Iindidi / i-pheochromocytoma / isiguli / i-pheochromocytoma-unyango-pdq

Unyango lwePheochromocytoma kunye nonyango lweParaganglioma (®) –uPawu lwePatient

Ulwazi ngokubanzi malunga nePheochromocytoma kunye neParaganglioma

IINGONGOMA EZIPHAMBILI

• I-Pheochromocytoma kunye neparaganglioma ngamathumba anqabileyo avela kuhlobo olunye lwethishu.

• I-Pheochromocytoma lithumba elinqabileyo elenza kwi-adrenal medulla (embindini wedlala ye-adrenal).

• Ifom yeParagangliomas ngaphandle kwe-adrenal gland.

• Ukuphazamiseka okuthile okuzuzwe njengelifa kunye notshintsho kwiijeni ezithile kunyusa umngcipheko we-pheochromocytoma okanye paraganglioma.

• Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zibandakanya uxinzelelo lwegazi oluphezulu kunye nentloko.

• Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zinokwenzeka ngalo naliphi na ixesha okanye ziziswe ziziganeko ezithile.

• Uvavanyo oluvavanya igazi kunye nomchamo zisetyenziselwa ukufumanisa (ukufumana) kunye nokuchonga i-pheochromocytoma kunye neparaganglioma.

• Ingcebiso ngemfuzo yinxalenye yesicwangciso sonyango kwizigulana ezine-pheochromocytoma okanye iparaganglioma.

• Izinto ezithile zichaphazela ukuxela kwangaphambili (ithuba lokufumana kwakhona) kunye nokhetho lonyango.

I-Pheochromocytoma kunye neparaganglioma ngamathumba anqabileyo avela kuhlobo olunye lwethishu.

Iifomathi zeParagangliomas kwizicwili zemithambo-luvo kwiincindi zeadrenal nakufutshane nemithambo yegazi ethile kunye neentshukumo. Iiparagangliomas ezenza amadlala adrenal abizwa ngokuba zii-pheochromocytomas. Iiparagangliomas ezenza ngaphandle kwamadlala e-adrenal zibizwa ngokuba yi-extra-adrenal paragangliomas. Kule sishwankathelo, ii-paragangliomas ezongezelelweyo ze-adrenal zibizwa ngokuba yi-paragangliomas.

I-Pheochromocytomas kunye neparagangliomas zinokuba nobungozi (hayi umhlaza) okanye isifo esibi (umhlaza).

I-Pheochromocytoma lithumba elinqabileyo elenza kwi-adrenal medulla (embindini wedlala ye-adrenal).

Iifom ze-Pheochromocytoma kwiidrenal gland. Kukho amadlala amabini adrenal, enye ngaphezulu kwezintso nganye ngasemva kwesisu esingaphezulu. Idlala ngalinye leadrenal linamalungu amabini. Uluhlu lwangaphandle lwe-adrenal gland yi-adrenal cortex. Iziko le-adrenal gland yi-adrenal medulla.

I-Pheochromocytoma sisisu esinqabileyo se-adrenal medulla. Ngokwesiqhelo, i-pheochromocytoma ichaphazela i-adrenal gland enye, kodwa inokuchaphazela zombini amadlala e-adrenal. Ngamanye amaxesha kukho ngaphezulu kwesinye ithumba kwithambo elinye leadrenal.

Amadlala adrenal enza iihormone ezibalulekileyo ezibizwa ngokuba zii-catecholamines. I-Adrenaline (epinephrine) kunye ne-noradrenaline (norepinephrine) ziindidi ezimbini zeekatecholamines ezinceda ukulawula ukubetha kwentliziyo, uxinzelelo lwegazi, iswekile yegazi, kunye nendlela umzimba ophendula ngayo kuxinzelelo. Ngamanye amaxesha i-pheochromocytoma iya kukhupha i-adrenaline eyongezelelweyo kunye ne-noradrenaline egazini kwaye ibangele iimpawu okanye iimpawu zesifo.

I-anatomy yebhinqa ye-adrenal. Kukho amadlala amabini adrenal, enye ngaphezulu kwentliziyo nganye. Inxalenye engaphandle yedlala ngalinye yidrenal cortex; Icandelo elingaphakathi yi-adrenal medulla.

Ifom yeParagangliomas ngaphandle kwe-adrenal gland.

Iiparagangliomas zizidumba ezinqabileyo ezakha kufutshane nomthambo wecarotid, kunye neendlela zemithambo-luvo entloko nasentanyeni, nakwezinye iindawo zomzimba. Ezinye iiparagangliomas zenza ii-catecholamines ezongezelelweyo ezibizwa ngokuba yi-adrenaline kunye ne-noradrenaline. Ukukhutshwa kwezi katecholamines zongezelelekileyo egazini kunokubangela iimpawu okanye iimpawu zesifo.

IParaganglioma yentloko nentamo. Ithumba elinqabileyo elihlala lisondela kufutshane nomthambo wecarotid. Inokuphinda yenze ngeendlela zentloko nasentanyeni nakwezinye iindawo zomzimba.

Ukuphazamiseka okuthile okuzuzwe njengelifa kunye notshintsho kwiijeni ezithile kunyusa umngcipheko we-pheochromocytoma okanye paraganglioma.

