Izinhlobo / pheochromocytoma / isiguli / i-pheochromocytoma-treatment-pdq

IPheochromocytoma neParaganglioma Treatment (®) –Patient Version

Imininingwane ejwayelekile Mayelana nePheochromocytoma neParaganglioma

AMAPHUZU ASEMQOKA

• I-Pheochromocytoma ne-paraganglioma yizicubu ezingavamile ezivela kuhlobo olufanayo lwezicubu.

• I-Pheochromocytoma yisimila esingavamile esakha i-adrenal medulla (maphakathi nendlala ye-adrenal).

• Ama-Paragangliomas akha ngaphandle kwendlala ye-adrenal.

• Ukuphazamiseka okuthile okuzuzwe njengefa kanye nezinguquko kuzakhi ezithile zofuzo kwandisa ubungozi be-pheochromocytoma noma i-paraganglioma.

• Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma zifaka umfutho wegazi ophezulu nekhanda.

• Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma kungenzeka noma nini noma zilethwe yimicimbi ethile.

• Ukuhlolwa okuhlola igazi nomchamo kusetshenziselwa ukuthola (ukuthola) nokuxilonga i-pheochromocytoma ne-paraganglioma.

• Ukwelulekwa ngofuzo kuyingxenye yohlelo lokwelashwa kweziguli ezine-pheochromocytoma noma i-paraganglioma.

• Izici ezithile zithinta ukubikezela (ithuba lokululama) kanye nezinketho zokwelashwa.

I-Pheochromocytoma ne-paraganglioma yizicubu ezingavamile ezivela kuhlobo olufanayo lwezicubu.

AmaParagangliomas akha izicubu zezinzwa kwizindlala ze-adrenal futhi eduze kwemithambo ethile yegazi nezinzwa. Ama-paragangliomas akha kwizindlala ze-adrenal abizwa ngokuthi ama-pheochromocytomas. Ama-paragangliomas akha ngaphandle kwezindlala ze-adrenal abizwa ngama-extra-adrenal paragangliomas. Kulesi sifinyezo, ama-paragangliomas angaphezulu kwe-adrenal abizwa ngokuthi ama-paragangliomas.

Ama-Pheochromocytomas nama-paragangliomas angaba nobungozi (hhayi umdlavuza) noma abulale (umdlavuza).

I-Pheochromocytoma yisimila esingavamile esakha i-adrenal medulla (maphakathi nendlala ye-adrenal).

Amafomu e-Pheochromocytoma kwizindlala ze-adrenal. Kunezindlala ezimbili ze-adrenal, eyodwa ngaphezulu kwesinso ngasinye ngemuva kwesisu esingenhla. Indlala ngayinye ye-adrenal inezingxenye ezimbili. Isendlalelo sangaphandle sendlala ye-adrenal yi-adrenal cortex. Isikhungo sendlala ye-adrenal yi-adrenal medulla.

I-Pheochromocytoma yisilonda esingajwayelekile se-adrenal medulla. Imvamisa, i-pheochromocytoma ithinta indlala eyodwa ye-adrenal, kepha ingathinta zombili izindlala ze-adrenal. Kwesinye isikhathi kuba nesimila esingaphezu kwesisodwa ku-adrenal gland eyodwa.

Izindlala ze-adrenal zenza ama-hormone abalulekile abizwa ngama-catecholamines. I-Adrenaline (epinephrine) ne-noradrenaline (norepinephrine) yizinhlobo ezimbili zama-catecholamine ezisiza ukulawula ukushaya kwenhliziyo, umfutho wegazi, ushukela wegazi, kanye nendlela umzimba ophendula ngayo ekucindezelekeni. Kwesinye isikhathi i-pheochromocytoma izokhipha i-adrenaline eyengeziwe ne-noradrenaline egazini bese ibangela izimpawu noma izimpawu zesifo.

I-anatomy ye-adrenal gland. Kunezindlala ezimbili ze-adrenal, eyodwa ngaphezulu kwesinso ngasinye. Ingxenye engaphandle yendlala ngayinye yi-adrenal cortex; ingxenye yangaphakathi yi-adrenal medulla.

Ama-Paragangliomas akha ngaphandle kwendlala ye-adrenal.

I-Paragangliomas yizicubu ezingavamile ezakha eduze nomthambo we-carotid, ezindleleni zemizwa ekhanda nasentanyeni, nakwezinye izingxenye zomzimba. Amanye ama-paragangliomas enza ama-catecholamines angeziwe okuthiwa i-adrenaline ne-noradrenaline. Ukudedelwa kwalawa ma-catecholamine engeziwe egazini kungadala izimpawu noma izimpawu zesifo.

I-Paraganglioma yekhanda nentamo. Isimila esingavamile esivame ukwakheka eduze nomthambo we-carotid. Ingase futhi yakhe ezindleleni zemizwa ekhanda nasentanyeni nakwezinye izingxenye zomzimba.

Ukuphazamiseka okuthile okuzuzwe njengefa kanye nezinguquko kuzakhi ezithile zofuzo kwandisa ubungozi be-pheochromocytoma noma i-paraganglioma.

Noma yini eyandisa amathuba akho okuthola isifo ibizwa ngokuthi ingozi. Ukuba nengcuphe akusho ukuthi uzothola umdlavuza; ukungabi nezici zobungozi akusho ukuthi ngeke uthole umdlavuza. Khuluma nodokotela wakho uma ucabanga ukuthi ungaba sengozini.

Ama-syndromes noma izinguquko zofuzo ezilandelayo zandisa ubungozi be-pheochromocytoma noma i-paraganglioma:

• I-endocrine neoplasia 2 syndrome, izinhlobo A no-B (MEN2A ne-MEN2B).

• I-von Hippel-Lindau (VHL) isifo.

• Uhlobo lwe-Neurofibromatosis 1 (NF1).

• Isizungu se-Hereditary paraganglioma.

• ICarney-Stratakis dyad (paraganglioma kanye nesisu somzimba esiswini [GIST]).

• ICarney triad (paraganglioma, GIST, kanye ne-chondroma yamaphaphu).

Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma zifaka umfutho wegazi ophezulu nekhanda.

Ezinye izicubu azenzi i-adrenaline noma i-noradrenaline eyengeziwe futhi azibangeli zimpawu nezimpawu. Lezi zicubu kwesinye isikhathi zitholakala lapho kutholakala isigaxa entanyeni noma lapho kwenziwa noma kwenziwa inqubo ngesinye isizathu. Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma zenzeka lapho i-adrenaline noma i-noradrenaline eningi kakhulu ikhishelwa egazini. Lezi kanye nezinye izimpawu nezimpawu zingabangelwa i-pheochromocytoma ne-paraganglioma noma ezinye izimo. Buza nodokotela wakho uma unokunye kwalokhu okulandelayo:

• Umfutho wegazi ophezulu.

• Ubuhlungu bekhanda.

• Ukujuluka okukhulu ngaphandle kwesizathu esaziwayo.

• Ukushaya kwenhliziyo okuqinile, okusheshayo, noma okungajwayelekile.

• Ukuthuthumela.

• Ukuba mhlophe ngokwedlulele.

Uphawu oluvame kakhulu umfutho wegazi ophakeme. Kungaba nzima ukulawula. Umfutho wegazi ophakeme kakhulu ungadala izinkinga ezinkulu zempilo njengokushaya kwenhliziyo okungajwayelekile, isifo senhliziyo, isifo sohlangothi noma ukufa.

Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma kungenzeka noma nini noma zilethwe yimicimbi ethile.

Izimpawu nezimpawu ze-pheochromocytoma ne-paraganglioma zingenzeka lapho kwenzeka enye yezinto ezilandelayo:

• Umsebenzi onzima womzimba.

• Ukulimala emzimbeni noma ukucindezeleka okuningi ngokomzwelo.

• Ukubeletha.

• Ukuya ngaphansi kwe-anesthesia.

• Ukuhlinzwa, kufaka phakathi izinqubo zokususa isimila.

• Ukudla ukudla okuphezulu nge-tyramine (okufana newayini elibomvu, ushokoledi noshizi).

Ukuhlolwa okuhlola igazi nomchamo kusetshenziselwa ukuthola (ukuthola) nokuxilonga i-pheochromocytoma ne-paraganglioma.

Lezi zivivinyo nezinqubo ezilandelayo zingasetshenziswa:

• Ukuhlolwa komzimba kanye nomlando: Ukuhlolwa komzimba ukuhlola izimpawu ezijwayelekile zempilo, kufaka phakathi nokuhlola izimpawu zezifo, ezifana nomfutho wegazi ophakeme noma yini enye ebonakala ingajwayelekile. Kuzothathwa nomlando wemikhuba yezempilo yesiguli kanye nokugula nokwelashwa kwangaphambilini.

• Ukuhlolwa komchamo wamahora angamashumi amabili nane: Isivivinyo lapho kuqoqwa khona umchamo amahora angama-24 ukukala amanani ama-catecholamines emchameni. Izilinganiso ezibangelwa ukuwohloka kwalawa ma-catecholamine nazo ziyalinganiswa. Inani elingajwayelekile (eliphakeme noma eliphansi kunokujwayelekile) lento kungaba uphawu lwesifo esithweni noma esikhunjeni esisenzayo. Inani eliphakeme kunokujwayelekile le-catecholamines ethile kungaba uphawu lwe-pheochromocytoma.

• Ucwaningo lwegazi catecholamine: Inqubo lapho kuhlolwe khona isampula segazi ukukala inani lama-catecholamine athile akhishwe egazini. Izilinganiso ezibangelwa ukuwohloka kwalawa ma-catecholamine nazo ziyalinganiswa. Inani elingajwayelekile (eliphakeme noma elingaphansi kokujwayelekile) lento kungaba uphawu lwesifo esithweni noma esikhunjeni esisenzayo. Inani eliphakeme kunokujwayelekile le-catecholamines ethile kungaba uphawu lwe-pheochromocytoma.

• I-CT scan (i-CAT scan): Inqubo eyenza uchungechunge lwezithombe eziningiliziwe zezindawo ezingaphakathi komzimba, ezifana nentamo, isifuba, isisu, nesikhumba, ezithathwe ezinhlangothini ezahlukene. Izithombe zenziwa yikhompyutha exhunywe kumshini we-x-ray. Idayi ingajovwa emthanjeni noma igwinywe ukusiza izitho noma izicubu zivele kahle. Le nqubo ibizwa nangokuthi i-computed tomography, i-computerized tomography, noma i-computer axial tomography.

• I-MRI (imaging resonance imaging): Inqubo esebenzisa uzibuthe, amagagasi omsakazo, nekhompyutha ukwenza uchungechunge lwezithombe eziningiliziwe zezindawo ezingaphakathi komzimba ezifana nentamo, isifuba, isisu kanye nesinqe. Le nqubo ibizwa nangokuthi i-nuclear magnetic resonance imaging (i-NMRI).

Ukwelulekwa ngofuzo kuyingxenye yohlelo lokwelashwa kweziguli ezine-pheochromocytoma noma i-paraganglioma.

Zonke iziguli ezitholwe zine-pheochromocytoma noma i-paraganglioma kufanele zithole ukwelulekwa ngezakhi zofuzo ukuthola ubungozi bazo bokuthola i-syndrome ezuzwe njengelifa neminye imidlavuza ehlobene nayo.

Ukuhlolwa kofuzo kunganconywa ngumeluleki wofuzo weziguli:

• Yiba nomlando womuntu siqu noma womndeni wezici ezixhunywe ne-pheochromocytoma noma i-paraganglioma syndrome.

• Yiba nezicubu kuzo zombili izindlala ze-adrenal.

• Yiba nesimila esingaphezu kwesisodwa ku-adrenal gland eyodwa.

• Yiba nezimpawu noma izimpawu zama-catecholamine athe xaxa akhishelwa egazini noma eparganglioma ebulalayo (enomdlavuza).

• Atholwa ngaphambi kweminyaka yobudala engama-40.

Ukuhlolwa kofuzo kwesinye isikhathi kunconywa iziguli ezine-pheochromocytoma:

• Baneminyaka engama-40 kuya kwengu-50 ubudala.

• Yiba nesimila kwesinye se-adrenal gland.

• Awunawo umlando womuntu siqu noma womndeni wesifo esizuzwe njengefa.

Lapho kutholakala izinguquko ezithile zofuzo ngesikhathi sokuhlolwa kofuzo, ukuhlolwa kuvame ukunikelwa kumalungu omndeni asengozini kodwa angenazo izimpawu noma izimpawu.

Ukuhlolwa kofuzo akunconyelwe iziguli ezindala kuneminyaka engama-50.

Izici ezithile zithinta ukubikezela (ithuba lokululama) kanye nezinketho zokwelashwa.

Isibikezelo (ithuba lokululama) nezinketho zokwelashwa zincike kokulandelayo:

• Noma ngabe isimila sinobungozi noma sibi.

• Ukuthi isimila sisendaweni eyodwa kuphela noma sisabalalele kwezinye izindawo emzimbeni.

• Ukuthi kukhona yini izimpawu noma izimpawu ezibangelwa inani elingaphezu kwelivamile lama-catecholamine.

• Ukuthi isimila sisanda kutholakala yini noma sesiphindile (buyela emuva).

Izigaba zePheochromocytoma neParaganglioma

AMAPHUZU ASEMQOKA

• Ngemuva kokutholakala kwe-pheochromocytoma ne-paraganglioma, kwenziwa ukuhlolwa ukuthola ukuthi isimila sesinabele yini kwezinye izingxenye zomzimba.

• Zintathu izindlela umdlavuza osakazeka ngayo emzimbeni.

• Umdlavuza ungasakazeka usuka lapho uqale khona kwezinye izingxenye zomzimba.

• Alukho uhlelo olujwayelekile lokubeka isiteji i-pheochromocytoma ne-paraganglioma.

• I-Pheochromocytoma ne-paraganglioma bachazwa njengabendawo, isifunda noma i-metastatic.

• I-pheochromocytoma yasendaweni ne-paraganglioma

• I-pheochromocytoma yesifunda ne-paraganglioma

• I-Metastatic pheochromocytoma ne-paraganglioma

Ngemuva kokutholakala kwe-pheochromocytoma ne-paraganglioma, kwenziwa ukuhlolwa ukuthola ukuthi isimila sesinabele yini kwezinye izingxenye zomzimba.

Izinga noma ukusabalala komdlavuza kuvame ukuchazwa njengesigaba. Kubalulekile ukwazi ukuthi umdlavuza ususakazekile yini ukuze uhlele ukwelashwa. Lezi zivivinyo nezinqubo ezilandelayo zingasetshenziswa ukuthola ukuthi isimila sesisabalalele yini kwezinye izingxenye zomzimba:

• I-CT scan (i-CAT scan): Inqubo eyenza uchungechunge lwezithombe eziningiliziwe zezindawo ezingaphakathi komzimba, ezifana nentamo, isifuba, isisu, nesikhumba, ezithathwe ezinhlangothini ezahlukene. Izithombe zenziwa yikhompyutha exhunywe kumshini we-x-ray. Idayi ingajovwa emthanjeni noma igwinywe ukusiza izitho noma izicubu zivele kahle. Isisu nesinqe kuthathwe isithombe ukuthola izimila ezikhipha i-catecholamine. Le nqubo ibizwa nangokuthi i-computed tomography, i-computerized tomography, noma i-computer axial tomography.

• I-MRI (imaging resonance imaging): Inqubo esebenzisa uzibuthe, amagagasi omsakazo, nekhompyutha ukwenza uchungechunge lwezithombe eziningiliziwe zezindawo ezingaphakathi komzimba ezifana nentamo, isifuba, isisu kanye nesinqe. Le nqubo ibizwa nangokuthi i-nuclear magnetic resonance imaging (i-NMRI).

• Ukuskena kwe-MIBG: Inqubo esetshenziselwa ukuthola izicubu ze-neuroendocrine, njenge-pheochromocytoma ne-paraganglioma. Inani elincane kakhulu lezinto ezibizwa nge-radioactive MIBG lifakwa emthanjeni futhi lihamba egazini. Amaseli we-Neuroendocrine tumor athatha i-MIBG enemisebe futhi atholwa isithwebuli. Ukuskena kungathathwa ngaphezu kwezinsuku eziyi-1-3. Isixazululo se-iodine singanikezwa ngaphambi noma ngesikhathi sokuhlolwa ukugcina indlala yegilo ingamunci iMIBG eningi kakhulu.

• ukuskena: Uhlobo lwe-radionuclide scan olusetshenziselwe ukuthola izicubu ezithile, kufaka phakathi izicubu ezikhipha i-catecholamine. Inani elincane kakhulu lemisebe (i-hormone enamathisela izicubu ezithile) lifakwa emthanjeni futhi lihambe phakathi kwegazi. I-radioactive inamathela ku-tumor nekhamera ekhethekile ethola ukusebenza kwe-radioactivity isetshenziselwa ukukhombisa ukuthi izicubu zikuphi emzimbeni.

