Singakhuluma ngesimila esingavamile esibizwa ngokuthi i-Pheochromocytoma namuhla?

Ingabe ngezinye izikhathi uba nomfutho wegazi ophakeme ngokuzumayo? Noma umane ujuluke? Ingabe uphathwa yikhanda ngenxa yenhliziyo eshaya ngamandla? Ungasheshi ukucabanga ukuthi ezinye zalezi zinto ziyisifo esibi. Kodwa-ke, uma lezi zimpawu ziqhubeka, ikakhulukazi ngomfutho wegazi ophakeme okunzima ukuwulawula, kuwumqondo omuhle ukukhathazeka kancane. Namuhla sizokhuluma ngesimo esingavamile kancane, kodwa uma sitholakale kahle, singelashwa kahle kakhulu. Lokho kubizwa ngokuthi i-pheochromocytoma.

Iyini i-Pheochromocytoma? Ake sikuqonde kalula.

Kalula nje, i-pheochromocytoma iyisimila esikhula engxenyeni ephakathi yezindlala ze-adrenal, i-adrenal medulla. Lawa mathumba akhiwa uhlobo olukhethekile lweseli olubizwa ngamaseli e-chromaffin. Lawa maseli akhiqiza futhi akhiphe ama-hormone egazini adingekayo ekusabeleni komzimba wethu "ukulwa noma ukubaleka". Cabanga ngakho, lapho usaba ngokuzumayo noma ubhekene nokucindezeleka okukhulu, izinguquko ezenzeka emzimbeni wakho - izinga lokushaya kwenhliziyo yakho liyakhuphuka, ujuluka, umzimba wakho uyathuthumela - lawa ma-hormone abangela lokhu.

I-Pheochromocytoma ivame ukwakheka endlaleni eyodwa ye-adrenal. Kodwa-ke, ngezinye izikhathi ingakhula kuzo zombili izindlala. Kungenzeka ukuthi kube nezimila ezingaphezu kweyodwa endlaleni eyodwa.

Okubalulekile, ama-pheochromocytomas amaningi awabi nobungozi (awabi nobungozi) . Lokhu kusho ukuthi awasabalali kwezinye izingxenye zomzimba. Kodwa-ke, phakathi kuka-10% no-15% angaba nomdlavuza. Uma lesi kuyisimo somdlavuza, sichazwa ngezigaba eziningana eziyinhloko, kuye ngokuthi sisabalale kanjani:

• I-pheochromocytoma etholakala endaweni ethile: Isimila sitholakala endlaleni eyodwa noma zombili ze-adrenal.
• I-pheochromocytoma yesifunda: Umdlavuza ususakazekele kuma-lymph node eduze kwezindlala ze-adrenal noma kwezinye izicubu.
• I-Metastatic pheochromocytoma: Umdlavuza ususakazekele ezithweni ezikude njengesibindi, amaphaphu, noma amathambo.
• I-pheochromocytoma ephindaphindayo: Umdlavuza ubuyile ngemuva kokwelashwa. Ungaba sendaweni efanayo owawuyiyo ngaphambili noma kwenye indawo.

Ziyini lezi zindlala ze-adrenal emzimbeni wethu? Uyini umsebenzi wazo?

Sinezindlala ezimbili ze-adrenal. Zitholakala ngemuva kwesisu sethu, ngaphezu kwezinso zethu, njengesivalo. Ziyingxenye yesistimu yethu ye-endocrine. Indlala ngayinye ye-adrenal inezingxenye ezimbili eziyinhloko. Ungqimba lwangaphandle lubizwa ngokuthi i-adrenal cortex, kanti ingxenye ephakathi ibizwa ngokuthi i-adrenal medulla.