Nantoni na eyandisa amathuba akho okufumana isifo ibizwa ngokuba yingozi. Ukuba nomngcipheko akuthethi ukuba uza kuba nomhlaza; ukungabinamngcipheko oko akuthethi ukuba awuzukufumana umhlaza. Thetha nogqirha wakho ukuba ucinga ukuba unokuba semngciphekweni.

Ezi syndromes zilandelayo kunye neenguqu zofuzo zonyusa umngcipheko we-pheochromocytoma okanye paraganglioma:

• I-endocrine neoplasia 2 syndrome, iintlobo A kunye no-B (MEN2A kunye neMEN2B).

• Isifo sikaV von Hippel-Lindau (VHL).

• Uhlobo lwe-Neurofibromatosis 1 (NF1).

• Ilifa lesifo separanglioma.

• UCarney-Stratakis dyad (paraganglioma kunye nethumbu lesisu esibuhlungu [GIST]).

• ICarney triad (paraganglioma, GIST, kunye ne-chondroma yemiphunga).

Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zibandakanya uxinzelelo lwegazi oluphezulu kunye nentloko.

Amanye amathumba awenzi i-adrenaline okanye i-noradrenaline kwaye ayibangeli zimpawu. Ezi tumors ngamanye amaxesha zifunyanwa xa kukho iqhuma entanyeni okanye xa kuvavanywa okanye kwenziwa inkqubo ngesinye isizathu. Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zenzeka xa i-adrenaline okanye i-noradrenaline ininzi ikhutshelwa egazini. Ezi kunye nezinye iimpawu kunye neempawu zinokubangelwa yi-pheochromocytoma kunye neparaganglioma okanye ngezinye iimeko. Jonga ugqirha wakho ukuba unayo nayiphi na kwezi zinto zilandelayo:

• Igazi elonyukayo.

• Intloko ebuhlungu.

• Ukubila ngamandla ngaphandle kwesizathu esaziwayo.

• Intliziyo eyomeleleyo, ekhawulezayo, okanye engaqhelekanga.

• Ukungcangcazela.

• Ukuba luthuthu kakhulu.

Olona phawu luqhelekileyo luxinzelelo lwegazi oluphezulu. Kunokuba nzima ukulawula. Uxinzelelo lwegazi oluphezulu kakhulu kunokubangela iingxaki zempilo ezinje ngokubetha kwentliziyo ngendlela engaqhelekanga, isifo sentliziyo, ukubetha, okanye ukufa.

Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zinokwenzeka ngalo naliphi na ixesha okanye ziziswe ziziganeko ezithile.

Iimpawu kunye neempawu zepheochromocytoma kunye neparaganglioma zinokwenzeka xa kusenzeka esinye sezi ziganeko zilandelayo:

• Umsebenzi onzima womzimba.

• Ukwenzakala emzimbeni okanye uxinzelelo lweemvakalelo oluninzi.

• Ukubeleka.

• Ukuya phantsi kweanesthesia.

• Utyando, kubandakanya neenkqubo zokususa ithumba.

• Ukutya ukutya okuphezulu kwi-tyramine (efana newayini ebomvu, itshokholethi kunye netshizi).

Uvavanyo oluvavanya igazi kunye nomchamo zisetyenziselwa ukufumanisa (ukufumana) kunye nokuchonga i-pheochromocytoma kunye neparaganglioma.

Ezi mvavanyo zilandelayo kunye neenkqubo zinokusetyenziswa:

• Uvavanyo lomzimba kunye nembali: Uvavanyo lomzimba ukukhangela iimpawu zempilo ngokubanzi, kubandakanya nokujonga iimpawu zesifo, ezinje ngegazi okanye nantoni na ebonakala ingaqhelekanga. Imbali yemikhwa yempilo yesigulana kunye nezifo zangaphambili kunye nonyango ziya kuthathwa.

• Uvavanyo lomchamo lweeyure ezingamashumi amabini anesine: Uvavanyo apho kuqokelelwa khona umchamo iiyure ezingama-24 ukulinganisa isixa seekatecholamines kumchamo. Izinto ezibangelwa kukophuka kwezi katecholamines nazo ziyalinganiswa. Inani elingaqhelekanga (eliphezulu okanye elisezantsi kunesiqhelo) lexabiso linokuba luphawu lwesifo kwilungu okanye kwithishu eyenzayo. Ixabiso elingaphezulu kwesiqhelo lee-catecholamines ezithile linokuba luphawu lwe-pheochromocytoma.

• Izifundo ze-catecholamine yegazi: Inkqubo apho kuvavanywa khona isampulu yegazi ukulinganisa inani leekatekamine ezikhutshelwe egazini. Izinto ezibangelwa kukophuka kwezi katecholamines nazo ziyalinganiswa. Inani elingaqhelekanga (elingaphezulu okanye elingaphantsi kwesiqhelo) lexabiso linokuba luphawu lwesifo kwilungu okanye kwithishu eyenzayo. Ixabiso elingaphezulu kwesiqhelo lee-catecholamines ezithile linokuba luphawu lwe-pheochromocytoma.

• Ukuskena i-CT (ukuskena i-CAT): Inkqubo eyenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni, ezinjengentamo, isifuba, isisu, kunye nesinqe, esithathwe kwiiangile ezahlukeneyo. Imifanekiso yenziwe yikhompyuter edityaniswe kumatshini we-x-ray. Idayi inokufakwa ngaphakathi emthanjeni okanye iginywe ukunceda amalungu okanye izicwili zibonise ngokucacileyo. Le nkqubo ikwabizwa ngokuba yi-computed tomography, ikhompyuter ye-tomography, okanye ikhompyuter ye-axial tomography.

• I-MRI (imaging resonance imaging): Inkqubo esebenzisa umazibuthe, amaza erediyo kunye nekhompyuter ukwenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni ezinjengentamo, isifuba, isisu kunye nethambo lomqolo. Le nkqubo ikwabizwa ngokuba yimifanekiso yenyukliya yokujonga umfanekiso (i-NMRI).

Ingcebiso ngemfuzo yinxalenye yesicwangciso sonyango kwizigulana ezine-pheochromocytoma okanye iparaganglioma.

Zonke izigulana ezifunyaniswa ukuba zine-pheochromocytoma okanye i-paraganglioma kufuneka zibe neengcebiso ngemfuzo ukuze zifumane umngcipheko wokuba nesifo esizuzwe njengelifa kunye neminye imihlaza enxulumene noko.

Uvavanyo lwemfuzo lunokucetyiswa ngumcebisi wemfuza kwizigulana:

• Unembali yobuqu okanye yosapho yeempawu ezinxulunyaniswa ne-pheochromocytoma okanye i-paraganglioma syndrome.

• Unamathumba kwizibini zombini ze-adrenal.

• Unesifo esingaphezulu kwesinye kwi-adrenal gland.

• Unemiqondiso okanye iimpawu zeekatecholamines ezongezelelweyo ezikhutshelwa egazini okanye ebulalayo (umhlaza) paraganglioma.

• Bafunyaniswa ngaphambi kweminyaka engama-40.

Ukuvavanywa kwemfuza ngamanye amaxesha kuyacetyiswa kwizigulana ezine-pheochromocytoma:

• Baneminyaka engama-40 ukuya kwengama-50 ubudala.

• Unethumba kwilungu elinye le-adrenal.

• Awunayo imbali yobuqu okanye yosapho yesifo esizuzwe njengelifa.

Xa utshintsho oluthile lofuzo lufunyanwa ngexesha lovavanyo lwemfuzo, uvavanyo luye lunikelwe kumalungu osapho asemngciphekweni kodwa angenazo iimpawu okanye iimpawu.

Ukuvavanywa kwemfuza akukhuthazwa kwizigulana ezingaphezulu kweminyaka engama-50.

Izinto ezithile zichaphazela ukuxela kwangaphambili (ithuba lokufumana kwakhona) kunye nokhetho lonyango.

Ukuxela kwangaphambili (ithuba lokufumana kwakhona) kunye nokhetho lonyango luxhomekeke koku kulandelayo:

• Ingaba i-tumor iyingozi okanye iyingozi.

• Nokuba ithumba likummandla omnye kuphela okanye linwenwele nakwezinye iindawo emzimbeni.

• Nokuba zikhona iimpawu okanye iimpawu ezibangelwe lixabiso elingaphezulu kwesiqhelo leekatecholamines.

• Nokuba ithumba lisandula ukufumanisa ukuba likhe lafunyaniswa okanye laphinda labuya (buyela).

Amanqanaba ePheochromocytoma kunye neParaganglioma

IINGONGOMA EZIPHAMBILI

• Emva kokuba i-pheochromocytoma kunye ne-paraganglioma zichongiwe, kwenziwa iimvavanyo zokufumanisa ukuba ngaba eli thumba linwenwele na kwamanye amalungu omzimba.

• Zintathu iindlela umhlaza onwenwa ngayo emzimbeni.

• Umhlaza unokusasazeka ukusuka apho waqala khona kwamanye amalungu omzimba.

• Akukho nkqubo yokulinganisa esemgangathweni ye-pheochromocytoma kunye neparaganglioma.

• I-Pheochromocytoma kunye neparaganglioma zichazwa njengendawo yokuhlala, yommandla, okanye ye-metastatic.

• I-pheochromocytoma yendawo kunye neparganglioma

• I-pheochromocytoma yengingqi kunye neparaganglioma

• I-pheochromocytoma yeMetastatic kunye neparaganglioma

Emva kokuba i-pheochromocytoma kunye ne-paraganglioma zichongiwe, kwenziwa iimvavanyo zokufumanisa ukuba ngaba eli thumba linwenwele na kwamanye amalungu omzimba.

Ubungakanani okanye ukusasazeka komhlaza kuhlala kuchazwa njengenqanaba. Kubalulekile ukuba wazi ukuba umhlaza sele usasazekile na ukuze ucwangcise unyango. Olu vavanyo lulandelayo kunye neenkqubo zinokusetyenziselwa ukumisela ukuba ngaba eli thumba linwenwele nakwezinye iindawo zomzimba:

• Ukuskena i-CT (ukuskena i-CAT): Inkqubo eyenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni, ezinjengentamo, isifuba, isisu, kunye nesinqe, esithathwe kwiiangile ezahlukeneyo. Imifanekiso yenziwe yikhompyuter edityaniswe kumatshini we-x-ray. Idayi inokufakwa ngaphakathi emthanjeni okanye iginywe ukunceda amalungu okanye izicwili zibonise ngokucacileyo. Isisu kunye ne-pelvis zinomfanekiso wokufumana amathumba akhupha ikateksamine. Le nkqubo ikwabizwa ngokuba yi-computed tomography, ikhompyuter ye-tomography, okanye ikhompyuter ye-axial tomography.

• I-MRI (imaging resonance imaging): Inkqubo esebenzisa umazibuthe, amaza erediyo kunye nekhompyuter ukwenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni ezinjengentamo, isifuba, isisu kunye nethambo lomqolo. Le nkqubo ikwabizwa ngokuba yimifanekiso yenyukliya yokujonga umfanekiso (i-NMRI).

• Iskena se-MIBG: Inkqubo esetyenziselwa ukufumana amathumba e-neuroendocrine, anje nge-pheochromocytoma kunye neparaganglioma. Inani elincinci kakhulu lezinto ezibizwa ngokuba yi-radioactive MIBG litofelwe kumthambo kwaye lihamba ngokuhamba kwegazi. Iiseli zesisu se-Neuroendocrine zithatha i-MIBG enemitha kwaye zifunyenwe siskena. Ukuskena kungathathwa ngaphezulu kweentsuku ezi-1-3. Isisombululo se-iodine sinokunikwa ngaphambi okanye ngexesha lovavanyo ukugcina i-thyroid gland ingafaki kakhulu kwi-MIBG.

• Iskena: Uhlobo lwe-radionuclide scan esetyenziselwa ukufumana amathumba athile, kubandakanya amathumba akhupha i-catecholamine. Inani elincinci kakhulu le-radioactive (i-hormone ehambelana nezicubu ezithile) ifakwe kwi-vein kwaye ihamba ngegazi. I-radioactive inamathela kwi-tumor kunye nekhamera ekhethekileyo efumanisa i-radioactivity isetyenziselwa ukubonisa ukuba ziphi na izidumbu emzimbeni.

• Iskena se-FDG-PET (i-fluorodeoxyglucose-positron emission tomography scan) Inkqubo yokufumana iiseli zesisu ezinobungozi emzimbeni. Inani elincinci le-FDG, uhlobo lweswekile ye-radioactive (iswekile), ifakwe kwilungu lomthambo. Iskena se-PET sijikeleza ujikeleze umzimba kwaye senze umfanekiso wendawo esetyenziswa kuyo iswekile emzimbeni. Iiseli zethumba ezinobungozi zibonakala ziqaqambile emfanekisweni kuba ziyasebenza kwaye zithatha iswekile eninzi kuneseli eziqhelekileyo.

Zintathu iindlela umhlaza onwenwa ngayo emzimbeni.

Umhlaza unokusasazeka ngezicubu, inkqubo ye-lymph, kunye negazi:

• Izicubu. Umhlaza usasazeka ukusuka apho waqala ngokukhula waya kwiindawo ezikufuphi.

• Inkqubo yeLymph. Umhlaza usasazeka ukusuka apho waqala ngokungena kwinkqubo ye-lymph. Umhlaza uhamba ngemithambo ye-lymph ukuya kwamanye amalungu omzimba.

• Igazi. Umhlaza usasazeka ukusuka apho waqala ngokungena egazini. Umhlaza uhamba ngemithambo yegazi uye kwamanye amalungu omzimba.

Umhlaza unokusasazeka ukusuka apho waqala khona kwamanye amalungu omzimba.

Xa umhlaza usasazeka kwelinye ilungu lomzimba, ubizwa ngokuba yi-metastasis. Iiseli zomhlaza ziyaphuma apho ziqale khona (ithumba lokuqala) kwaye zihamba ngenkqubo ye-lymph okanye igazi.

• Inkqubo yeLymph. Umhlaza ungena kwinkqubo ye-lymph, uhamba kwiinqanawa ze-lymph, kwaye wenze i-tumor (i-metastatic tumor) kwenye indawo yomzimba.

• Igazi. Umhlaza ungena egazini, uhambe kwimithambo yegazi, kwaye wenze ithumba (metastatic tumor) kwelinye ilungu lomzimba.

I-tumor ye-metastatic yinto efanayo yomhlaza njenge-tumor yokuqala. Umzekelo, ukuba i-pheochromocytoma iyasasazeka ithambo, iiseli zomhlaza kwithambo eneneni ziiseli ze-pheochromocytoma. Esi sifo sisifo se-metastatic pheochromocytoma, hayi umhlaza wamathambo.

Akukho nkqubo yokulinganisa esemgangathweni ye-pheochromocytoma kunye neparaganglioma.

I-Pheochromocytoma kunye neparaganglioma zichazwa njengendawo yokuhlala, yommandla, okanye ye-metastatic.

I-pheochromocytoma yendawo kunye neparganglioma

I-tumor ifunyenwe kwelinye okanye zombini i-adrenal glands (pheochromocytoma) okanye kwindawo enye kuphela (paraganglioma).

I-pheochromocytoma yengingqi kunye neparaganglioma

Umhlaza unwenwele kwii-lymph node okanye ezinye izicubu ezikufutshane nalapho kwaqala khona eli thumba.

I-pheochromocytoma yeMetastatic kunye neparaganglioma

Umhlaza usasazeke kwamanye amalungu omzimba, anjengesibindi, imiphunga, ithambo, okanye ii-lymph node ezikude.

Ukuphinda iPheochromocytoma kunye neParaganglioma

I-pheochromocytoma ephindaphindiweyo okanye iparaganglioma ngumhlaza ophindaphindiweyo (ubuyile) emva kokuba unyangiwe. Umhlaza ungabuya ubuye endaweni enye okanye kwelinye ilungu lomzimba.

Unyango ngoKhetho lonyango

IINGONGOMA EZIPHAMBILI

• Kukho iintlobo ezahlukeneyo zonyango kwizigulana ezine-pheochromocytoma okanye paraganglioma.

• Izigulana ezine-pheochromocytoma kunye neparaganglioma ezibangela imiqondiso okanye iimpawu zinyangwa ngonyango lweziyobisi.

• Iindidi ezintandathu zonyango zisetyenziswa:

• Ugqirha

• Unyango ngemitha

• Unyango ngamayeza

• Unyango lwe-Ablation

• Unyango lokudibanisa

• Unyango ekujoliswe kulo

• Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.

• Unyango lwe-pheochromocytoma kunye neparaganglioma kunokubangela iziphumo ebezingalindelekanga.

• Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.

• Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango lomhlaza.

• Iimvavanyo zokulandelela ziya kufuneka.

Kukho iintlobo ezahlukeneyo zonyango kwizigulana ezine-pheochromocytoma okanye paraganglioma.

Iindidi ezahlukeneyo zonyango ziyafumaneka kwizigulana ezine-pheochromocytoma okanye paraganglioma. Olunye unyango lusemgangathweni (unyango olusetyenziswayo ngoku), kwaye olunye luvavanywa kuvavanyo lweklinikhi. Uvavanyo lweklinikhi yonyango luphononongo olwenzelwe ukunceda ukuphucula unyango lwangoku okanye ukufumana ulwazi kunyango olutsha kwizigulana ezinomhlaza. Xa uvavanyo lwezonyango lubonisa ukuba unyango olutsha lungcono kunonyango oluqhelekileyo, unyango olutsha kunokuba lunyango olusemgangathweni. Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi. Olunye uvavanyo lwezonyango luvuleleke kuphela kwizigulana ezingaluqalanga unyango

Izigulana ezine-pheochromocytoma kunye neparaganglioma ezibangela imiqondiso okanye iimpawu zinyangwa ngonyango lweziyobisi.

Unyango lweziyobisi luqala xa kufunyaniswa i-pheochromocytoma okanye i-paraganglioma. Oku kunokubandakanya:

• Iziyobisi ezigcina uxinzelelo lwegazi luqhelekile. Umzekelo, olunye uhlobo lwesiyobisi ekuthiwa yi-alpha-blockers luyeka i-noradrenaline ekwenzeni imithambo yegazi encinci icuthe. Ukugcina imithambo yegazi ivulekile kwaye ikhululekile kuphucula ukuhamba kwegazi kunye nokunciphisa uxinzelelo lwegazi.

• Iziyobisi ezigcina ukubetha kwentliziyo eqhelekileyo. Umzekelo, olunye uhlobo lweziyobisi olubizwa ngokuba yi-beta-blockers luyeka ukusebenza kwe-noradrenaline kakhulu kwaye lucothise ukubetha kwentliziyo.

• Iziyobisi ezithintela ifuthe lehomoni ezongezelelweyo ezenziwe yidrenal gland.

Unyango lweziyobisi luhlala lunikwa iveki enye ukuya kwezintathu ngaphambi kotyando.

Iindidi ezintandathu zonyango zisetyenziswa:

Ugqirha

Utyando lokususa i-pheochromocytoma luhlala luyi-adrenalectomy (ukususwa kwenye okanye zombini iincindi zamadlala). Ngexesha lotyando, izicubu kunye ne-lymph node ngaphakathi kwesisu ziya kujongwa kwaye ukuba ithumba lisasazekile, ezi zicubu zinokususwa. Iziyobisi zinokunikwa ngaphambi, ngexesha nasemva kotyando ukugcina uxinzelelo lwegazi kunye nokubetha kwentliziyo eqhelekileyo.

Emva kotyando lokususa ithumba, kuhlolwe amanqanaba e-catecholamine egazini okanye kumchamo. Amanqanaba aqhelekileyo e-catecholamine luphawu lokuba zonke iiseli ze-pheochromocytoma zisuswe.

Ukuba zombini iincindi zamadlala zisusiwe, unyango lobomi lwehomoni oluya kuhlala ixesha elide lubuyisela iihomoni ezenziwe ngamadlala adrenal.

Unyango ngemitha

Unyango ngemitha lunyango olusebenzisa amandla e-x-reyi okanye ezinye iintlobo zemitha yokubulala iiseli zomhlaza okanye ukuzigcina zikhula. Zimbini iintlobo zonyango lwe-radiation:

• Unyango lwangaphandle lwemitha lusebenzisa umatshini ngaphandle komzimba ukuthumela imitha kumhlaza.

• Unyango lwangaphakathi lwemitha lusebenzisa into enemitha ye-radio etywinwe kwiinaliti, iimbewu, iingcingo, okanye iicatheters ezibekwe ngqo okanye kufutshane nomhlaza.

Indlela unyango lwe-radiation olunikezelwa ngayo luxhomekeke kuhlobo lomhlaza ophathwayo kwaye nokuba ubekwindawo, yengingqi, ye-metastatic, okanye ephindaphindayo. Unyango lwangaphandle lwemitha kunye nonyango lwe-131I-MIBG zisetyenziselwa ukunyanga i-pheochromocytoma.