• Ukuskena kwe-FDG-PET (ukuskena kwe-fluorodeoxyglucose-positron emission tomography): Inqubo yokuthola amangqamuzana amabi esigaxa emzimbeni. Inani elincane le-FDG, uhlobo lwe-glucose enemisebe (ushukela), lufakwa emthanjeni. Isithwebuli se-PET sizungeza umzimba futhi senze isithombe lapho i-glucose isetshenziswa khona emzimbeni. Amaseli amathumba amabi abonakala ekhanya esithombeni ngoba ayasebenza futhi athatha i-glucose eningi kunamaseli avamile.

Zintathu izindlela umdlavuza osakazeka ngayo emzimbeni.

Umdlavuza ungasakazeka ngezicubu, uhlelo lwe-lymph, kanye negazi:

• Izicubu. Umdlavuza usakazeka lapho uqale khona ngokukhulela ezindaweni eziseduze.

• Uhlelo lweLymph. Umdlavuza usakazeka lapho uqale khona ngokungena ohlelweni lwe-lymph. Umdlavuza udabula imithambo ye-lymph uye kwezinye izingxenye zomzimba.

• Igazi. Umdlavuza usakazeka lapho uqale khona ngokungena egazini. Umdlavuza uhamba ngemithambo yegazi uye kwezinye izingxenye zomzimba.

Umdlavuza ungasakazeka usuka lapho uqale khona kwezinye izingxenye zomzimba.

Lapho umdlavuza usakazekela kolunye uhlangothi lomzimba, ubizwa ngokuthi yi-metastasis. Amaseli womdlavuza aqhamuka lapho aqale khona (isimila sokuqala) futhi ahamba ngohlelo lwe-lymph noma igazi.

• Uhlelo lweLymph. Umdlavuza ungena ohlelweni lwe-lymph, udabule emithanjeni ye-lymph, bese wenza isimila (isimila se-metastatic) kwenye ingxenye yomzimba.

• Igazi. Umdlavuza ungena egazini, udabule emithanjeni yegazi, bese wenza isimila (isimila se-metastatic) kwenye ingxenye yomzimba.

Isigaxa se-metastatic luhlobo olufanayo lomdlavuza njengesimila sokuqala. Isibonelo, uma i-pheochromocytoma isakazeka ithambo, amangqamuzana omdlavuza asethanjeni angamaseli e-pheochromocytoma empeleni. Lesi sifo yi-pheochromocytoma ye-metastatic, hhayi umdlavuza wamathambo.

Alukho uhlelo olujwayelekile lokubeka isiteji i-pheochromocytoma ne-paraganglioma.

I-Pheochromocytoma ne-paraganglioma bachazwa njengabendawo, isifunda noma i-metastatic.

I-pheochromocytoma yasendaweni ne-paraganglioma

Isimila sitholakala koyedwa noma zombili izindlala ze-adrenal (pheochromocytoma) noma endaweni eyodwa kuphela (paraganglioma).

I-pheochromocytoma yesifunda ne-paraganglioma

Umdlavuza usabalalele kuma-lymph node noma ezinye izicubu eziseduze nalapho kuqala khona isimila.

I-Metastatic pheochromocytoma ne-paraganglioma

Umdlavuza usabalalele kwezinye izingxenye zomzimba, njengesibindi, amaphaphu, ithambo, noma ama-lymph node akude.

I-Pheochromocytoma ephindaphindiwe neParaganglioma

I-pheochromocytoma ephindaphindekayo noma i-paraganglioma wumdlavuza ophindile (wabuya) ngemuva kokuba welashwe. Umdlavuza ungabuya endaweni efanayo noma kwenye ingxenye yomzimba.

Uhlolojikelele lwenketho yokwelashwa

AMAPHUZU ASEMQOKA

• Kunezinhlobo ezahlukahlukene zokwelashwa kweziguli ezine-pheochromocytoma noma i-paraganglioma.

• Iziguli ezine-pheochromocytoma ne-paraganglioma ezidala izimpawu noma izimpawu ziphathwa ngemithi yokwelapha.

• Izinhlobo eziyisithupha zokwelashwa okujwayelekile zisetshenzisiwe:

• Ukuhlinzwa

• Ukwelashwa ngemisebe

• Ukwelashwa ngamakhemikhali

• Ukwelashwa kwe-Ablation

• Ukwelashwa kokuhlanganisa

• Ukwelashwa okuhlosiwe

• Izinhlobo ezintsha zokwelashwa ziyahlolwa ezivivinyweni zokwelashwa.

• Ukwelashwa kwe-pheochromocytoma ne-paraganglioma kungadala imiphumela emibi.

• Iziguli zingafuna ukucabanga ngokubamba iqhaza esivivinyweni somtholampilo.

• Iziguli zingangena ezivivinyweni zokwelashwa ngaphambi, ngesikhathi, noma ngemuva kokuqala ukwelashwa kwazo komdlavuza.

• Ukuhlolwa kokulandelela kuzodingeka.

Kunezinhlobo ezahlukahlukene zokwelashwa kweziguli ezine-pheochromocytoma noma i-paraganglioma.

Izinhlobo ezahlukene zokwelashwa ziyatholakala ezigulini ezine-pheochromocytoma noma i-paraganglioma. Ezinye izindlela zokwelapha zijwayelekile (indlela esetshenziswa njengamanje), kanti ezinye ziyahlolwa ezivivinyweni zokwelashwa. Isivivinyo somtholampilo wokwelashwa isifundo socwaningo esihloselwe ukusiza ukuthuthukisa ukwelashwa kwamanje noma ukuthola ulwazi ngezindlela zokwelashwa ezintsha zeziguli ezinomdlavuza. Lapho izivivinyo zokwelashwa zibonisa ukuthi ukwelashwa okusha kungcono kunokwelashwa okujwayelekile, ukwelashwa okusha kungaba ukwelashwa okujwayelekile. Iziguli zingafuna ukucabanga ngokubamba iqhaza esivivinyweni somtholampilo. Ezinye izivivinyo zokwelashwa zivulekele kuphela iziguli ezingakaqali ukwelashwa

Iziguli ezine-pheochromocytoma ne-paraganglioma ezidala izimpawu noma izimpawu ziphathwa ngemithi yokwelapha.

Ukwelashwa kwezidakamizwa kuqala lapho kutholakala i-pheochromocytoma noma i-paraganglioma. Lokhu kungafaka:

• Izidakamizwa ezigcina umfutho wegazi uvamile. Isibonelo, uhlobo olulodwa lomuthi olubizwa nge-alpha-blockers lumisa i-noradrenaline ekwenzeni imithambo yegazi emincane ibe mincane. Ukugcina imithambo yegazi ivulekile futhi ikhululekile kuthuthukisa ukugeleza kwegazi futhi kwehlise umfutho wegazi.

• Izidakamizwa ezigcina isilinganiso senhliziyo sijwayelekile. Isibonelo, uhlobo olulodwa lomuthi olubizwa nge-beta-blockers lumisa ukusebenza kwe-noradrenaline eningi futhi lwehlise izinga lokushaya kwenhliziyo.

• Izidakamizwa ezivimba umphumela wamahomoni angeziwe enziwa yindlala ye-adrenal.

Imithi yokwelashwa ivame ukunikezwa isonto elilodwa kuya kwamathathu ngaphambi kokuhlinzwa.

Izinhlobo eziyisithupha zokwelashwa okujwayelekile zisetshenzisiwe:

Ukuhlinzwa

Ukuhlinzwa ukususa i-pheochromocytoma kuvame ukuba yi-adrenalectomy (ukususwa kwendlala eyodwa noma zombili ze-adrenal). Ngesikhathi sokuhlinzwa, izicubu nama-lymph node ngaphakathi kwesisu kuzohlolwa futhi uma isimila sesisakazekile, lezi zicubu nazo zingasuswa. Izidakamizwa zinganikezwa ngaphambi, phakathi nangemva kokuhlinzwa ukugcina umfutho wegazi nokushaya kwenhliziyo kujwayelekile.

Ngemuva kokuhlinzwa ukuze kususwe isimila, kuhlolwa amazinga e-catecholamine egazini noma emchameni. Amazinga ajwayelekile e-catecholamine awuphawu lokuthi wonke amaseli e-pheochromocytoma asusiwe.

Uma zombili izindlala ze-adrenal zisuswa, ukwelashwa kwama-hormone okuphila impilo yonke ukufaka ama-hormone akhiqizwe yizindlala ze-adrenal kuyadingeka.

Ukwelashwa ngemisebe

Ukwelashwa ngemisebe ukwelashwa komdlavuza okusebenzisa amandla amakhulu ama-x-ray noma ezinye izinhlobo zemisebe ukubulala amangqamuzana omdlavuza noma ukuwagcina ekukhuleni. Kunezinhlobo ezimbili zokwelashwa ngemisebe:

• Ukwelashwa kwemisebe kwangaphandle kusebenzisa umshini ongaphandle komzimba ukuthumela imisebe ebheke emdlavuzeni.

• Ukwelashwa kwemisebe kwangaphakathi kusebenzisa into enemisebe eboshwe ngezinaliti, izimbewu, izintambo, noma ama-catheters abekwe ngqo noma eduze komdlavuza.

Indlela yokwelashwa ngemisebe enikezwa ngayo incike ohlotsheni lomdlavuza owelashwayo nokuthi ngabe usendaweni yakhona, uyisifunda, uyisifo semithi, noma uyenzeka kabusha. Ukwelashwa kwemisebe yangaphandle kanye nokwelashwa kwe-131I-MIBG kusetshenziselwa ukwelapha i-pheochromocytoma.

I-Pheochromocytoma kwesinye isikhathi ilashwa nge-131I-MIBG, ethwala imisebe ngqo kumaseli wesimila. I-131I-MIBG yinto enemisebe eqoqa ezinhlotsheni ezithile zamangqamuzana ezimila, ibabulale ngemisebe enikezwayo. I-131I-MIBG inikezwa ngokumnika. Akuwona wonke ama-pheochromocytomas athatha i-131I-MIBG, ngakho-ke ukuhlolwa kwenziwa kuqala ukubheka lokhu ngaphambi kokwelashwa.

Ukwelashwa ngamakhemikhali

I-Chemotherapy yindlela yokwelapha umdlavuza esebenzisa izidakamizwa ukuvimba ukukhula kwamangqamuzana omdlavuza, kungaba ngokubulala amaseli noma ngokuwavimba ekuhlukaniseni. Lapho i-chemotherapy ithathwa ngomlomo noma ifakwa emthanjeni noma emisipheni, izidakamizwa zingena egazini futhi zingafinyelela kumaseli womdlavuza emzimbeni wonke (systemic chemotherapy). Lapho i-chemotherapy ifakwa ngqo ku-cerebrospinal fluid, isitho, noma umgodi womzimba njengesisu, izidakamizwa zithinta kakhulu amangqamuzana omdlavuza kulezo zindawo (i-chemotherapy yesifunda). Inhlanganisela yamakhemikhali ukwelashwa kusetshenziswa izidakamizwa ezingaphezu kwesisodwa ze-anticancer. Indlela i-chemotherapy enikezwa ngayo ixhomeke ohlotsheni lomdlavuza owelashwayo nokuthi ngabe usendaweni yakhona, uyisifunda, uyisifo semithi, noma uyenzeka kabusha.

Ukwelashwa kwe-Ablation

I-Ablation yimithi yokususa noma yokucekela phansi isitho somzimba noma isicubu noma umsebenzi waso. Izindlela zokwelapha ze-Ablation ezisetshenziselwa ukubulala amangqamuzana omdlavuza zifaka:

• I-Radiofrequency ablation: Inqubo esebenzisa amagagasi omsakazo ukushisa nokubhubhisa amaseli angajwayelekile. Amagagasi omsakazo ahamba ngama-electrode (amadivayisi amancane athwala ugesi). Ukukhishwa kwe-Radiofrequency kungasetshenziswa ukwelapha umdlavuza nezinye izimo.

• I-Cryoablation: Inqubo lapho izicubu zifriziwe ukubhubhisa amaseli angajwayelekile. I-nitrogen ewuketshezi noma i-liquid carbon dioxide isetshenziselwa ukuqandisa izicubu.

Ukwelashwa kokuhlanganisa

Ukwelashwa kokuhlanganisa kuyindlela yokwelapha ukuvimba umthambo oholela ku-adrenal gland. Ukuvimbela ukugeleza kwegazi kuya kwizindlala ze-adrenal kusiza ukubulala amangqamuzana omdlavuza akhula lapho.

Ukwelashwa okuhlosiwe

Ukwelashwa okuhlosiwe ukwelashwa okusebenzisa izidakamizwa noma ezinye izinto ukukhomba nokuhlasela amangqamuzana omdlavuza athile ngaphandle kokulimaza amaseli ajwayelekile. Izindlela zokwelashwa ezihlosiwe zisetshenziselwa ukwelapha i-pheochromocytoma ye-metastatic and recurrent.

ISunitinib (uhlobo lwe-tyrosine kinase inhibitor) ukwelashwa okusha okufundelwa i-pheochromocytoma yemetastatic. Ukwelashwa kweTyrosine kinase inhibitor luhlobo lwezokwelapha ezihlosiwe ezivimba amasiginali adingekayo ukuze izicubu zikhule.

Izinhlobo ezintsha zokwelashwa ziyahlolwa ezivivinyweni zokwelashwa.

Imininingwane mayelana nokuhlolwa kwemitholampilo iyatholakala kuwebhusayithi ye-NCI.

Ukwelashwa kwe-pheochromocytoma ne-paraganglioma kungadala imiphumela emibi.

Ngemininingwane ngemiphumela engemihle ebangelwe ukwelashwa komdlavuza, bheka ikhasi lethu le-Side Effects.

Iziguli zingafuna ukucabanga ngokubamba iqhaza esivivinyweni somtholampilo.

Kwezinye iziguli, ukubamba iqhaza esivivinyweni somtholampilo kungaba yindlela engcono kakhulu yokwelashwa. Ukuhlolwa kwemitholampilo kuyingxenye yenqubo yocwaningo lomdlavuza. Ukuhlolwa komtholampilo kuyenziwa ukuthola ukuthi ngabe ukwelashwa komdlavuza okusha kuphephile futhi kuyasebenza yini noma kungcono kunokwelashwa okujwayelekile.

Eziningi zezindlela zokwelashwa ezijwayelekile zomdlavuza zisuselwa ekuhlolweni kokuqala komtholampilo. Iziguli ezibamba iqhaza esivivinyweni somtholampilo zingathola ukwelashwa okujwayelekile noma zibe phakathi kwabokuqala ukuthola ukwelashwa okusha.

Iziguli ezibamba iqhaza ezivivinyweni zokwelashwa zisiza nokwenza ngcono indlela umdlavuza ozophathwa ngayo ngokuzayo. Noma izilingo zomtholampilo zingaholeli ekwelashweni okusha okusebenzayo, zivame ukuphendula imibuzo ebalulekile futhi zisize ukuqhubela phambili ucwaningo.

Iziguli zingangena ezivivinyweni zokwelashwa ngaphambi, ngesikhathi, noma ngemuva kokuqala ukwelashwa kwazo komdlavuza.

Ezinye izivivinyo zokwelashwa zifaka kuphela iziguli ezingakatholi ukwelashwa. Ezinye izilingo zivivinya ukwelashwa kweziguli ezinomdlavuza ongakabi ngcono. Kukhona nokuhlolwa komtholampilo okuhlola izindlela ezintsha zokunqanda umdlavuza ukuthi ungaphindi (ubuye) noma kunciphise imiphumela emibi yokwelashwa komdlavuza.

Ukuhlolwa kwemitholampilo kuyenzeka ezingxenyeni eziningi zezwe. Imininingwane mayelana nokuhlolwa kwemitholampilo esekelwa yi-NCI ingatholakala ekhasini lewebhu le-NCI lokuhlolwa kokuhlolwa kwemitholampilo. Izivivinyo zomtholampilo ezisekelwa ezinye izinhlangano zingatholakala kuwebhusayithi yeClinicalTrials.gov.

Ukuhlolwa kokulandelela kuzodingeka.

Olunye uvivinyo olwenziwe ukuthola umdlavuza noma ukuthola ukuthi umdlavuza ungakanani lungaphindwa. Olunye uvivinyo luzophindwa ukuze kubonakale ukuthi umuthi usebenza kanjani. Izinqumo mayelana nokuqhubeka, ukushintsha, noma ukuyeka ukwelashwa zizosuselwa emiphumeleni yalezi zivivinyo.

Ezinye zezivivinyo zizoqhubeka ukwenziwa ngezikhathi ezithile ngemuva kokuphela kokwelashwa. Imiphumela yalezi zivivinyo ingakhombisa ukuthi ngabe isimo sakho sishintshile noma uma umdlavuza ubuyile (buyela emuva). Lezi zivivinyo kwesinye isikhathi zibizwa ngokuthi izivivinyo zokulandelela.

Ezigulini ezine-pheochromocytoma noma i-paraganglioma edala izimpawu, amazinga e-catecholamine egazini nasemchameni azohlolwa njalo. Amazinga e-Catecholamine aphakeme kakhulu kunokujwayelekile angaba wuphawu lokuthi umdlavuza ubuyile.

Ezigulini ezine-paraganglioma ezingabangeli zimpawu, izivivinyo zokulandelela ezifana ne-CT, MRI, noma i-MIBG scan kufanele zenziwe minyaka yonke.

Ezigulini ezine-pheochromocytoma ezuzwe njengefa, amazinga e-catecholamine egazini nasemchameni azohlolwa njalo. Olunye uvivinyo lokuhlola luzokwenziwa ukubheka ezinye izicubu ezixhunyaniswe nesifo esizuzwe njengefa.

Khuluma nodokotela wakho mayelana nokuthi yikuphi ukuhlolwa okufanele kwenziwe nokuthi kwenziwa kangaki. Iziguli ezine-pheochromocytoma noma i-paraganglioma zidinga ukulandelwa impilo yonke.

Izinketho Zokwelashwa zePheochromocytoma neParaganglioma

Kulesi Sigaba

• I-Pheochromocytoma ne-Paraganglioma eyenziwe yasendaweni

• I-Pheochromocytoma ezuzwe njengefa

• IsiFunda iPheochromocytoma neParaganglioma

• I-Metastatic Pheochromocytoma neParaganglioma

• I-Pheochromocytoma ephindaphindiwe neParaganglioma

Ngemininingwane mayelana nokwelashwa okubalwe ngezansi, bheka isigaba se-Treatment Option Overview section.

I-Pheochromocytoma ne-Paraganglioma eyenziwe yasendaweni

Ukwelashwa kwe-pheochromocytoma noma i-paraganglioma enezindawo ezivame ukwelashwa kuvame ukuhlinzwa ukuze kususwe ngokuphelele isimila. Uma isimila sisendlala ye-adrenal, kukhishwa yonke indlala ye-adrenal.

Sebenzisa ukusesha kwethu kokuhlolwa komtholampilo ukuthola izivivinyo zomtholampilo ezisekelwa yi-NCI ezamukela iziguli. Ungasesha izivivinyo ngokususelwa ohlotsheni lomdlavuza, iminyaka yesiguli, nalapho kwenziwa khona izivivinyo. Imininingwane ejwayelekile mayelana nokuhlolwa kwemitholampilo iyatholakala.

I-Pheochromocytoma ezuzwe njengefa

Ezigulini ezine-pheochromocytoma ezuzwe njengefa ezixhunyaniswe ne-endocrine neoplasia (MEN2) noma i-von Hippel-Lindau (VHL) syndrome, izicubu zivame ukwakheka kuzo zombili izindlala ze-adrenal. Izimila zivame ukuba nobungozi.

• Ukwelashwa kwe-pheochromocytoma ezuzwe njengefa okwenzeka ku-adrenal gland eyodwa ukuhlinzwa ukuze kususwe ngokuphelele indlala. Lokhu kuhlinzwa kungasiza iziguli ukuthi zigweme ukwelashwa okufaka esikhundleni se-steroid i-steroid impilo yonke nokungasebenzi kahle kwe-adrenal.

• Ukwelashwa kwe-pheochromocytoma ezuzwe njengefa okwakhiwa kuzo zombili izindlala ze-adrenal noma amafomu akamuva kule ndlala esele ye-adrenal kungaba ukuhlinzwa ukuze kususwe isimila kanye nezicubu ezincane ezijwayelekile ku-adrenal cortex ngangokunokwenzeka. Lokhu kuhlinzwa kungasiza iziguli ukuthi zigweme ukwelashwa okubuyisela ama-hormone okuhlala isikhathi eside nezinkinga zempilo ngenxa yokulahleka kwamahomoni enziwa yindlala ye-adrenal.

IsiFunda iPheochromocytoma neParaganglioma

Ukwelashwa kwe-pheochromocytoma noma i-paraganglioma esabalele ezithweni eziseduze noma kuma-lymph node ukuhlinzwa ukuze kususwe ngokuphelele isimila. Izitho eziseduze umdlavuza osabalalele kuzo, njengezinso, isibindi, ingxenye yomthambo omkhulu wegazi, nama-lymph node, nazo zingasuswa.

Sebenzisa ukusesha kwethu kokuhlolwa komtholampilo ukuthola izivivinyo zomtholampilo ezisekelwa yi-NCI ezamukela iziguli. Ungasesha izivivinyo ngokususelwa ohlotsheni lomdlavuza, iminyaka yesiguli, nalapho kwenziwa khona izivivinyo. Imininingwane ejwayelekile mayelana nokuhlolwa kwemitholampilo iyatholakala.

I-Metastatic Pheochromocytoma neParaganglioma

Ukwelashwa kwe-metastatic pheochromocytoma noma i-paraganglioma kungafaka okulandelayo:

• Ukuhlinzwa ukukhipha ngokuphelele umdlavuza, kufaka phakathi izicubu ezisabalalele ezingxenyeni ezikude zomzimba.

• Ukwelashwa kwe-palliative, ukunciphisa izimpawu nokuthuthukisa ikhwalithi yempilo, kufaka phakathi:

• Ukuhlinzwa ukususa umdlavuza omningi ngangokunokwenzeka.

• Inhlanganisela chemotherapy.

• Ukwelashwa ngemisebe nge-131I-MIBG.

• Ukwelashwa kwemisebe yangaphandle ezindaweni (ezinjengethambo) lapho umdlavuza usabalale futhi ungeke ususwe ngokuhlinzwa.

• Ukuhlanganiswa (ukwelashwa ukuvimba umthambo onikeza igazi ku-tumor).

• Ukwelashwa kwe-Ablation usebenzisa i-radiofrequency ablation noma i-cryoablation yamathumba esibindi noma ethanjeni.

• Isivivinyo somtholampilo sokwelapha okuqondisiwe nge-tyrosine kinase inhibitor.

• Isivivinyo somtholampilo sokwelapha ngemisebe yangaphakathi kusetshenziswa into entsha enemisebe.

Sebenzisa ukusesha kwethu kokuhlolwa komtholampilo ukuthola izivivinyo zomtholampilo ezisekelwa yi-NCI ezamukela iziguli. Ungasesha izivivinyo ngokususelwa ohlotsheni lomdlavuza, iminyaka yesiguli, nalapho kwenziwa khona izivivinyo. Imininingwane ejwayelekile mayelana nokuhlolwa kwemitholampilo iyatholakala.

I-Pheochromocytoma ephindaphindiwe neParaganglioma

Ukwelashwa kwe-pheochromocytoma ephindaphindwayo noma i-paraganglioma kungafaka okulandelayo:

• Ukuhlinzwa ukususa ngokuphelele umdlavuza.

• Lapho ukuhlinzwa ukukhipha umdlavuza kungenzeki, ukwelashwa okunciphisayo ukukhulula izimpawu nokuthuthukisa ikhwalithi yempilo, kufaka phakathi:

• Inhlanganisela chemotherapy.

• Ukwelashwa okuhlosiwe.

• Ukwelashwa ngemisebe kusetshenziswa i-131I-MIBG.

• Ukwelashwa kwemisebe yangaphandle ezindaweni (ezinjengethambo) lapho umdlavuza usabalale futhi ungeke ususwe ngokuhlinzwa.

• Ukwelashwa kwe-Ablation usebenzisa i-radiofrequency ablation noma i-cryoablation.

Sebenzisa ukusesha kwethu kokuhlolwa komtholampilo ukuthola izivivinyo zomtholampilo ezisekelwa yi-NCI ezamukela iziguli. Ungasesha izivivinyo ngokususelwa ohlotsheni lomdlavuza, iminyaka yesiguli, nalapho kwenziwa khona izivivinyo. Imininingwane ejwayelekile mayelana nokuhlolwa kwemitholampilo iyatholakala.

I-Pheochromocytoma Ngesikhathi Sokukhulelwa

AMAPHUZU ASEMQOKA

• Abesifazane abakhulelwe abane-pheochromocytoma badinga ukunakekelwa okukhethekile.

• Ukwelashwa kwabesifazane abakhulelwe abane-pheochromocytoma kungafaka ukuhlinzwa.

Ngemininingwane mayelana nokwelashwa okubalwe ngezansi, bheka isigaba se-Treatment Option Overview section.

Abesifazane abakhulelwe abane-pheochromocytoma badinga ukunakekelwa okukhethekile.

Yize kungatholakali kutholakala ngesikhathi sokukhulelwa, i-pheochromocytoma ingaba yimbi kakhulu kumama nasenganeni. Abesifazane abanobungozi obandayo be-pheochromocytoma kufanele bahlolwe ngaphambi kokubeletha. Abesifazane abakhulelwe abane-pheochromocytoma kufanele belashwe yiqembu lodokotela abangochwepheshe kulolu hlobo lokunakekelwa.

Izimpawu ze-pheochromocytoma ekukhulelweni zingafaka noma yikuphi okulandelayo:

• Umfutho wegazi ophakeme phakathi nezinyanga ezi-3 zokuqala ukhulelwe.

• Izikhathi ezingazelelwe zomfutho wegazi ophakeme.

• Umfutho wegazi ophakeme okunzima kakhulu ukuwelapha.

Ukuxilongwa kwe-pheochromocytoma kwabesifazane abakhulelwe kufaka phakathi ukuhlolwa kwamazinga e-catecholamine egazini nasemchameni. Bheka isigaba solwazi jikelele ukuthola incazelo yalezi zivivinyo nezinqubo. I-MRI ingenziwa ukuthola ngokuphepha izicubu kwabesifazane abakhulelwe ngoba ayivezi umbungu emisebeni.

Ukwelashwa kwabesifazane abakhulelwe abane-pheochromocytoma kungafaka ukuhlinzwa.

Ukwelashwa kwe-pheochromocytoma ngesikhathi sokukhulelwa kungafaka okulandelayo:

• Ukuhlinzwa ukususa ngokuphelele umdlavuza phakathi ne-trimester yesibili (inyanga yesine kuya kwelesithupha yokukhulelwa).

• Ukuhlinzwa ukususa ngokuphelele umdlavuza kuhlanganiswe nokulethwa kombungu ngengxenye yokuhlinzwa.

Ukuze ufunde kabanzi ngePheochromocytoma neParaganglioma

Ngeminye imininingwane evela ku-National Cancer Institute mayelana ne-pheochromocytoma ne-paraganglioma, bheka okulandelayo:

• Ikhasi Lasekhaya lePheochromocytoma neParaganglioma

• Ingane Pheochromocytoma kanye neParaganglioma Ukwelashwa

• I-Cryosurgery ekwelashweni komdlavuza: Imibuzo nezimpendulo

• Izindlela Zokwelapha Zomdlavuza

• Ukuhlolwa Kofuzo Kwezifo Ezizuzwayo Zokuthola Umdlavuza

Ngemininingwane ejwayelekile yomdlavuza nezinye izinsiza ezivela ku-National Cancer Institute, bheka okulandelayo:

• Mayelana Nomdlavuza

• Isiteji

• I-Chemotherapy kanye Nawe: Ukusekelwa Kwabantu Abanomdlavuza

• Ukwelashwa Kwemisebe Nawe: Ukusekelwa Kwabantu Abanomdlavuza

• Ukubhekana Nomdlavuza

• Imibuzo Ongayibuza Udokotela Wakho mayelana Nomdlavuza

• Kwabasindile Nabanakekeli

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