I-adrenal medulla yethu ikhiqiza iqembu lamahomoni abizwa ngokuthi ama-catecholamines. Lawa mahomoni alawula izinqubo eziningana ezibaluleke kakhulu emzimbeni wethu:

• Isilinganiso senhliziyo
• Ukushaya kwegazi
• Izinga likashukela egazini `(I-glucose egazini)`
• Indlela umzimba wethu osabela ngayo ekucindezelekeni (impendulo "yokulwa noma yokubaleka")

Izinhlobo eziyinhloko zama-catecholamine yilezi:

• I-Dopamine
• I-Epinephrine (i-Adrenaline) `(i-Epinephrine / i-Adrenaline)`
• I-Norepinephrine (i-Noradrenaline) `(i-Norepinephrine / i-Noradrenaline)`

Uma kukhona i-pheochromocytoma, ingakhipha ama-hormone amaningi, i-adrenaline kanye ne-noradrenaline, egazini kunokuba kudingekile. Yilapho izimpawu ezahlukahlukene ziqala khona ukuvela.

Uyini umehluko phakathi kwePheochromocytoma neParaganglioma?

Lezi yizinhlobo ezimbili ezifanayo kakhulu, ezingavamile zamathumba. Zombili zivela kumaseli afanayo e-chromaffin esixoxe ngawo ngaphambili. Kodwa-ke, i-pheochromocytoma ivela engxenyeni ephakathi ye-adrenal gland (i-adrenal medulla), kuyilapho i-paraganglioma ivela kwezinye izindawo ezingaphandle kwe-adrenal gland .

Ubani onamathuba amaningi okuthola lesi simo? Sivame kangakanani?

I-Pheochromocytoma ingakhula kunoma yimuphi ubudala, kodwa ivame kakhulu kubantu abaneminyaka ephakathi kuka-30 no-50. Cishe u-10% wamacala abikwa ebuntwaneni.

Lesi yisifo esingavamile kakhulu . Kunzima ukusho ukuthi bangaki abantu abanalesi simo. Ngenxa yokuthi abanye abantu ababonisi zimpawu, lesi sifo singase singatholakali. Kulinganiselwa ukuthi abantu abangaphansi kuka-1% abanomfutho wegazi ophezulu bane-pheochromocytoma.

Yiziphi izimpawu eziboniswa umuntu one-pheochromocytoma?

Izimpawu ze-Pheochromocytoma zenzeka lapho isimila sikhipha ama-hormone amaningi kakhulu i-adrenaline (i-epinephrine) noma i-noradrenaline (i-norepinephrine) egazini. Kodwa-ke, amanye ama-tumors awakhiqizi la ma-hormone amaningi kakhulu futhi angase angabonakali.

Izimpawu ezivame ukubonakala yilezi:

• Umfutho wegazi ophezulu (Umfutho wegazi ophakeme): Lolu uphawu oluyinhloko. Ngezinye izikhathi kunzima ukulawula.
• Ikhanda elibuhlungu: Kungaba ikhanda elibuhlungu elibuhlungu kakhulu, elingazelelwe.
• Ukujuluka ngokweqile ngaphandle kwesizathu.
• Ukushaya kwenhliziyo kuwumuzwa wokushaya kwenhliziyo okusheshayo, okungajwayelekile, noma okushaya ngamandla.
• Ukuzizwa sengathi umzimba wakho uyathuthumela.

Izimpawu ezingavamile:

• Ubuhlungu besifuba kanye/noma besisu.
• Isikhumba siba mhlophe ngokuzumayo kunokujwayelekile.
• Ukucanuzela kwenhliziyo kanye/noma ukuhlanza.
• Uhudo.
• Ukuqunjelwa.
• Ukwehla kwengcindezi yegazi okubangelwa yi-orthostatic kuwukwehla kwengcindezi yegazi ngokuzumayo uma usukuma. Lokhu kusho ukuthi uzizwa unesizungu uma usukuma ngokuzumayo uhleli.
• Ukwehlisa isisindo ngaphandle kwesizathu.

Lezi zimpawu zingase zivele noma zibe zimbi kakhulu ngemva kwezehlakalo ezithile. Isibonelo:

• Umsebenzi onzima womzimba.
• Ukulimala ngokomzimba noma ukucindezeleka okukhulu kwengqondo.
• Ukubeletha.
• Ukuthola i-anesthesia.
• Ukwenza ukuhlinzwa.
• Ukudla ukudla okucebile nge-tyramine (isib. iwayini elibomvu, ushokoledi, ushizi).

Ingabe izimpawu zihlala zikhona? Noma ziyafika zidlule?

