Izimpawu, Ukwelashwa, Nokuguqulwa Kwe-Neuroblastoma
Uma ngabe ingane yakho noma ingane yomngane wakho itholwe nge-neuroblastoma, yini okudingeka uyikwazi? Kujwayelekile kangakanani lolu hlobo lomdlavuza wezingane? Kuphathwa kanjani futhi yikuphi ukubikezela?
Iyiphi i-Neuroblastoma?
Njengenye yezinhlobo ezivame kakhulu zomdlavuza ebuntwaneni, i-neuroblastoma ingumdlavuza otholakala ezinganeni ezincane. I-tumor iqala ngama-neuroblasts (amangqamuzana angama-immter cells) we-system central negazi.
Ikakhulukazi, i-neuroblastoma ihlanganisa amangqamuzana omgogodla ayingxenye yesimiso somzwelo ozwelayo. Izinhlelo zokuzwa izinzwa ezizwela futhi ezihlukumezayo ziyingxenye yesimiso sezinzwa ezizimele, uhlelo olulawula izinqubo emzimbeni ukuthi asidingi ukucabanga ngokuphefumula nokugaya. Isistimu yomzwelo ezwelayo yenzelwe ukusabela "ukulwa noma ukushayela" okwenzeka lapho sigxilile noma besaba.
Isistimu yomzwelo enozwela inezindawo ezibhekiswe njenge-ganglia, ezitholakala emazingeni ahlukene omzimba. Kuncike ekutheni i-neuroblastoma iqala kuphi kulesi simiso, ingase iqale ezinhlobonhlobo eziyingozi (cishe ingxenye yesithathu yamacala), isisu, isifuba, isifuba noma intamo.
Izibalo Nge-Neuroblastoma
I-neuroblastoma ingumdlavuza ovamile wabantwana , kubalwa amaphesenti angu-7 e-khansa engaphansi kweminyaka engu-15, kanti amaphesenti angama-25 ekhansela athuthuka phakathi nonyaka wokuqala wokuphila.
Ngakho-ke, uhlobo oluvame kakhulu lomdlavuza ezinganeni. Kuyinto evamile kakhulu emadodeni kunabesifazane. Amaphesenti angama-65 ama-neuroblastomas atholakala ngaphambi kokuba abe nezinyanga ezingu-6 ubudala, futhi lolu hlobo lomdlavuza aluvamile ngemva kweminyaka eyi-10. I-neuroblastoma inesibopho sezingu-15 ezingamaphesenti angu-15 okufa komdlavuza ezinganeni.
Izimpawu Nezibonakaliso Ze-Neuroblastoma
Izimpawu nezimpawu ze-neuroblastoma ziyahlukahluka kuye ngokuthi zivelaphi emzimbeni nokuthi ngabe umdlavuza usakaze yini kwezinye izifunda noma cha.
Isibonakaliso esivela kunazo zonke sokuletha isisindo esikhulu samathumbu. Isisindo singase senzeke nakweminye imiphakathi lapho kukhona khona i-ganglia njengesifuba, isikhumba, noma intamo. Kungenzeka ukuthi kunomkhuhlane futhi izingane kungenzeka ukuthi zenzeke isisindo noma "ukuhluleka ukuphumelela."
Uma umdlavuza usakazeke (okuvame ukufika emnothweni wesibindi, isibindi, noma amathambo) ezinye izimpawu zingase zibe khona. Ama- metastase ngemuva nangaseduze kwamehlo (ama-metastases we-periorbital) angabangela ukuba usana lwangenele (i- proptosis ) ngemibuthano emnyama ngaphansi kwamehlo akhe. Ama-metastases esikhumbeni angabangela ama-blue patches (ama-ecchymoses) aphakamise igama elithi "i-blueberry muffin baby." Ukucindezela emgodleni ovela emathanjeni amathambo kungabangela izimpawu zamathumbu noma isithombeni. Ama-metastases amathambo amathambo avame ukubangela ubuhlungu futhi angabangela ukuhlukunyezwa kwegazi (ukwephulwa kwethambo elibuthakathaka ngenxa yokutholakala komdlavuza ethambo).
