilidluliselwa ezizukulwaneni futhi ayigijimi emindenini. Ukuhlukahluka kwe-genetic okubangela lo mdlavuza kubonakala kwenzeka ngokungahleliwe ngesikhathi sokuhlukaniswa kwesicubu. Ukuba nelungu lomndeni eline-DSRCT akukhuphuli ingozi yakho yokuthuthukisa.

I-DSRCT iyimfihlo kangakanani?

I-DSRCT iyimfihlo kakhulu, ngezimo ezintsha ezingaphansi kuka-200 ezibikwa emhlabeni jikelele unyaka ngamunye. Ukuze sibeke lokhu ngokucacile, kunokwenzeka kakhulu ukuthi ushaywe yisibane kunokuthi uthuthukise i-DSRCT. Ukuba yimfihlo kwalo mdlavuza kuyisizathu esisodwa sokuthi kungani kunzima ukuhlonishwa ekuqaleni.

Ingabe i-DSRCT ingaphiliswa?

Nakuba i-DSRCT iyisifo esibi somdlavuza, abanye abantu bayafinyelela ekuphumuleni isikhathi eside ngokwelashwa okukhulu. Ukuhlanganiswa kwe-chemotherapy, ukuhlinzwa, ne-radiation therapy kuye kwakusiza abanye abantu ukuphila bengenasifo iminyaka eminingi. Imiphumela yokwelapha iyaqhubeka ithuthuka njengoba odokotela befundela kabanzi ngalo mdlavuza omfihlekile.

Yimiphi iminyaka evamile lapho i-DSRCT ihlonishwa khona?

Abantu abaningi abane-DSRCT bahlonishwa phakathi kweminyaka engu-10 kuya kwengu-30, ngenani elikhulu lezimpawu eyenzeka ekuqaleni kweminyaka yobudala. Noma kunjalo, izimo zibikiwe kubantwana abaneminyaka engu-5 kanye nabantu abadala abaneminyaka engu-50, nakuba lokhu kungavamile kakhulu.

Ukwelashwa kwe-DSRCT ngokuvamile kuthatha isikhathi esingakanani?

Inqubo ephelele yokwelashwa ngokuvamile ithatha izinyanga ezingu-12-18, nakuba lokhu kungase kuhluke kuye ngezimo zomuntu ngamunye. Lokhu kuhlanganisa izinyanga eziningi ze-chemotherapy, okulandela ukuhlinzwa (uma kungenzeka), bese kuba ne-chemotherapy eyengeziwe noma i-radiation therapy. Iqembu lakho lezokwelapha lizokunikeza isimiso sesikhathi esithile esisekelwe ehlelweni lakho lokwelapha.