Umuntu one-pheochromocytoma angase abe nomfutho wegazi ophakeme oqhubekayo, noma ungase ubuye udlule .

Abanye abantu bangase babe "nokuhlaselwa yi-paroxysmal," noma ukuhlaselwa yizimpawu ngokuzumayo. Lokhu kusho ukuthi kukhona ukwanda okungazelelwe komfutho wegazi, okuhambisana nezimpawu ezifana nekhanda elibuhlungu, ukushaya kwenhliziyo okukhuphukile, kanye nokujuluka ngokweqile. Lokhu "kuhlaselwa" kungenzeka izikhathi eziningana ngosuku, noma kanye noma kabili ngenyanga.

Okubalulekile: Uma unezinye zalezi zimpawu, ikakhulukazi umfutho wegazi ophakeme ngokuzumayo, ikhanda elibuhlungu, ukujuluka, kanye nokushaya kwenhliziyo, kubaluleke kakhulu ukufuna iseluleko sikadokotela.

Kungani i-pheochromocytoma ikhula? Ziyini izimbangela?

Ezimweni eziningi, akukho mbangela ethile engatholakala ye-pheochromocytoma. Kwenzeka ngokungahleliwe.

Kodwa-ke, ezimweni ezingama-25% kuya ku-35%, lesi simo sihlobene nezimo zofuzo. Ezinye zezimo eziyinhloko yilezi:

• I-Multiple endocrine neoplasia 2 syndrome, izinhlobo A kanye no-B (MEN2A kanye ne-MEN2B)
• Isifo se-Von Hippel-Lindau (VHL)
• Uhlobo 1 lwe-Neurofibromatosis (NF1)
• I-Hereditary paraganglioma syndrome
• I-Carney-Stratakis dyad [i-paraganglioma kanye ne-gastrointestinal stromal tumor (GIST)]
• I-Carney triad (i-paraganglioma, i-GIST kanye ne-pulmonary chondroma)

Ngaphezu kwalezi zimo zofuzo, i-pheochromocytoma ingakhula ngenxa yokuguquka kwezakhi zofuzo okungenani eziyi-10 ezahlukene.

Lesi sifo sitholakala kanjani? (Ukuxilongwa)

I-Pheochromocytoma ingaba nzima ukuyixilonga ngoba iyisimila esingavamile futhi ngezinye izikhathi ayibangeli zimpawu. Ngezinye izikhathi, isimila sitholakala ngengozi ngesikhathi sokuhlolwa okwenziwe ngesinye isizathu.

Kunezizathu eziningana zokuthi kungani udokotela engase asole lesi sifo:

• EyakhoUmlando wezokwelapha onemininingwane, ikakhulukazi ukuthi ngabe kukhona yini emndenini oke waba ne-pheochromocytoma ngaphambili.
• Ukuhlolwa okuphelele ngokomzimba nangokwezokwelapha.
• Ezinye izimpawu ezithile , isibonelo "ukuhlaselwa kwe-paroxysmal" esikhulume ngakho kanye nomfutho wegazi ophakeme ongalawulwa ukwelashwa okuvamile.

Hlobo luni lokuhlolwa okwenziwayo?

Udokotela angase ancome ukuhlolwa okulandelayo ukuqinisekisa ukuthi une-pheochromocytoma:

• Ukuhlolwa komchamo kwamahora angama-24: Lokhu kuhilela ukuqoqa umchamo wakho usuku lonke nokulinganisa inani lama-catecholamine kuwo. Kuphinde kulinganise izinto ezikhiqizwa lapho la ma-hormone ewohloka. Uma la mazinga ephakeme kunokuvamile, kungaba uphawu lwe-pheochromocytoma.
• Ukuhlolwa kwe-catecholamine yegazi: Lokhu kulinganisa amazinga e-catecholamine egazini. Njengokuhlolwa komchamo, kuhlola nezinto ezikhiqizwa ukuwohloka kwama-hormone.
• I-CT scan (i-Computer Tomography scan): Lokhu kuthatha uchungechunge lwe-X-rays ukudala izithombe ezinemininingwane zangaphakathi lomzimba wakho. Udokotela wakho angase akuncome lokhu ukuze abheke izindlala zakho ze-adrenal.
• I-MRI scan (MRI - Magnetic Resonance Imaging): Lokhu kusebenzisa i-magnet, amaza omsakazo, kanye nekhompyutha ukwenza izithombe ezinemininingwane yengaphakathi lomzimba. Kuphinde kusetshenziswe ukuhlola izindlala ze-adrenal.