Uphi i-Neuroblastomas Spread (Metastasize)?
I-neuroblastomas ingasakazeka kusuka kusayithi lokuqala ngokuyi-bloodstream noma uhlelo lymphatic.
Izindawo zomzimba ezisakazeka kakhulu zihlanganisa:
- Amangqamuzana (okwenza ubuhlungu bethambo nobuhlungu)
- Isibindi
- Amapulazi (okwenza kube nzima ukuphefumula noma ukuphefumula)
- Umnkantsha we-Bone (okwenza kube nzima kakhulu futhi ubuthakathaka ngenxa yegazi legazi)
- Isifunda se-Periorbital (eduze kwamehlo okwenza bulging)
- Isikhumba (esibangela ukubukeka kwe-muffin e-blueberry)
Ukuxilongwa kwe-Neuroblastoma
Ukuxilongwa kwe-neuroblastoma ngokuvamile kufaka phakathi kokubili ukuhlolwa kwegazi okufuna amaki (izinto ezakhiwe ngamangqamuzana omdlavuza) kanye nezifundo zokucabanga.
Njengengxenye yesimiso somzwelo ozwelayo, amaseli we-neuroblastoma ahlanganisa ama-hormone aziwa ngokuthi ama-catecholamines. Lezi zihlanganisa ama-hormone afana ne-epinephrine, i-norepinephrine, ne-dopamine.
Izinto ezivame ukulinganisa ekwenzeni ukuxilongwa kwe-neuroblastoma zifaka i-homovanillic acid (HVA) ne-vanillylmandelic acid (i-VMA). I-HVA ne-VMA yi-metabolites (imikhiqizo yokudiliza) ye- norepinephrine ne-dopamine.
Ukuhlola ukuhlolwa okuwenzelwe ukuhlola isisu (nokubhekisisa ama-metastase) kungabandakanya ukuhlola kwe-CT, i-MRIs kanye ne- PET scan . Ngokungafani ne-CT ne-MRI okuyizifundo "zesakhiwo", izibalo ze-PET ziyizifundo "zokusebenza". Kulesi sivivinyo, inani elincane le-ushukela omsakazo lujojowe emsakazweni wegazi. Amaseli akhula ngokushesha, njengama-cell tumor, athatha okungaphezulu kwalokhu ushukela futhi angatholakala nge-imaging.
I- bone marrow biopsy ivame ukwenza kahle kusukela lezi zicubu zivame ukusabalalisa emangeni.
Isivivinyo esiyingqayizivele se-neuroblastomas yi-scan ye-MIBG. I-MIBG imelela i-meta-iodobenzylguanidine. Amaseli we-Neuroblastoma athatha i-MIBG ehlangene ne-iodine yama-radioactive. Lezi zifundo ziwusizo ekuhloleni amathisaseli we-bone kanye nokubandakanyeka komnyo we-bone.
Olunye uhlolo oluvame ukukwenziwa isifundo se-MYCN sokukhulisa. I-MYCN iyigugu elibalulekile ekukhuleni kweseli. Ezinye ze-neuroblastomas zinamakhophi angaphezu kwalesi sakhi (amakhophi angaphezu kuka-10), okutholakele okubizwa ngokuthi "i-MYCH ukukhulisa." I-Neuroblastomas nge-amplification ye-MYCN ayinakwenzeka kakhulu ukubhekana nemithi yokwelashwa ye-neuroblastoma futhi kungenzeka ukuthi ikwazi ukubhekana nezinye izifunda zomzimba.
I-Neuroblastoma Screening
Njengoba amazinga e-urine vanillylmandelic acid kanye ne-homovanillic acid elula kakhulu ukuthola futhi engavamile amazinga atholakala kumaphesenti angama-75 kuya kwangu-90 ama-neuroblastomas, kungenzeka ukuthi ukuxoxwa ngazo zonke izingane kulesi sifo.
