Ngaba uyazi ngalo mhlaza ungaqhelekanga? Masithethe nge-NUT Carcinoma

Ngaba wakha weva nge-NUT Carcinoma? Mhlawumbi akunjalo. Kuba luhlobo lomhlaza olungaqhelekanga, olungaqhelekanga kakhulu, nolukhula ngokukhawuleza. Kodwa kubaluleke kakhulu ukwazi ngalo, kuba le meko inokukhula nakubani na. Ngoko ke namhlanje siza kuthetha ngalo ngokweenkcukacha, ngokungathi sithetha nomhlobo.

Yintoni i-NUT Carcinoma?

Ngamafutshane, i-NUT Carcinoma luhlobo lomhlaza olukhula ngokukhawuleza kakhulu nolunobundlongondlongo . Luthathwa njengolona lundlongondlongo kakhulu kwii-squamous cell carcinomas. Ngoku usenokuba uzibuza ukuba zeziphi ii-squamous cells. Ezi luhlobo lweeseli ezifumaneka eluswini lwethu. Ayisiyiyo loo nto kuphela, kodwa nezinye izitho zangaphakathi zomzimba wethu nazo zigqunywe zezi seli.

Le NUT Carcinoma ibangelwa kukuguquka kwezakhi zofuzo kwiijini zethu. Ngokuchanekileyo, ibangelwa sisiphene kwijini ebizwa ngokuba yi `NUTm1`. Uyazi, ezi jini zinika iiseli zethu imiyalelo yendlela yokusebenza. Ngoko ke, xa kukho impazamo kwezi miyalelo, oko kukuthi, `ukuguqulwa`, neeseli eziphilileyo ziqala ukujika zibe `iiseli ezinomhlaza`. Emva koko ezi seli zomhlaza ziyaqokelelana zenze `iithumba`.

Amaxesha amaninzi, ezi carcinomas ze-NUT zikhula phakathi emzimbeni wethu, okanye ecaleni kwe-"midline" . Yiyo loo nto zazibizwa ngokuba yi-NUT-midline carcinoma ngaphambili. Nangona kunjalo, ezi tumors zinokukhula nakwezinye iindawo zomzimba.

Zingavela phi ezi thumba zinomhlaza?

Abantu abafunyaniswe bene-NUT Carcinoma banezimila kwezi ndawo zilandelayo emizimbeni yabo:

• Intloko, ingakumbi kwiisinuses (le yeyona ixhaphakileyo)
• Entanyeni (oku kubonwa rhoqo)
• Kwimiphunga (oku kuqhelekile)
• Kwindawo ephakathi yesifuba, indawo apho intliziyo nemiphunga ikhona khona (i-'mediastinum') (oku kuqhelekile)
• I-Pancreas
• Izintso
• Iindlala ze-adrenal
• Isisu
• Isinyi
• Emathanjeni (`Amathambo`)

Lo mhlaza, obizwa ngokuba yi-NUT Carcinoma , unokukhula kwaye usasazeke ngokukhawuleza, nokuba unyangiwe . Leyo yeyona nto iyingozi ngayo.

Inqabile kangakanani i-NUT Carcinoma?

Oku kuthathwa njengomhlaza ongaqhelekanga . Nangona kunjalo, kunokubakho izigulane ezininzi kunokuba sicinga. I-International NUT Midline Carcinoma Registry ibika amatyala amatsha angama-20 kuphela ngonyaka.

Esinye isizathu sokuba kubekho eli nani liphantsi kubunzima bokufumanisa esi sifo. Izazinzulu zifumene uphawu oluphambili lwalo mhlaza, "uguquko" kwijini ye-"NUTm1", ngo-2004. Ngoko ke, ayizizo zonke izibhedlele ezine "zixhobo zovavanyo" ezinokubona esi jini.

Cinga nje ngokufumanisa isifo esitsha. Kuthatha ixesha ukuba uvavanyo olufanelekileyo lufumaneke kuyo yonke indawo.

Kwakhona, i-NUT Carcinoma idideka lula nezinye iintlobo zomhlaza eziqhelekileyo. Kunokwenzeka kuphela ukufumanisa ngokuchanekileyo ukuba umhlaza yi-NUT Carcinoma ukuba i-'NUTm1` gene mutation ichongiwe. Abanye abantu abafunyaniswe benomhlaza wentloko nentamo, 'umhlaza wemiphunga', kunye nezinye iintlobo zomhlaza banokuba nalo mhlaza.