I-Pheochromocytoma ngamanye amaxesha iphathwa nge-131I-MIBG, ethwala imitha ngqo kwiiseli zethumba. I-131I-MIBG yinto enemitha eqokelela kwiintlobo ezithile zeeseli zethumba, ibabulale ngemitha enikezelweyo. I-131I-MIBG inikwa ngokufakwa. Ayizizo zonke ii-pheochromocytomas ezithatha i-131I-MIBG, ke uvavanyo lwenziwa kuqala ukukhangela oku ngaphambi kokuba unyango luqale.

Unyango ngamayeza

I-Chemotherapy yonyango lomhlaza olusebenzisa iziyobisi ukunqanda ukukhula kweeseli zomhlaza, nokuba zibulale iiseli okanye uziyekise ekwahlukaneni. Xa i-chemotherapy ithathwa ngomlomo okanye ifakwe kwi-vein okanye kwimisipha, amachiza angena kwigazi kwaye anokufikelela kwiiseli zomhlaza kuwo wonke umzimba (systemic chemotherapy). Xa i-chemotherapy ibekwa ngqo kwi-cerebrospinal fluid, ilungu, okanye indawo yomzimba efana nesisu, iziyobisi zichaphazela kakhulu iiseli zomhlaza kwezo ndawo (chemotherapy yengingqi). Umdibaniso wekhemotherapy yonyango olusebenzisa ngaphezulu kwesiyobisi esinye somhlaza. Indlela i-chemotherapy enikezelwa ngayo ixhomekeke kuhlobo lomhlaza ophathwayo kunye nokuba ikwindawo ethile, yengingqi, ye-metastatic, okanye ephindaphindayo.

Unyango lwe-Ablation

I-Ablation lunyango lokususa okanye lokutshabalalisa ilungu lomzimba okanye izicwili okanye umsebenzi walo. Iindlela zokunyanga ezisetyenziselwa ukunceda ukubulala iiseli zomhlaza zibandakanya:

• Ukuchithwa kweRadiofrequency: Inkqubo esebenzisa amaza erediyo ukutshisa kunye nokutshabalalisa iiseli ezingaqhelekanga. Amaza kanomathotholo ahamba ngee-electrode (izixhobo ezincinci ezithwala umbane). Ukurhoxiswa kwemitha kungasetyenziselwa ukunyanga umhlaza kunye nezinye iimeko.

• Cryoablation: Inkqubo apho izicwili zomkhenkce zitshabalalise iiseli ezingaqhelekanga. I-nitrogen engamanzi okanye i-carbon dioxide engamanzi isetyenziselwa ukubamba izicwili.

Unyango lokudibanisa

Unyango lokunyanzeliswa lonyango lokuthintela umthambo okhokelela kwincindi yeadrenal. Ukuthintela ukuhamba kwegazi ukuya kwiincindi zeadrenal kunceda ukubulala iiseli zomhlaza ezikhula apho.

Unyango ekujoliswe kulo

Unyango ekujoliswe kulo lunyango olusebenzisa iziyobisi okanye ezinye izinto ukuchonga nokuhlasela iiseli ezithile zomhlaza ngaphandle kokonakalisa iiseli eziqhelekileyo. Unyango ekujoliswe kulo lusetyenziselwa ukunyanga i-pheochromocytoma ye-metastatic kunye nephindaphindayo.

I-Sunitinib (uhlobo lwe-tyrosine kinase inhibitor) lunyango olutsha olufundwayo lwe-pheochromocytoma ye-metastatic. Unyango lweTyrosine kinase inhibitor luhlobo lonyango ekujoliswe kulo oluvimba iibhloko ezifunekayo ekukhuleni kwamathumba.

Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.

Ulwazi malunga novavanyo lweklinikhi luyafumaneka kwiwebhusayithi yeNCI.

Unyango lwe-pheochromocytoma kunye neparaganglioma kunokubangela iziphumo ebezingalindelekanga.

Ngolwazi malunga neziphumo ebezingalindelekanga ezibangelwa lunyango lomhlaza, jonga iphepha lethu Iziphumo ezingalunganga.

Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.

Kwezinye izigulana, ukuthatha inxaxheba kuvavanyo lweklinikhi kunokuba lolona khetho lonyango lufanelekileyo. Uvavanyo lwezonyango luyinxalenye yenkqubo yophando ngomhlaza. Uvavanyo lwezonyango luyenziwa ukufumanisa ukuba unyango lomhlaza olutsha lukhuselekile kwaye luyasebenza okanye lungcono kunonyango oluqhelekileyo.

Uninzi lonyango oluqhelekileyo lwanamhlanje lomhlaza lusekwe kuvavanyo lweklinikhi lwangaphambili. Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zingafumana unyango olusemgangathweni okanye zibe phakathi kwabokuqala ukufumana unyango olutsha.

Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zikwanceda ukuphucula indlela umhlaza oza kunyangwa ngayo kwixa elizayo. Nokuba uvavanyo lwezonyango alukhokeleli kunyango olusebenzayo, bahlala bephendula imibuzo ebalulekileyo kwaye bancedisa ukuqhubela phambili uphando.

Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango lomhlaza.

Olunye uvavanyo lwezonyango lubandakanya kuphela izigulana ezingekafumani unyango. Olunye uvavanyo lokuvavanywa kunyango kwizigulana ezinomhlaza ongakhange ubengcono. Kukwakho nezilingo zeklinikhi ezivavanya iindlela ezintsha zokumisa umhlaza ekubuyeleni (ekubuyeni) okanye ukunciphisa iziphumo ebezingalindelekanga zonyango lomhlaza.