Izifundo zibuke ukuhlolisisa ngalezi zivivinyo, ngokuvamile ezinyangeni ezingu-6 ubudala. Ngenkathi ukuhlolwa kuthatha izingane eziningi nge-neuroblastoma yasencane, akubonakali ukuthi kunomthelela ekufeni kwesifo futhi akukhuthazwa njengamanje.
Isiteji se-Neuroblastoma
Njengamanye amaningi kansa, i-neuroblastoma ihlukaniswe phakathi kwezigaba I no-IV kuye ngokuthi izinga lokusabalala komdlavuza lilingani. Izigaba zifaka:
- Isigaba I - Esigabeni sesiteji i-tumor isendaweni yangakini, nakuba kungenzeka ukuthi isakaze ezithombeni eziseduze. Kulesi sigaba, lesi sifo singasuswa ngokugcwele ngesikhathi sokuhlinzwa.
- Isigaba II - I-tumor ivinjelwe endaweni lapho iqala khona futhi izitho zamakhansela eziseduze zingathinteka, kepha isisu asikwazi ukususwa ngokuphelele ngokuhlinzwa.
- Isigaba III - Isidumbu asikwazi ukususwa ngokuhlinzwa (singenakuphulukiswa). Kungenzeka ukuthi isakaze ezindaweni zamakhemikhali eziseduze noma ezifunda, kodwa hhayi kwezinye izifunda zomzimba.
- Isigaba IV - Isigaba IV sihlanganisa noma yisiphi isisu nganoma yisiphi isayizi noma ngaphandle kwama-lymph nodes esesakaze esitheni, emasheni, esibindi noma esikhumba.
- Isigaba IV-S - Ukuhlelwa okukhethekile kwesigaba IV-S kwenziwa ngezinhlayiya ezitholakala endaweni yangakini, kodwa naphezu kwalokhu kusakaze emnothweni wefu, isikhumba noma isibindi ezinganeni ezingaphansi kweminyaka eyodwa ubudala. Umnkantsha wamathambo ungabandakanyeka, kepha isisu sikhona ngamaphesenti angaphansi kuka-10 emnkantsheni wethambo.
Amaphesenti angama-60 kuya kwangu-80 wezingane atholakala lapho umdlavuza wesigaba IV.
I-International Neuroblastoma Group Risk Staging System (INRGSS)
I-International Neuroblastoma Group Risk Staging System yindlela enye i-neuroblastomas echazwa ngayo futhi inikeza ulwazi "engozini" yomdlavuza, ngamanye amazwi, ukuthi lesi sifo singelashwa kanjani.
Ukusebenzisa lesi simiso, izicubu zibhekwa njengengozi enkulu noma ingozi ephansi, okusiza ukuqondisa ukwelashwa.
Izimbangela Nezici Zengozi Ye-Neuroblastoma
Iningi lezingane ezine-neuroblastoma azikho umlando womndeni wesifo. Lokho kusho ukuthi ukuguqulwa kwezakhi zofuzo kucatshangwa ukuthi kubhekene namaphesenti angu-10 ama-neuroblastomas.
Izinguquko ku-ALK (i-anticlastic lymphoma kinase) zakhi zofuzo ziyimbangela enkulu ye-neuroblastoma yomndeni. Ukuguqulwa kwamagciwane ku-PHOX2B kukhonjisiwe ku-subset ye-neuroblastoma yomndeni.
Ezinye izinto ezingase zibe yingozi ezifakiwe zibandakanya ukubhema komzali, ukusetshenziswa kotshwala, imithi ethile ngesikhathi sokukhulelwa nokuvezwa kwamakhemikhali athile, kodwa ngalesi sikhathi akuqiniseki ukuthi laba badlala indima noma cha.