Uma lesi sifo sesiqinisekisiwe, kungase kwenziwe ezinye izivivinyo ukuze kutholakale ukuthi isimila sinomdlavuza (siyingozi) noma asibi (singebi), nokuthi ngabe sesisakazekele kwezinye izingxenye zomzimba.

Kungani Ukuhlolwa Kwezakhi Zofuzo Kubalulekile?

Uma kutholakala ukuthi une-pheochromocytoma, udokotela wakho cishe uzoncoma ukwelulekwa nokuhlolwa kwezakhi zofuzo ukuze kutholakale ukuthi unesifo esizuzwe njengefa esikubeka engcupheni yokuthola eminye imidlavuza.

Ukuhlolwa kofuzo kunganconywa ezimweni ezinjengalezi:

• Uma wena noma othile emndenini wakho enezimpawu ezihlobene ne-pheochromocytoma noma i-paraganglioma syndrome.
• Uma unezimila kuzo zombili izindlala zakho ze-adrenal.
• Uma kunezimila ezingaphezu kweyodwa endlaleni eyodwa ye-adrenal.
• Uma kunezimpawu zokwanda kwamazinga e-catecholamine egazini.
• Uma i-pheochromocytoma itholakala ingakafiki eminyakeni engama-40.

Uma ukuhlolwa kwezakhi zofuzo kuthola ushintsho lwezakhi zofuzo, umeluleki wezakhi zofuzo angancoma ukuthi amanye amalungu omndeni wakho (labo abangenazo izimpawu kodwa abasengozini) nawo ahlolwe.

Yiziphi izindlela zokwelapha i-Pheochromocytoma?

Ukwelashwa okungcono kakhulu kwalokhu ukususa isimila ngokuhlinzwa, uma kungenzeka .

Ukukhetha ukwelashwa kuncike ezintweni eziningana:

• Usayizi wesimila.
• Kungakhathaliseki ukuthi isimila sinomdlavuza (siyingozi) noma asibi (singebi).
• Ingabe kukhona izimpawu ezibangelwa ukwanda kwamahomoni e-catecholamine.
• Ingabe lesi simila sihlala endaweni eyodwa, noma sisakazekele kwezinye izingxenye zomzimba? (Sisabalale)
• Ukuthi lesi sifo satholakala okokuqala, noma ukuthi sesiphinde savela yini ngemva kokwelashwa kwangaphambilini.

Uma unezimpawu ezibangelwa ukwanda kwama-hormone e-adrenal, udokotela wakho angase akunike imithi yokulawula lezo zimpawu. Isibonelo:

• Imithi yokugcina umfutho wegazi usezingeni elijwayelekile, njenge-alpha-blockers.
• Imithi yokugcina izinga lokushaya kwenhliziyo lisezingeni elijwayelekile, njenge-beta-blockers.
• Imithi evimba imiphumela yama-hormone akhishwa ngokweqile yi-adrenal gland.

Izindlela eziyinhloko zokwelapha i-pheochromocytoma yilezi:

• Ukuhlinzwa
• Ukwelashwa ngemisebe
• Ukwelashwa ngamakhemikhali
• Ukwelashwa kokukhipha isisu
• Ukwelashwa kwe-Embolization
• Ukwelashwa okuqondiwe

Ngokubambisana, wena nethimba lakho lezokwelapha nizonquma uhlelo lokwelashwa olulungele wena.

Izindlela zokwelapha eziyinhloko

• Ukuhlinzwa:

Lena yindlela eyinhloko yokwelapha i-pheochromocytoma. Cishe ama-90% ezimila angasuswa ngempumelelo ngokuhlinzwa .