Uvavanyo lwezonyango lwenzeka kwiindawo ezininzi zelizwe. Ulwazi malunga novavanyo lweklinikhi oluxhaswe yi-NCI lunokufumaneka kwiphepha lewebhu le-NCI. Uvavanyo lwezonyango oluxhaswa yiminye imibutho lunokufumaneka kwiwebhusayithi yeClinicalTrials.gov.

Iimvavanyo zokulandelela ziya kufuneka.

Olunye uvavanyo olwenziweyo ukufumanisa umhlaza okanye ukufumanisa ubungakanani bomhlaza lunokuphindwa. Olunye uvavanyo luya kuphindwa ukuze kubonwe ukuba lusebenza njani unyango. Izigqibo malunga nokuqhubeka, ukutshintsha, okanye ukuyeka unyango ziya kusekelwa kwiziphumo zolu vavanyo.

Ezinye zeemvavanyo ziya kuqhubeka ukwenziwa amaxesha ngamaxesha emva kokuba unyango luphelile. Iziphumo zolu vavanyo zingabonisa ukuba imeko yakho itshintshile okanye ukuba umhlaza ubuyile (buyela). Olu vavanyo ngamanye amaxesha lubizwa ngokuba luvavanyo olulandelayo.

Kwizigulana ezine-pheochromocytoma okanye iparaganglioma ebangela iimpawu, amanqanaba e-catecholamine egazini nasemchameni aya kujongwa rhoqo. Amanqanaba eCatecholamine aphezulu kunesiqhelo angangumqondiso wokuba umhlaza ubuyile.

Kwizigulana ezineparaganglioma ezingabangeli zimpawu, iimvavanyo zokulandela ezinje nge-CT, iMRI, okanye iMIBG scan kufuneka yenziwe minyaka le.

Kwizigulana ezine-pheochromocytoma ezuzwe njengelifa, amanqanaba e-catecholamine egazini nasemchameni aya kujongwa rhoqo. Olunye uvavanyo lokuvavanywa luyakwenziwa ukukhangela amanye amathumba anxulunyaniswe nesifo esizuzwe njengelifa.

Thetha nogqirha wakho malunga nokuba zeziphi iimvavanyo ekufuneka zenziwe kwaye kaninzi kangakanani. Izigulana ezine-pheochromocytoma okanye iparaganglioma zifuna ukulandelelwa ubomi bazo bonke.

Ukhetho kunyango lwePheochromocytoma kunye neParaganglioma

Kweli Candelo

• Indawo yasePheochromocytoma kunye neParaganglioma

• Ilifa lePheochromocytoma

• Ingingqi yePheochromocytoma kunye neParaganglioma

• I-Metastatic Pheochromocytoma kunye neParaganglioma

• Ukuphinda iPheochromocytoma kunye neParaganglioma

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

Indawo yasePheochromocytoma kunye neParaganglioma

Unyango lwe-pheochromocytoma okanye i-paraganglioma enobungozi bendawo ihlala ityandwa ukuze isuse ngokupheleleyo ithumba. Ukuba ithumba ikwindawo ye-adrenal, yonke le adrenal idlala isuswe.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Ilifa lePheochromocytoma

Kwizigulana ezine-pheochromocytoma ezuzwe njengelifa elinxulunyaniswe ne-endocrine neoplasia (MEN2) okanye i-von Hippel-Lindau (VHL) syndrome, amathumba ahlala esenza kwiincindi zeadrenal. Amathumba ahlala enobungozi.

• Unyango lwe-pheochromocytoma olilifa lemveli elenza kwilungu elinye le-adrenal lutyando lokususa ngokupheleleyo le ndlala. Olu tyando lunokunceda abaguli bathintele i-steroid engapheliyo kunyango olunokutshintsha kunye nokungabikho ngokwaneleyo kwe-adrenal.

• Unyango lwe-pheochromocytoma olilifa lemveli elenzeka kuzo zombini iadrenal gland okanye kamva kwiifom kwi-adrenal gland eseleyo inokuhlinzwa ukuze kususwe ithumba kunye nezicubu ezincinci eziqhelekileyo kwi-adrenal cortex kangangoko. Olu tyando lunokunceda abaguli ukuba baphephe unyango lwehomoni oluza kuthatha ixesha elide kunye neengxaki zempilo ngenxa yokulahleka kwehomoni eyenziwe yindlala ye-adrenal.

Ingingqi yePheochromocytoma kunye neParaganglioma

Unyango lwe-pheochromocytoma okanye i-paraganglioma ethe yasasazeka kwizitho ezikufuphi okanye kwii-lymph node ngotyando lokususa ngokupheleleyo ithumba. Izitho ezikufutshane apho umhlaza usasazeke khona, njengezintso, isibindi, inxalenye yesitya esikhulu segazi, kunye ne-lymph nodes, nazo zingasuswa.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

I-Metastatic Pheochromocytoma kunye neParaganglioma

Unyango lwe-pheochromocytoma ye-metastatic okanye i-paraganglioma inokubandakanya oku kulandelayo:

• Ugqirha lokususa umhlaza ngokupheleleyo, kubandakanya amathumba athe saa kwiindawo ezikude zomzimba.