Ukwelashwa kwe-Neuroblastoma
Kunezinketho eziningi zokwelapha ze-neuroblastoma. Ukuzikhethela kwalokhu kuyoxhomekeka ukuthi ngabe ukuhlinzwa kungenzeka nezinye izici. Kungakhathaliseki ukuthi i-tumor "ingozi enkulu" ngokusho kwe-INCRSS iphinde iqhaza ekukhetheni ukwelashwa, futhi izinqumo zivame ukwenziwa ngokusekelwe ukuthi ingabe isisu sinengozi enkulu noma ingozi ephansi. Izinketho zihlanganisa:
- Ukuhlinzwa - Ngaphandle kokuba i-neuroblastoma isakazeke (isigaba IV), ukuhlinzeka kuvame ukukhipha isisu ngangokunokwenzeka. Uma ukuhlinzwa kungakwazi ukususa sonke isisu, ukwelashwa okwengeziwe nge-chemotherapy kanye nemisebe kuvame ukuphakanyiswa.
- I-Chemotherapy - I- Chemotherapy ihilela ukusetshenziswa kwezidakamizwa ezibulala amaseli ahlukanisa ngokushesha. Njengalokhu, kungabangela imiphumela emibi ngenxa yemiphumela yawo kumaseli ajwayelekile emzimbeni ohlukanisa ngokushesha njengamanye ematheni, izinwele zezinwele, kanye nomgudu wokugaya. Imithi ye-chemotherapy evame ukusetshenziswa ekuhlinzekeni i-neuroblastoma ihlanganisa i-Cytoxan (cyclophosphamide), i-Adriamycin (doxorubicin), i-Platinol (i-cisplatin), ne-etoposide. Izidakamizwa ezengeziwe zivame ukusetshenziselwa uma lesi sifo kubhekwa njengengozi enkulu.
- Ukufakelwa kwe-stem cell - Ukwelashwa kwe-high-dose chemotherapy kanye nokwelashwa kwemisebe okulandelwa yi- stem cell transplant iyinye indlela yokwelapha. Lezi zingxenyana zingaba yi-autologous (isebenzisa amaseli wengane eyayihoxiswa ngaphambi kwe-chemotherapy) noma i-allogeneic (isebenzisa amaseli avela kumnikeli njengomzali noma umhlinzeki ongathintani).
- Ezinye zokwelashwa - Ukuze izifo zibuyele emuva, ezinye izigaba zokwelashwa ezingacatshangelwa zihlanganisa ukwelashwa kwe-retinoid, i- immunotherapy , kanye nokwelashwa okuhlosiwe nge-ALK inhibitors nezinye izifo, njengokusebenzisa amakhemikhali athathwa ngamaseli we-neuroblastoma ahlanganiswe nezinhlayiya ze-radioactive.
Ukukhululwa okungajwayelekile kwe-Neuroblastoma
Isenzakalo esibizwa ngokuthi ukuxoxwa okuzenzekelayo kungenzeka, ikakhulukazi kulabo bantwana abanezinambuzane ezingaphansi kuka-5 cm (2½ amasentimitha ubukhulu), basesiteji I noma isigaba II, futhi bangaphansi kweminyaka engaphansi kweminyaka.
Ukuxoshwa okungajwayelekile lapho kwenzeka ukuthi izidumbu "zinyamalale" zodwa ngaphandle kokwelashwa. Ngenkathi lesi simo singavamile nakwezinye izinhlobo eziningi ze-kansa, akuyona into engavamile nge-neuroblastoma, mhlawumbe ngamathumba okuqala noma ama-metastases. Asiqiniseki ukuthi yini eyenza ezinye zezicubu zisuke nje, kodwa kungenzeka ukuthi zihlobene nesistimu yomzimba ngandlela-thile.
Ukubikezelwa kwe-Neuroblastoma
Ukuchazwa kwe-neuroblastoma kungahlukahluka kakhulu phakathi kwezingane ezahlukene. Ubudala bokuxilongwa yiyona namba eyodwa yezinto ezithinta ukubikezela. Izingane ezitholwa ngaphambi kweminyaka yobudala zinezibikezelo ezinhle kakhulu, ngisho nezigaba eziphambili ze-neuroblastoma.