Udokotela wakho angase akuncome ukuhlinzwa ukuze kususwe eyodwa noma zombili izindlala zakho ze-adrenal (i-adrenalectomy). Ngesikhathi sokuhlinzwa, udokotela ohlinzayo uzohlola izicubu ezizungezile kanye nama-lymph node ukuze abone ukuthi isimila sesisabalele yini. Uma sesisabalele, uzosusa leso zicubu, uma kungenzeka.

Ngemva kokuhlinzwa, igazi noma umchamo wakho kuzohlolwa amazinga e-catecholamine. Uma amazinga ebuyela esimweni esijwayelekile, kusho ukuthi wonke amaseli e-pheochromocytoma asusiwe.

Uma zombili izindlala ze-adrenal zisusiwe, kuzodingeka uthathe ukwelashwa ngama-hormone impilo yakho yonke ukuze uthathe indawo yama-hormone akhiqizwa yizindlala ze-adrenal.

• Ukwelashwa ngemisebe:

Lokhu kuyindlela yokwelapha esetshenziselwa ukubulala amangqamuzana omdlavuza noma ukuwavimba ekukhuleni kwawo. Lokhu kwenziwa ngendlela engalimazi izicubu ezinempilo ngangokunokwenzeka.

Kunezinhlobo ezimbili zokwelapha ngemisebe:

• Ukwelashwa ngemisebe yangaphandle: Imisebe ihanjiswa endaweni yomdlavuza isuka emshinini ongaphandle komzimba.
• Ukwelashwa ngemisebe yangaphakathi: Into ekhipha imisebe ifakwa ezinalitini, ezinhlamvini, ezintanjeni, noma kuma-catheter bese ifakwa udokotela endaweni noma eduze nendawo yomdlavuza.

Uhlobo lokwelashwa ngemisebe luncike ekutheni umdlavuza usendaweni ethile, ungowesifunda, usakazeka kalula, noma uphindaphinda. I-pheochromocytoma ebulalayo ivame ukwelashwa ngokwelashwa ngemisebe yangaphandle kanye/noma ngokwelashwa nge-131I-MIBG. I-131I-MIBG iyisithako esikhipha imisebe esinamathela kwamanye amangqamuzana omdlavuza bese siwabulala.

• Ukwelashwa ngamakhemikhali:

Lokhu kusebenzisa imithi ukubulala amangqamuzana omdlavuza, ukuwavimba ukuthi angahlukani futhi angaphindaphindi, noma ukuvimba ukukhula komdlavuza. Le mithi ivame ukunikezwa ngemithambo yegazi. Nakuba lokhu kuyindlela yokwelapha ephumelelayo, ingaba nemiphumela emibi.

• Ukwelashwa kokukhipha isisu:

Lokhu kuyindlela yokwelapha engangenisi kakhulu. Kusebenzisa ukushisa okukhulu noma ukubanda okukhulu ukubhubhisa izimila.

• Ukususwa kwama-radiofrequency: Ukususwa kwama-radiofrequency kusebenzisa amaza omsakazo ukushisa nokubhubhisa amangqamuzana omdlavuza kanye namangqamuzana angajwayelekile.
• I-Cryoablation: Isebenzisa i-nitrogen ewuketshezi noma i-carbon dioxide ewuketshezi ukuze iqandiswe futhi ibhubhise amangqamuzana omdlavuza kanye namangqamuzana angavamile.

• Ukwelashwa kwe-Embolization:

Kulokhu, umthambo ohlinzeka ngegazi endlaleni ye-adrenal uyavaleka. Lokhu kumisa ukugeleza kwegazi endlaleni, futhi amangqamuzana omdlavuza akhula lapho ayafa.

• Ukwelashwa okuqondiwe:

Lokhu kusebenzisa izidakamizwa noma ezinye izinto ezihlasela ngqo amangqamuzana omdlavuza kuphela ngaphandle kokulimaza amangqamuzana anempilo. Lokhu kwelashwa kusetshenziselwa i-metastatic kanye ne-recurrent pheochromocytoma.

Kucwaningwa nge-tyrosine kinase inhibitor ebizwa ngokuthi i-sunitinib ukuze kutholakale i-metastatic pheochromocytoma. Le mithi ivimbela ukukhula kwesimila.

Ingabe lesi sifo singavinjelwa?