Kodwa ngoku, ngenxa yokuba ulwazi ngolu hlobo lomhlaza luye lwanda, kwaye ngenxa yokuba ubuchwepheshe bokufumanisa olu tshintsho lwemfuza luye lwaphucuka, kuye kwaba lula kwiingcali zomhlaza ukufumanisa i-NUT carcinoma kunangaphambili.

Ngubani ofumana i-NUT Carcinoma?

Lo mhlaza udla ngokufunyaniswa kubantu abancinci . Ubudala obuphakathi bokuxilongwa bumalunga neminyaka engama-24. Oku kuthetha ukuba malunga nesiqingatha sabo bafunyenwe benaso singaphantsi kweminyaka engama-24 ubudala, kwaye esinye isiqingatha sikhulile.

Nangona kunjalo, lo mhlaza ungakhula nakubani na . Ufunyenwe kwiintsana ezisandul’ ukuzalwa, nakubantu abakwiminyaka yabo yama-80. Ngoko ke akunakwenzeka ukucinga ukuba awuyi kwenzeka ngokuxhomekeke kwiminyaka yobudala.

Zithini iimpawu ze-NUT Carcinoma?

Ixesha elininzi, lo mhlaza awubonisi zimpawu kwasekuqaleni . Iimpawu ziqala ukubonakala xa umhlaza ukhule kancinci kwaye uqale ukuchaphazela izicubu ezijikeleze ithumba. Ngelo xesha, unokuziva udiniwe kwaye ungaphilanga kakuhle.

Ngenxa yokuba iithumba zihlala zikhula kwimingxunya ye-sinus kunye nemiphunga, ezona mpawu zixeliweyo zinxulumene nazo.

Iimpawu eziqhelekileyo:

• Ukudinwa/ukudinwa kakhulu (`Ukudinwa`)
• Ukunciphisa umzimba ngendlela engachazekiyo
• Iqhuma elibuhlungu okanye ukudumba (`Iqhuma elibuhlungu`)

Iimpawu ze-sinus cyst:

• Uxinzelelo lweSinus, ukuqina, okanye intlungu
• Ukopha rhoqo kwempumlo
• Ukuxinana kwempumlo okanye ukuxinana
• Ukulahlekelwa yimvakalelo yakho yokunuka

Iimpawu zethumba lemiphunga:

• Ukuphefumla nzima okanye ukuphefumla nzima
• Ukukhwehlela okungapheliyo (ukukhwehlela okungapheliyo okungapheliyo)

Musa ukoyika i-NUT Carcinoma kuba nje unale mpawu. Kodwa ukuba ezi mpawu ziyaqhubeka,Kubaluleke kakhulu ukubona ugqirha kwaye ufumane ingcebiso.

Yintoni ebangela i-NUT Carcinoma?

Lo mhlaza ubangelwa kukudibana kweejini ezimbini ezahlukeneyo, okudala ijini exutyiweyo engaqhelekanga (`ijini exutyiweyo` okanye ijini exutyiweyo`). Kuwo wonke umguli one-NUT Carcinoma, ijini ethi `NUTm1` idityaniswa nenye ijini ekungafanele ukuba idityaniswe nayo. Ijini ethi `NUTm1` ayibangeli umhlaza yodwa, kodwa le jini exutyiweyo entsha yiyo ebangela ukuba iiseli zomhlaza zikhule.

Kwi-75% yabantu abane-NUT carcinoma, i-'NUTm1` gene idityaniswe ne-'BRD4` gene. Kwi-15% enye, i-'NUTm1` gene idityaniswe ne-'BRD3` gene. Kunqabile kakhulu ukuba idityaniswe nezinye ii-gene.

Izazinzulu azikaqiniseki ukuba yintoni ebangela olu tshintsho lwemfuza. Kodwa kukho into enye ecacileyo: aluzuzwa njengelifa. Izinto ezinobungozi bomhlaza, ezifana nokutshaya nokuchaphazeleka zizinto ezibangela umhlaza, azibonakali zibandakanyeka. Kufuneka uphando olungakumbi ukuze kuqondwe ukuba yintoni ephembelela olu tshintsho lwemfuza.

Ifunyanwa njani i-NUT Carcinoma?

Oogqirha bafumanisa esi sifo nge-biopsy. Oku kuquka ukuthatha isampuli encinci yezicubu ezivela kwithumba besebenzisa inaliti ekhethekileyo engenanto. Emva koko isampuli ithunyelwa kwilabhoratri ukuze ivavanywe iiseli zomhlaza.

Ugqirha wakho uza kusebenzisa iinkqubo ezikhethekileyo zokuthatha imifanekiso ukujonga ithumba elingaphakathi emzimbeni wakho aze amisele inqanaba lalo. Ukumisela umhlaza yinkqubo yokufumanisa ukuba umhlaza unzima kangakanani, ngokusekelwe kubukhulu bethumba, indawo elikuyo, kunye nokuba lisasazeke na (lisasazeke) ukusuka kwithumba eliphambili ukuya kwezinye iindawo zomzimba. Ukwanda komhlaza kwenzeka xa iiseli zomhlaza ziphuma kwithumba lokuqala, zihamba ngolwelo lwe-lymph okanye igazi, kwaye zenze iithumba ezintsha kwizitho okanye kwizicubu ezikude.

I-CT scan kunye ne-MRI scan zinokubonisa apho ithumba likhona emzimbeni. I-PET scan inokubonisa ukuba umhlaza usasazekile na.

Zeziphi iimvavanyo ezenziwayo ukuze kufunyaniswe esi sifo?

Uvavanyo olukhethekileyo olubizwa ngokuba yi-`Immunohistochemistry` lwenziwa kwilebhu ukuze kufunyaniswe ukuba i-`NUTm1` gene ikhona na kwisampuli ye-biopsy. Ukuba i-`NUTm1` gene ikhona kwisampuli (`positive`), ifunyaniswa njenge-NUT Carcinoma.

Ezinye iimvavanyo ezinokwenziwa ukuchonga le gene luvavanyo lweFluorescence In Situ Hybridization (FISH) okanye ezinye iimvavanyo zemfuza ezikhethekileyo.

Inyangwa njani i-NUT Carcinoma?

Ungayisusa ithumba.Kunokufuneka utyando kunye nonyango lwemitha. Ukuba umhlaza usasazeke ngaphaya kwethumba, i-chemotherapy nayo inokunikwa. Rhoqo, olunye okanye ngaphezulu kwezi ndlela zonyango lunikwa kunye.

Nangona kunjalo, ukunyanga i-NUT carcinoma kunzima kakhulu. Rhoqo, nokuba unyango luyawuthintela umhlaza ukuba ungabi mbi, uya kuqala ukukhula kwaye usasazeke kwakhona emva kwexesha elithile.

Eyona nto ibalulekileyo ngamaxesha anje kukuhlala siqinile. Kufuneka simamele oko kuthethwa ngoogqirha size sijongane nako kunye nabo.

Ukuba ufunyaniswe une-NUT Carcinoma, uvavanyo lweklinikhi lunokuba lolona khetho lufanelekileyo lonyango . Uvavanyo lweklinikhi zizifundo ezivavanya unyango olutsha okanye iindlela zonyango. Oososayensi okwangoku baphanda unyango olujoliswe kwi-NUT Carcinoma. Unyango olujoliswe kwi-target lunyango olujolise kwinguqu yemfuza ebangela ukuba iseli ephilileyo ibe ngumhlaza.

Enye yeendlela zonyango ezijoliswe kuzo ze-NUT carcinoma zi -'BET` inhibitors . La mayeza ajolise kwiijini ezimbini, i-'BRD4` kunye ne-'BRD3`, eziqhele ukunxulunyaniswa nejini ye-'NUTm1`. Ezi zithinteli, kunye nejini ye-'NUTm1`, ziyamisa ukukhula kweeseli zomhlaza.

Ngaba i-NUT Carcinoma inganyangeka?

Ngelishwa, akukho nyango lwe-NUT Carcinoma. Ngumhlaza okhula ngokukhawuleza osasazeka ngokukhawuleza kwamanye amalungu omzimba. Ngokomyinge, abantu abafumana unyango baphila malunga neenyanga ezili-10 emva kokuba befunyaniswe benale ngxaki. Abantu ababini ukuya kwabathathu kwabalishumi abafunyaniswe bene-NUT Carcinoma baphila iminyaka emibini.

Kodwa, njengakuyo nayiphi na imhlaza, ikamva lakho, okanye ukuba uza kusabela njani kunyango, kuxhomekeke kwizinto ezininzi. Indawo ekuyo ithumba, nokuba linokususwa ngokupheleleyo na ngotyando okanye ngonyango lwemitha, kunye nezinye izifundo zibonise ukuba ezinye ii-genes ezidityaniswe ne-NUTm1 gene nazo zidlala indima.

Ugqirha wakho uza kukuchazela iinjongo zonyango ngokusekelwe kwisifo sakho.

Ndingazinyamekela njani?

Kubalulekile ukuzingqonge nabantu obathembayo nobaxhasayo ngeli xesha. Sebenzisa zonke izixhobo ezikhoyo ukukunceda uhlangabezane nemingeni eza nolu xilongo.

Unyango lokudambisa iintlungu oluvela kugqirha wakhoFunda ngeenkonzo. Iingcali zokhathalelo lwentlungu zingakunceda ulawule iimfuno ezintsha zobomi bakho ngenxa yolu xilongo. Zinokukunceda ulawule iziphumo ebezingalindelekanga ze-chemotherapy kunye nonyango lwe-radiation, kwaye zikuncede ubhekane noxanduva lwemihla ngemihla oluza nomhlaza.

Khumbula, awuwedwa. Ungaze unqikaze ukucela uncedo nenkxaso.

Ndingayibuza yiphi imibuzo ugqirha wam?

Zininzi izinto onokuthi uzibuze ugqirha wakho ngexesha elinje. Nazi ezinye zemizekelo:

• Ingaba umhlaza usasazekile?
• Ngaba kufuneka ndidibane nogqirha okanye ndiye kwisibhedlele esithile ukuze ndifumane unyango?
• Luhlobo luni lonyango olucetyiswayo?
• Ziziphi iingenelo kunye neengozi ezinokubakho zonyango?
• Zithini iinjongo zonyango?
• Ungandinceda ndifumane ezinye iinkonzo zenkxaso, kuquka nonyango lokuthomalalisa iintlungu?

Unyango lwe-NUT Carcinoma lunokuba nzima kakhulu. Ngokudibana rhoqo nogqirha we-oncologist kunye noxinzelelo lobomi bemihla ngemihla, ukujongana nokuxilongwa komhlaza kunokuba nzima. Izixhobo zonyango ezifana nonyango lokudambisa iintlungu zinokunceda. Kwaye ukufikelela kubantu obathandayo kunokuba ngumthombo omkhulu wamandla. Ukususela kwangoko xa ufumana ukuxilongwa kwakho, kubalulekile ukuthetha ngokukhululekileyo neqela lakho lokhathalelo lomhlaza ukuze uqonde ezona ndlela zibalaseleyo zokhathalelo oluya phambili. Amava omhlaza womntu wonke ahlukile. Iqela lakho lezonyango likho ukuze likukhokele kweli xesha linzima.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, masishwankathele ezona zinto zibalulekileyo ekufuneka uzikhumbule kwinto esithethe ngayo:

• I-NUT Carcinoma luhlobo lomhlaza olungaqhelekanga, kodwa olusasazeka ngokukhawuleza nolunobundlongondlongo.
• Oku kubangelwa kukuguquka kwejini ethi ``NUTm1``. Oku akuyonto ezuzwa njengelifa.
• Iimpawu zihlala zibonakala emva kokuba umhlaza udlule kancinci. Musa ukuyityeshela, kwaye funa ingcebiso kagqirha ukuba uneempawu ezingaqhelekanga ezihlala zikho.
• Kufuneka uvavanyo lwe-biopsy kunye novavanyo olukhethekileyo lwe-genetic ukuze kufunyanwe isifo.
• Unyango lunzima, kodwa uphando luyaqhubeka malunga nonyango olutsha. Uvavanyo lwezonyango lunokuba lukhetho olufanelekileyo.
• Ukhathalelo oluthomalalisayo kunye nenkxaso yabantu obathandayo kubaluleke kakhulu kolu hambo.
• Thetha ngokukhululekileyo ngayo yonke into neqela lakho lezonyango.

Ndiyathemba ukuba olu lwazi luluncedo kuwe. Ukuqaphela esi sifo kuya kukunceda wenze izigqibo ezifanelekileyo xa ixesha lifika.

I -NUT Carcinoma, umhlaza, utshintsho lwemfuza, umhlaza ongaqhelekanga, umhlaza we-squamous cell, iimpawu zomhlaza, unyango lomhlaza