Izinto ezihlobene nokuchazwa kwe-neuroblastoma zihlanganisa:
- Ukuguga lapho kutholakala khona
- Isiteji sesifo
- Ukutholakala kwezakhi zofuzo ezifana ne-ploidy nokukhulisa
- Inkulumo nge-tumor yamaprotheni athile
Ukuthola Ukusekela
Omunye usho njalo ukuthi into ebi kakhulu kunokuba nomdlavuza ngokwakho ukuthi ingane yakho ibhekane nomdlavuza, futhi kukhona iqiniso elikhulu kulowo mazwi. Njengabazali, sifuna ukuphulukisa izingane zethu ubuhlungu. Ngenhlanhla, izidingo zabazali bezingane ezinomdlavuza ziye zathola ukunakwa okuningi eminyakeni yamuva. Kunezinhlangano eziningi ezenzelwe ukusekela abazali abanabantwana abanomdlavuza, futhi kunabantu abaningi kumuntu kanye namaqembu asekela inthanethi nemiphakathi. Lawa maqembu akuvumela ukuthi ukhulume nabanye abazali ababhekene nezinselele ezifanayo futhi uthole ukwesekwa okuvela kokwazi ukuthi awodwa. Thatha isikhashana ukuhlola ezinye zezinhlangano ezisekela abazali abanomntwana onomdlavuza.
Kubalulekile ukukhuluma ngezingane zakithi kanye nabantwana ababhekana nezinkinga zokuba nomntwana onomdlavuza ngenkathi beba nesikhathi esincane kakhulu nabazali babo. Kukhona izinhlangano zokusekela ngisho namakamu aklanyelwe ukuhlangabezana nezidingo zezingane ezibhekene nalokho okungenzeka abangani babo abaningi abangakwazi ukukuqonda. I-CancerCare inezinsiza zokusiza izingane zakubo ngengane yomdlavuza. I-SuperSibs inikezelwe ezinganeni eziduduzayo nezinika amandla abanazo izingane ezinomdlavuza futhi inezinhlelo eziningana ezihlukene zokuhlangabezana nezidingo zalezi zingane. Ungase ufise ukuhlola amakamu nokubuyela emindenini kanye nezingane ezithinteka ngumdlavuza.
Izwi elivela
I-neuroblastoma yilona uhlobo oluvame kakhulu lomdlavuza ezinganeni ngesikhathi sonyaka wokuqala wokuphila kodwa kungavamile kamuva ebuntwaneni noma ekukhuleni. Izimpawu zivame ukufaka ukuthola isisindo esiswini, noma izimpawu ezinjenge-"blueberry muffin".
Izinketho eziningi zokwelashwa ziyatholakala kokubili ze-neuroblastomas ezitholakale zifunyenwe noma lezo eziphindaphindiwe. I-prognosis ixhomeke ezintweni eziningi, kodwa ukusinda kunesifo esiphakeme lapho lesi sifo sitholakala ngonyaka wokuqala wokuphila, ngisho noma sesakazeke kabanzi. Eqinisweni, ezinye ze-neuroblastomas, ikakhulukazi ezisezinsana ezisencane, ziyanyamalala ngokuzenzekelayo ngaphandle kokwelashwa.
Ngisho nalapho ukuchazwa kahle kuhle, i-neuroblastoma iyinkimbinkimbi ehlukumezayo kubazali, okungenzeka ukuthi bazonakekelwa kakhulu kunokuthi izingane zabo zibhekane nomdlavuza. Ngenhlanhla, kuye kwaba nokucwaninga okukhulu nokuthuthuka ekwelapheni ukwelashwa kwengane eminyakeni yamuva, kanti izindlela ezintsha zokwelapha zenziwa njalo ngonyaka.
> Imithombo:
> Berlanga, P., Canete, A., no-V. Castel. Ukuthuthukiswa kwezidakamizwa eziphuthumayo zokwelashwa kwe-neuroblastoma. Umbono wezobuchwepheshe ngezidakamizwa ezikhulayo . 2017. 22 (1): 63-75.
> Esposito, M., Aveic, S., Seydel, A., noG. Tonini. I-Neuroblastoma Ukwelashwa Era Post-Genomic. Journal of Science Biomedical . 2017. 24 (1): 14.
> National Cancer Institute. Ukwelashwa kwe-Neuroblastoma (PDQ) - I-Health Professional Version. Kubuyekezwe 06/21/17. https://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq