Singathetha ngethumba elingaqhelekanga elibizwa ngokuba yiPheochromocytoma namhlanje?

Ngaba ngamanye amaxesha uba noxinzelelo lwegazi oluphezulu ngequbuliso? Okanye uyabila nje? Ngaba uphathwa yintloko ebuhlungu ngenxa yentliziyo ebetha ngamandla? Musa ukungxama ukucinga ukuba ezinye zezi zinto zisisifo esibi. Nangona kunjalo, ukuba ezi mpawu ziyaqhubeka, ingakumbi xa uxinzelelo lwegazi luphezulu kunzima ukululawula, licebo elihle ukuba ukhathazeke kancinci. Namhlanje siza kuthetha ngemeko engaqhelekanga, kodwa ukuba ifunyenwe ngokufanelekileyo, inokunyangeka kakuhle. Oko kubizwa ngokuba yi-pheochromocytoma.

Yintoni iPheochromocytoma? Masiyiqonde ngokulula.

Ngamafutshane, i-pheochromocytoma sisimila esikhula embindini we-adrenal glands, i-adrenal medulla. Ezi simila zenziwe luhlobo oluthile lweeseli ezibizwa ngokuba zii-chromaffin cells. Ezi seli zivelisa kwaye zikhuphe ii-hormone egazini ezifunekayo kwimpendulo yomzimba wethu "yokulwa okanye yokubaleka". Cinga ngako, xa ngequbuliso usoyika okanye ujongene noxinzelelo olukhulu, utshintsho olwenzekayo emzimbeni wakho - izinga lentliziyo yakho liyanyuka, ubila, umzimba wakho uyangcangcazela - ezi hormone zinoxanduva loku.

I-Pheochromocytoma idla ngokukhula kwi-adrenal gland enye kuphela. Nangona kunjalo, ngamanye amaxesha inokukhula kuzo zombini ii-gland. Kunokwenzeka ukuba kubekho ii-tumor ezingaphezu kwesinye kwi-gland enye.

Okubalulekileyo kukuba, uninzi lwee-pheochromocytomas azinabungozi (azinabungozi) . Oku kuthetha ukuba azisasazeki kwezinye iindawo zomzimba. Nangona kunjalo, phakathi kwe-10% kunye ne-15% zinokuba ngumhlaza. Ukuba le yimeko yomhlaza, ichazwa kwiindidi ezininzi eziphambili, kuxhomekeke kwindlela esasazeke ngayo:

• I-pheochromocytoma ekwindawo ethile: Ithumba lifumaneka kwi-adrenal gland enye okanye zombini.
• I-pheochromocytoma yengingqi: Umhlaza usasazeke kwi-lymph nodes kufutshane ne-adrenal glands okanye kwezinye izicubu.
• I-Metastatic pheochromocytoma: Umhlaza usasazeke kwiindawo ezikude ezifana nesibindi, imiphunga, okanye amathambo.
• I-pheochromocytoma ephindaphindayo: Umhlaza ubuyile emva konyango. Unokuba kwindawo efanayo naleyo wawuyiyo ngaphambili okanye kwenye indawo.

Zithini ezi glands ze-adrenal emizimbeni yethu? Yintoni umsebenzi wazo?

Sinezindlala ezimbini ze-adrenal. Zikwindawo engasemva kwesisu sethu, ngaphezu kwezintso zethu, njengesiciko. Ziyinxalenye yenkqubo yethu ye-endocrine. Indlala nganye ye-adrenal ineenxalenye ezimbini eziphambili. Umaleko wangaphandle ubizwa ngokuba yi-adrenal cortex, kwaye inxalenye ephakathi ibizwa ngokuba yi-adrenal medulla.

I-adrenal medulla yethu ivelisa iqela leehomoni ezibizwa ngokuba zii-catecholamines. Ezi homoni zilawula iinkqubo ezininzi ezibalulekileyo emzimbeni wethu:

• Inqanaba lentliziyo
• Ifuthe legazi
• Inqanaba leswekile egazini `(iglucose yegazi)`
• Indlela umzimba wethu osabela ngayo kuxinzelelo (impendulo "yokulwa okanye yokubaleka")

Iintlobo eziphambili ze-catecholamines zezi:

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

Xa kukho i-pheochromocytoma, inokukhupha ezi hormones, i-adrenaline kunye ne-noradrenaline, egazini kunokuba kuyimfuneko. Kulapho ke iimpawu ezahlukeneyo ziqala ukubonakala khona.

Yintoni umahluko phakathi kwePheochromocytoma kunye neParanglioma?

Ezi ziintlobo ezimbini ezifanayo kakhulu nezingaqhelekanga zeethumba. Zombini zivela kwiiseli ze-chromaffin ezifanayo esizixubushileyo ngaphambili. Nangona kunjalo, i-pheochromocytoma ivela kumbindi we-adrenal gland (i-adrenal medulla), ngelixa i-paraganglioma ivela kwezinye iindawo ezingaphandle kwe-adrenal gland .

Ngubani onokuthi abe nale meko? Ixhaphake kangakanani?

I-Pheochromocytoma inokukhula nangaliphi na ixesha, kodwa ixhaphake kakhulu kubantu abaphakathi kweminyaka engama-30 ukuya kwengama-50. Malunga ne-10% yamatyala axelwa ebuntwaneni.

Eli lithumba elingaqhelekanga kakhulu . Kunzima ukusho ngokuthe ngqo ukuba bangaphi abantu abanale meko. Ngenxa yokuba abanye abantu bengabonakalisi zimpawu, esi sifo sisenokungafunyaniswa. Kuqikelelwa ukuba ngaphantsi kwe-1% yabantu abanoxinzelelo lwegazi oluphezulu bane-pheochromocytoma.

Ziziphi iimpawu ezibonakaliswa ngumntu one-pheochromocytoma?

Iimpawu ze-Pheochromocytoma zenzeka xa ithumba likhupha iihomoni ezininzi ze-adrenaline (epinephrine) okanye i-noradrenaline (norepinephrine) egazini. Nangona kunjalo, ezinye iithumba azivelisi ezi homoni zininzi kwaye zisenokungabi nazimpawu.

Iimpawu ezibonakala kakhulu zezi:

• Uxinzelelo lwegazi oluphezulu (uxinzelelo lwegazi oluphezulu): Olu luphawu oluphambili. Ngamanye amaxesha kunzima ukululawula.
• Intloko ebuhlungu: Inokuba yintloko ebuhlungu kakhulu, ekhawulezileyo.
• Ukubila kakhulu ngaphandle kwesizathu.
• Ukubetha kwentliziyo ngokukhawuleza, ngokungangqalanga, okanye ukubetha kwentliziyo ngokukhawuleza.
• Ukuziva ngathi umzimba wakho uyangcangcazela.

Iimpawu ezingaqhelekanga kakhulu:

• Intlungu yesifuba kunye/okanye yesisu.
• Ulusu luba mhlophe ngequbuliso kunesiqhelo.
• Isicaphucaphu kunye/okanye ukuhlanza.
• Urhudo.
• Ukuqhinwa.
• I-Orthostatic hypotension kukwehla ngequbuliso koxinzelelo lwegazi xa usukuma. Oku kuthetha ukuba uziva unesizungu xa usukuma ngequbuliso uhleli.
• Ukunciphisa umzimba ngaphandle kwesizathu.

Ezi mpawu zinokubonakala okanye zibe mandundu emva kweziganeko ezithile. Umzekelo:

• Umsebenzi womzimba onzima.
• Ukulimala emzimbeni okanye uxinzelelo olukhulu lwengqondo.
• Ukuzala.
• Ukufumana i-anesthesia.
• Ukwenza utyando.
• Ukutya ukutya okutyebileyo kwi-tyramine (umz. iwayini ebomvu, itshokolethi, itshizi).

Ngaba iimpawu zihlala zikhona? Okanye ziyafika zidlule?

Umntu one-pheochromocytoma unokuba noxinzelelo lwegazi oluphezulu oluhlala luhleli, okanye lusenokufika luze luphele .

Abanye abantu banokufumana "ukuhlaselwa yi-paroxysmal," okanye ukuhlaselwa ngequbuliso kweempawu. Oku kuthetha ukuba kukho ukunyuka ngequbuliso koxinzelelo lwegazi, okuhamba neempawu ezifana nentloko ebuhlungu kakhulu, ukunyuka kwesantya sentliziyo, kunye nokubila okugqithisileyo. Ezi "hlaselo" zinokwenzeka amaxesha amaninzi ngemini, okanye kube kanye okanye kabini ngenyanga.

Kubalulekile: Ukuba unayo nayiphi na kwezi mpawu, ingakumbi uxinzelelo lwegazi oluphezulu ngequbuliso, intloko ebuhlungu, ukubila, kunye nokubetha kwentliziyo, kubaluleke kakhulu ukufuna ingcebiso kagqirha.

Kutheni i-pheochromocytoma ikhula? Zithini izizathu?

Kwiimeko ezininzi, akukho sizathu sithile sifumanekayo se-pheochromocytoma. Yenzeka ngokungacwangciswanga.

Nangona kunjalo, kwiimeko ezingama-25% ukuya kuma-35%, le meko inxulumene neemeko zofuzo. Ezinye zezona ziphambili zezi:

• I-Multiple endocrine neoplasia 2 syndrome, iintlobo A kunye no-B (MEN2A kunye neMEN2B)
• Isifo seVon Hippel-Lindau (VHL)
• Uhlobo lwe-Neurofibromatosis 1 (NF1)
• Isifo separaganglioma esililifa
• I-Carney-Stratakis dyad [i-paraganglioma kunye ne-gastrointestinal stromal tumor (GIST)]
• I-Carney triad (i-paraganglioma, i-GIST kunye ne-pulmonary chondroma)

Ukongeza kwezi meko zemfuza, i-pheochromocytoma inokukhula ngenxa yotshintsho kwizakhi zofuzo ezili-10 ezahlukeneyo.

Sifunyaniswa njani esi sifo? (Ukuxilongwa)

I-Pheochromocytoma inokuba nzima ukuyifumanisa kuba yithumba elingaqhelekanga kwaye ngamanye amaxesha alibangeli zimpawu. Ngamanye amaxesha, ithumba lifunyanwa ngengozi ngexesha lovavanyo olwenziwe ngesinye isizathu.

Kukho izizathu ezininzi zokuba ugqirha angarhanela esi sifo:

• EyakhoImbali yezonyango eneenkcukacha, ingakumbi ukuba kukho nabani na kusapho lwakhe owakha waba ne-pheochromocytoma ngaphambili.
• Uvavanyo olupheleleyo lomzimba nolwezonyango.
• Ezinye iimpawu ezithile , umzekelo "uhlaselo lwe-paroxysmal" esithethe ngalo kunye noxinzelelo lwegazi oluphezulu olungalawulwa ngonyango oluqhelekileyo.

Luhlobo luni lovavanyo oluqhutywayo?

Ugqirha wakho unokucebisa ezi mvavanyo zilandelayo ukuqinisekisa ukuba unayo i-pheochromocytoma:

• Uvavanyo lomchamo lweeyure ezingama-24: Oku kuquka ukuqokelela umchamo wakho imini yonke nokulinganisa ubungakanani be-catecholamines kuwo. Kukwalinganisa nezinto eziveliswayo xa ezi hormone ziwohloka. Ukuba la manqanaba aphezulu kunesiqhelo, inokuba luphawu lwe-pheochromocytoma.
• Uvavanyo lwe-catecholamine egazini: Olu vavanyo lulinganisa amanqanaba e-catecholamines egazini. Njengovavanyo lomchamo, lukwakhangela izinto eziveliswa kukuqhekeka kwee-hormone.
• I-CT scan (i-Computer Tomography scan): Oku kuthatha uthotho lwee-X-rays ukwenza imifanekiso eneenkcukacha zangaphakathi lomzimba wakho. Ugqirha wakho unokucebisa oku ukujonga amadlala akho e-adrenal.
• I-MRI scan (MRI - Magnetic Resonance Imaging): Oku kusebenzisa imagnethi, amaza erediyo, kunye nekhompyutha ukwenza imifanekiso eneenkcukacha zangaphakathi lomzimba. Ikwasetyenziselwa ukuhlola amadlala e-adrenal.

Nje ukuba isifo siqinisekiswe, kunokwenziwa olunye uvavanyo ukuze kuqinisekiswe ukuba ithumba linomhlaza (elibi) okanye alinabungozi (elibi), kwaye nokuba lisasazeke na kwamanye amalungu omzimba.

Kutheni Uvavanyo Lwemfuza Lubalulekile?

Ukuba ufunyaniswe une-pheochromocytoma, ugqirha wakho uya kucebisa ukuba ufumane iingcebiso ngemfuza kunye novavanyo ukuze kuqinisekiswe ukuba unesifo esizuzwe njengelifa esikubeka emngciphekweni wokufumana eminye imidlaza.

Uvavanyo lwe-genetic lunokucetyiswa kwiimeko ezifana nezi:

• Ukuba wena okanye umntu kusapho lwakho uneempawu ezinxulumene ne-pheochromocytoma okanye i-paraganglioma syndrome.
• Ukuba unezimila kuzo zombini ii-adrenal glands zakho.
• Ukuba kukho iithumba ezingaphezu kwesinye kwi-adrenal gland enye.
• Ukuba kukho iimpawu zokunyuka kwamanqanaba e-catecholamines egazini.
• Ukuba i-pheochromocytoma ifunyaniswa ingekafiki kwiminyaka engama-40.

Ukuba uvavanyo lwemfuza lufumanisa utshintsho lwemfuza, umcebisi wemfuza unokucebisa ukuba namanye amalungu osapho lwakho (abo bangenazo iimpawu kodwa abasengozini) nabo bavavanywe.

Ziziphi iindlela zonyango zePheochromocytoma?

Eyona ndlela ilungileyo yokunyanga oku kukususa ithumba ngotyando, ukuba kunokwenzeka .

Ukukhetha unyango kuxhomekeke kwizinto ezininzi:

• Ubungakanani bethumba.
• Nokuba ithumba linesifo somhlaza (elibi) okanye elingalunganga (elingenobungozi).
• Ingaba kukho iimpawu ezibangelwa kukwanda kweehomoni ze-catecholamine.
• Ingaba ithumba lihlala kwindawo enye, okanye lisasazeke kwamanye amalungu omzimba? (Lisasazekile)
• Nokuba esi sifo sifunyenwe okokuqala, okanye nokuba siphinde savela emva konyango lwangaphambili.

Ukuba uneempawu ezibangelwa kukwanda kweehomoni ze-adrenal, ugqirha wakho unokukunika amayeza okulawula ezo mpawu. Umzekelo:

• Amayeza okugcina uxinzelelo lwegazi lukwinqanaba eliqhelekileyo, afana ne-alpha-blockers.
• Amayeza okugcina isantya sentliziyo sikwinqanaba eliqhelekileyo, njengee-beta-blockers.
• Amayeza athintela imiphumo yeehomoni ezikhutshwa ngokugqithisileyo yi-adrenal gland.

Iindlela eziphambili zonyango lwe-pheochromocytoma zezi:

• Utyando
• Unyango ngemitha
• Unyango ngamakhemikhali
• Unyango lokukhupha isisu
• Unyango lwe-embolization
• Unyango olujoliswe kulo

Kunye, wena kunye neqela lakho lezonyango niya kugqiba isicwangciso sonyango esilungele wena.

Iindlela eziphambili zonyango

• Utyando:

Olu lolona nyango luphambili lwe-pheochromocytoma. Malunga ne-90% yeethumba zinokususwa ngempumelelo ngotyando .

Ugqirha wakho unokucebisa utyando lokususa idlala lakho le-adrenal okanye zombini (i-adrenalectomy). Ngexesha lotyando, ugqirha uza kuhlola izicwili ezijikelezileyo kunye nee-lymph nodes ukuze abone ukuba ithumba lisasazekile na. Ukuba lisasazekile, baza kulisusa elo candelo, ukuba kunokwenzeka.

Emva kotyando, igazi okanye umchamo wakho uza kuvavanywa ukuba unamanqanaba e-catecholamine. Ukuba amanqanaba abuyela esiqhelweni, oko kuthetha ukuba zonke iiseli ze-pheochromocytoma zisusiwe.

Ukuba zombini ii-adrenal glands zisusiwe, kuya kufuneka uthathe unyango lweehomoni ubomi bakho bonke ukuze kuthathelwe indawo iihomoni eziveliswa zii-adrenal glands.

• Unyango ngemitha:

Olu lunyango olusetyenziselwa ukubulala iiseli zomhlaza okanye ukuzithintela ukuba zingakhuli. Oku kwenziwa ngendlela engonakalisi izicubu eziphilileyo kangangoko kunokwenzeka.

Kukho iintlobo ezimbini zonyango lwe-radiation:

• Unyango lwemitha yangaphandle: Imitha ithunyelwa kwindawo yomhlaza isuka kumatshini ongaphandle komzimba.
• Unyango lwemitha yangaphakathi: Into enokusasazeka ngemitha ifakwa kwiinaliti, imbewu, iingcingo, okanye ii-catheters ize ifakelwe ngugqirha kwindawo okanye kufutshane nendawo yomhlaza.

Uhlobo lonyango lwemitha luxhomekeke ekubeni umhlaza ungowasekuhlaleni, ungowengingqi, usasazeka okanye uphindaphinda. I-pheochromocytoma ebulalayo idla ngokuphathwa ngonyango lwemitha yangaphandle kunye/okanye unyango lwe-131I-MIBG. I-131I-MIBG sisithako semitha esinamathela kwezinye iiseli zomhlaza size sizibulale.

• Unyango ngamakhemikhali:

Oku kusebenzisa amayeza ukubulala iiseli zomhlaza, ukuzithintela ukuba zingahlukani kwaye zingaphindaphindani, okanye ukunqanda ukukhula komhlaza. La mayeza adla ngokunikezelwa ngemithambo yegazi. Nangona olu lunyango oluphumelelayo, lunokuba nemiphumo emibi.

• Unyango lokukhupha isisu:

Olu lunyango olungenabungozi kangako. Lusebenzisa ubushushu obugqithisileyo okanye ukubanda kakhulu ukutshabalalisa amathumba.

• Ukususwa kwee-radiofrequency: Ukususwa kwee-radiofrequency kusebenzisa amaza erediyo ukufudumeza nokutshabalalisa iiseli zomhlaza kunye neeseli ezingaqhelekanga.
• I-Cryoablation: Isebenzisa i-nitrogen engamanzi okanye i-carbon dioxide engamanzi ukukhenkceza nokutshabalalisa iiseli zomhlaza kunye neeseli ezingaqhelekanga.

• Unyango lwe-Embolization:

Kule meko, umthambo ohambisa igazi kwi-adrenal gland uyavalwa. Oku kuyayekisa ukuhamba kwegazi ukuya kwi-gland, nto leyo ebulala iiseli zomhlaza ezikhula apho.

• Unyango olujoliswe kulo:

Oku kusebenzisa amayeza okanye ezinye izinto ezihlasela ngokukodwa iiseli zomhlaza kuphela ngaphandle kokulimaza iiseli eziphilileyo. Olu nyango lusetyenziselwa i-metastatic kunye ne-recurrent pheochromocytoma.

Kufundwa nge-tyrosine kinase inhibitor ebizwa ngokuba yi-sunitinib ukuze kufunyanwe i-metastatic pheochromocytoma. La mayeza athintela ukukhula kwethumba.

Ngaba esi sifo singathintelwa?

Ngelishwa, akukho ndlela yokuthintela i-pheochromocytoma. Nangona kunjalo, ukuba usemngciphekweni wokufumana esi sifo ngenxa yezifo ezibangelwa lufuzo kunye ne-genes, iingcebiso nge-genetic zinokukunceda ekuhlolweni nasekubonweni kwangethuba.