Ngeshwa, ayikho indlela yokuvimbela i-pheochromocytoma. Kodwa-ke, uma usengozini yokuthola lesi sifo ngenxa yezifo zofuzo kanye nezifo ezidluliselwa ngofuzo, ukwelulekwa ngofuzo kungasiza ekuhlolweni nasekutholakaleni kusenesikhathi.

Uma noma yimuphi wamalungu omndeni wakho aseduze (abantakwenu, abazali) eke waba ne-pheochromocytoma, noma uma unesifo sofuzo esifana nalesi esixoxe ngaso ngaphambilini (i-Multiple endocrine neoplasia 2 syndrome, isifo se-Von Hippel-Lindau (VHL), i-Neurofibromatosis uhlobo 1 (NF1), i-Hereditary paraganglioma syndrome, i-Carney-Stratakis dyad, i-Carney triad), kubaluleke kakhulu ukukhuluma nodokotela wakho ngakho.

Iyini ithuba lokululama ngemva kokwelashwa? (Isibikezelo)

Umbono we-pheochromocytoma ngokuvamile muhle kakhulu uma welashwa.Sesishilo kakade ukuthi cishe ama-90% ezimila angasuswa ngempumelelo ngokuhlinzwa.

Kodwa-ke, uma ingelashwa, lesi simo singaholela ezinkingeni ezinkulu, ezisongela ngisho nokuphila . Isibonelo:

• I-cardiomyopathy
• I-Myocarditis
• Ukopha okungalawuleki ebuchosheni (Ukuphuma kwegazi ebuchosheni)
• Ukuqongelela koketshezi emaphashini (i-pulmonary edema)

Ezinye iziguli ezine-pheochromocytoma nazo zisengozini yokushaywa yisifo sohlangothi noma i-myocardial infarction.

Kufanele ngimbone nini udokotela?

• Uma kutholakala ukuthi une-pheochromocytoma futhi uba nanoma yiziphi izimpawu ezikukhathazayo, bheka udokotela wakho ngokushesha.
• Uma unezimpawu ze-pheochromocytoma, njengomfutho wegazi ophakeme kanye nekhanda elibuhlungu, khuluma nodokotela wakho. Nakuba i-pheochromocytoma ingavamile, kubalulekile ukwelapha umfutho wegazi ophakeme.
• Uma wazi ukuthi omunye wezihlobo zakho eziseduze (abafowenu nodadewenu, abazali) unesifo sofuzo esifana nesifo se-'Multiple endocrine neoplasia 2' noma isifo se-'Von Hippel-Lindau (VHL), ungase ube sengozini enkulu yokuthola i-pheochromocytoma, ngakho-ke bonana nodokotela ukuze uxoxe ngokuhlolwa kofuzo.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Uma kutholakale ukuthi une-pheochromocytoma, kungaba usizo ukubuza udokotela wakho le mibuzo:

• Kungani ngathola i-pheochromocytoma?
• Ingabe izingane zami kanye/noma izihlobo zami zingaba ne-pheochromocytoma?
• Yiziphi izindlela zokwelapha enginazo?
• Iyini imiphumela emibi yokwelashwa okuhlukahlukene?
• Ngingazilawula kanjani izimpawu zami?

Okokugcina, izinto ezibaluleke kakhulu okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngakho-ke, yize i-pheochromocytoma iyisimila esingavamile, ivame ukuba yingozi futhi yelapheka . Futhi, ingahamba emindenini, ngakho-ke uma wena noma othile emndenini wakho etholakala enalesi simo, kubalulekile ukuthola ukuhlolwa kofuzo. Lokhu kungasiza futhi ekunqumeni ukuthi usengozini yini yezinye izinkinga zempilo.

Khumbula, uma unemibuzo mayelana nengozi yakho yokuthola i-pheochromocytoma noma ngalesi sifo, ungesabi ukukhuluma nodokotela wakho. Bakhona ukuzokusiza. Hlala uphilile!

I-Pheochromocytoma , i-adrenal gland, ama-catecholamine, umfutho wegazi ophakeme, ikhanda elibuhlungu, ukujuluka, isimila, uhlelo lwe-endocrine