• Unyango lokunyanga, ukunciphisa iimpawu kunye nokuphucula umgangatho wobomi, kubandakanya:

• Ugqirha lokususa umhlaza omninzi kangangoko kunokwenzeka.

• Ukudibanisa i-chemotherapy.

• Unyango ngemitha kunye ne131I-MIBG.

• Unyango lwangaphandle lwemitha kwiindawo (ezinje ngethambo) apho umhlaza usasazeke khona kwaye awunakususwa ngotyando.

• Ukudibanisa (unyango lokuthintela umthambo onikezela ngegazi kwithumba).

• Unyango lwe-Ablation usebenzisa i-radiofrequency ablation okanye i-cryoablation yamathumba kwisibindi okanye ithambo.

• Uvavanyo lweklinikhi yonyango ekujoliswe kulo kunye ne-tyrosine kinase inhibitor.

• Uvavanyo lweklinikhi yonyango lwangaphakathi lwemitha usebenzisa into entsha enemitha.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Ukuphinda iPheochromocytoma kunye neParaganglioma

Unyango lwe-pheochromocytoma ephindaphindiweyo okanye iparaganglioma inokubandakanya oku kulandelayo:

• Ugqirha ukususa ngokupheleleyo umhlaza.

• Xa utyando lokususa umhlaza lungenakwenzeka, unyango olunyangayo lokunciphisa iimpawu kunye nokuphucula umgangatho wobomi, kubandakanya:

• Ukudibanisa i-chemotherapy.

• Unyango ekujoliswe kulo.

• Unyango lwe-radiation usebenzisa i-131I-MIBG.

• Unyango lwangaphandle lwemitha kwiindawo (ezinje ngethambo) apho umhlaza usasazeke khona kwaye awunakususwa ngotyando.

• Unyango lwe-Ablation usebenzisa i-radiofrequency ablation okanye i-cryoablation.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

I-Pheochromocytoma Ngexesha lokukhulelwa

IINGONGOMA EZIPHAMBILI

• Abafazi abakhulelweyo abane-pheochromocytoma bafuna ukhathalelo olukhethekileyo.

• Unyango lwabasetyhini abakhulelweyo abane-pheochromocytoma inokubandakanya utyando.

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

Abafazi abakhulelweyo abane-pheochromocytoma bafuna ukhathalelo olukhethekileyo.

Nangona kunqabile ukuba kufunyaniswe ukuba ukhulelwe, i-pheochromocytoma inokuba mandundu kakhulu kumama nakwisana. Abasetyhini abanomngcipheko okhulayo we-pheochromocytoma kufuneka bavavanywe ngaphambi kokubeleka. Abafazi abakhulelweyo abane-pheochromocytoma kufuneka banyangwe liqela loogqirha abaziingcali kolu hlobo lokhathalelo.

Iimpawu ze-pheochromocytoma xa ukhulelwe zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Uxinzelelo lwegazi oluphezulu ngexesha leenyanga ezintathu zokuqala zokukhulelwa.

• Amaxesha ngesiquphe koxinzelelo lwegazi.

• Uxinzelelo lwegazi oluphezulu kunzima kakhulu ukunyanga.

Ukuchongwa kwe-pheochromocytoma kubafazi abakhulelweyo kubandakanya ukuvavanywa kwamanqanaba e-catecholamine egazini nasemchameni. Jonga icandelo loLwazi ngokubanzi malunga nenkcazo yezi mvavanyo kunye neenkqubo. I-MRI inokwenziwa ukufumana ngokukhuselekileyo amathumba kwabasetyhini abakhulelweyo kuba ayivezi mntwana kwimitha.

Unyango lwabasetyhini abakhulelweyo abane-pheochromocytoma inokubandakanya utyando.

Unyango lwe-pheochromocytoma ngexesha lokukhulelwa kunokubandakanya oku kulandelayo:

• Ugqirha lokususa ngokupheleleyo umhlaza kwikota yesibini (kwinyanga yesine ukuya kwinyanga yesithandathu yokukhulelwa).

• Ugqirha lokususa ngokupheleleyo umhlaza odityaniswe nokuhanjiswa komntwana ngecandelo le-cearean.

Ukufunda ngakumbi ngePheochromocytoma kunye neParaganglioma

Ngolwazi oluthe kratya kwiZiko leSizwe loMhlaza malunga ne-pheochromocytoma kunye neparaganglioma, jonga oku kulandelayo:

• IPheochromocytoma kunye neKhaya laseParaganglioma

• Ubuntwana bePheochromocytoma kunye nonyango lweParaganglioma

• I-Cryosurgery kunyango lomhlaza: Imibuzo kunye neempendulo

• Iithagethi zoMhlaza ezijolise kuko

• Uvavanyo lweMfuzo kwii-Syndromes eziSebenzayo zoMhlaza

Ngolwazi lomhlaza ngokubanzi kunye nezinye izixhobo ezivela kwiNational Cancer Institute, jonga oku kulandelayo:

• Malunga noMhlaza

• Ukuqokelela

• I-Chemotherapy kunye nawe: Inkxaso yabantu abanomdlavuza

• Unyango ngemitha kunye nawe: Inkxaso yabantu abanomhlaza

• Ukujamelana noMhlaza

• Imibuzo yokubuza ugqirha wakho malunga neCancer

• Abasindileyo kunye nabaNonopheli

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