Ukuba kukho namalungu osapho lwakho asondeleyo (abantakwenu, abazali) abakhe baba ne-pheochromocytoma, okanye ukuba unesifo semfuza esifana neso besithetha ngaso ngaphambili (i-Multiple endocrine neoplasia 2 syndrome, isifo seVon Hippel-Lindau (VHL), isifo seNeurofibromatosis uhlobo 1 (NF1), isifo seHereditary paraganglioma, iCarney-Stratakis dyad, iCarney triad), kubaluleke kakhulu ukuthetha nogqirha wakho ngaso.

Lithini ithuba lokuchacha emva konyango? (Ingxelo yokuxela kwangaphambili)

Ithemba le-pheochromocytoma lihlala lilungile kakhulu ukuba linyangiwe.Sele sitshilo ukuba malunga ne-90% yeethumba zinokususwa ngempumelelo ngotyando.

Nangona kunjalo, ukuba ayinyangwa, le meko inokukhokelela kwiingxaki ezinzulu, ezisongela ubomi . Umzekelo:

• I-Cardiomyopathy
• I-Myocarditis
• Ukopha okungalawulekiyo engqondweni (Ukopha ebuchosheni)
• Ukuqokelelana kolwelo emiphungeni (i-Pulmonary edema)

Abanye abantu abane-pheochromocytoma nabo basengozini yokuba nesifo sohlangothi okanye isifo sentliziyo.

Ndifanele ndimbone nini ugqirha?

• Ukuba ufunyaniswe une-pheochromocytoma kwaye ubonakalise naziphi na iimpawu ezikukhathazayo, bona ugqirha wakho ngoko nangoko.
• Ukuba uneempawu ze-pheochromocytoma, ezinje ngoxinzelelo lwegazi oluphezulu kunye nentloko ebuhlungu, thetha nogqirha wakho. Nangona i-pheochromocytoma ingaqhelekanga, kubalulekile ukunyanga uxinzelelo lwegazi oluphezulu.
• Ukuba uyazi ukuba omnye wezalamane zakho ezisondeleyo (abantakwenu, abazali) unesifo semfuza esifana nesifo se-'Multiple endocrine neoplasia 2' okanye isifo se-'Von Hippel-Lindau (VHL), usenokuba semngciphekweni omkhulu wokufumana i-pheochromocytoma, ngoko ke bonana nogqirha ukuze bathethe ngovavanyo lwemfuza.

Ndingayibuza yiphi imibuzo ugqirha?

Ukuba ufunyaniswa une-pheochromocytoma, kunokuba luncedo ukubuza ugqirha wakho le mibuzo:

• Kutheni ndifumene i-pheochromocytoma?
• Ngaba abantwana bam kunye/okanye izalamane zam zinokuba ne-pheochromocytoma?
• Ziziphi iindlela zonyango endinazo?
• Zithini iziphumo ebezingalindelekanga zonyango olwahlukeneyo?
• Ndingazilawula njani iimpawu zam?

Okokugqibela, ezona zinto zibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngoko ke, nangona i-pheochromocytoma sisimila esingaqhelekanga, sidla ngokuba singenabungozi kwaye siyanyangeka . Kwakhona, sinokuhamba kwiintsapho, ngoko ke ukuba wena okanye umntu kusapho lwakho ufunyaniswe enale meko, kubalulekile ukuba kwenziwe uvavanyo lwemfuza. Oku kunokunceda ekuqinisekiseni ukuba usemngciphekweni wezinye iingxaki zempilo.

Khumbula, ukuba unemibuzo malunga nomngcipheko wakho wokuba ne-pheochromocytoma okanye ngesi sifo, ungoyiki ukuthetha nogqirha wakho. Balapha ukukunceda. Hlala usempilweni!

I-Pheochromocytoma , i-adrenal gland, ii-catecholamines, uxinzelelo lwegazi oluphezulu, intloko ebuhlungu, ukubila, ithumba, inkqubo ye-endocrine