Uma utshelwe ukuthi une-syndrome ye-endocrine neoplasia (MEN) eminingi, uyazi ukuthi kunokuhlolwa okuphelele nokuphathwa kwalesi simo esingavamile.
Incazelo ye-MEN
I-endocrine neoplasia eminingi (i-MEN) yisifo sezokwelapha esibonakala ngokuba nesisindo esingaphezu kwesisodwa sezingxenye ze-endocrine ngesikhathi esisodwa. Kunezinhlanganisela eziningana ezihlukahlukene zezicubu ze-endocrine eziyaziwayo ukuthi zenzeka ndawonye, futhi ngayinye yalezi zibonelo zihlukaniswa njengenye yezinkinga ezihlukahlukene (MEN).
Amathumba ahlakulela njengengxenye yesifo ngasinye se-MEN angase abe yinhlanhla noma ayimangalisa. Izicubu ze-Benign ziyi-tumor ezizimele ezingabangela izimpawu zezokwelapha kepha zikhule kancane, zingasabalali kwezinye izingxenye zomzimba futhi azibulali. Ngakolunye uhlangothi, izicubu ezinomdlavuza zingakwazi ukukhula ngokushesha, zingasakazeka kwezinye izingxenye zomzimba, futhi zingaba zibulalayo uma zishiywa zingaphathwa kabi.
Ama-syndromes
Kunezinhlobo ezintathu ze-syndromes ezibhekwa njengama-syndromes ajwayelekile kakhulu e-MEN. I-syndrome ngayinye ibangelwa ukungajwayelekile kwezesayensi, okusho ukuthi inhlanganisela ye-tumor isebenza emindenini njengesimo sokufa. I-syndromes ye-MEN ibizwa ngokuthi i-MEN 1, i-MEN 2A, ne-MEN 2B.
ABANTU 1: Abantu abaxilongwe nge-MEN 1 banezinambuzane ze-pituitary gland, i-parathyroid gland, nama-pancreas. Ngokuvamile, lezi zicubu ziyingozi, nakuba kungenakwenzeka ukuba zibe zimbi.
Amadoda 1 Izimpawu: Izimpawu ze-MEN 1 zingaqala ngesikhathi sokukhula noma ngesikhathi somuntu omdala.
Izimpawu ngokwazo ziguquguquka ngoba izicubu zibandakanya izitho ze-endocrine ezingakwazi ukuveza ububanzi bomphumela emzimbeni. I-tumor ngayinye ngayinye idala izinguquko ezingavamile ezihlobene nokusebenza ngokweqile kwe-hormonal.
- Amathumba e-pituitary abangela ukukhanda ikhanda , ukwehluleka kokuya esikhathini, ukungabi namntwana, ukukhiqiza ubisi lwebele, nokwehla kwamathambo.
- Amathumba e-Pancreatic enza ubuhlungu besisu, ukuhlanza, isifo sohudo, nezilonda.
- Izicubu ze-parathyroid zibangelwa amazinga aphezulu e-calcium, okungabangela ukuba izinso zezinso zibe uphawu lokuqala olubonakalayo.
ABANTU 2A: Abantu abane-MEN 2 banezicubu ze-thyroid, izicubu ze-adrenal gland, nezicubu ze-parathyroid.
AMANZI 2A Izimpawu: Izimpawu ze-MEN 2A ziqala ekukhuleni; ngokuvamile uma umuntu esendaweni yakhe engu-30. Njengawo amanye ama-syndromes ama-MEN, izimpawu zibangelwa ukungasebenzi kwamathumba e-endocrine.
- Izicubu ze-thyroid zivame ukuqala izicubu ezibonakalayo ku-MEN 2A, futhi zingabangela ukuvuvukala noma ukucindezela esifundeni sezintamo, futhi zingase zibe zimbi.
- I-adrenal gland tumors, ebizwa nge- pheochromocytoma , ihilela ngokukhethekile isigaba se-adrenal gland okuthiwa i-adrenal medulla. I-Pheochromocytoma ingabangela ukucindezela kwegazi eliphezulu, izinga lokushaya kwenhliziyo ngokushesha, nokujuluka.
- Amathumba e-parathyroid angabangela amazinga aphezulu e-calcium, futhi izimpawu zingase zihlanganise ukoma ngokweqile nokuvuthwa njalo.
MEN 2B: Lokhu kuyinto engavamile kakhulu kulezi zindlela zokungahambisani nesisu futhi kubonakala ngendlala ye-thyroid, i-adrenal gland tumor, neuromas (ama-cysts) kuwo wonke umlomo kanye nesistimu yokugaya umzimba, ukungavamile kwesakhiwo samathambo, nesimo esingavamile nesiguli esichazwe ngokuthi i-marfanoid izici.
AMANZI 2B Izimpawu: Izimpawu zingase ziqale ebuntwaneni, ngokuvamile ngaphambi kweminyaka eyi-10. Uphawu olucacile kakhulu lwe-MEN 2B lubukeka obude, obubonakalayo. I-Neuromas ibonakala njengezikhukhula ngaphakathi nangaphakathi komlomo, futhi abantu abane-MEN 2B bangase babe nezinkinga zesisu nokugaya, ngaphezu kwezimpawu zomdlavuza wegciwane nepheochromocytoma.
Ukuxilongwa
Udokotela wakho uzokhathazeka ukuthi ungaba nesifo se-MEN esekelwe iphethini le-tumor endocrine engaphezu kweyodwa, kanye nomlando womndeni wakho. Awudingi ukuthi ube nazo zonke izicubu zesignesha zenye ye-MEN syndromes kudokotela wakho ukuze ucabangele ama-MEN. Uma une-tumor engaphezu kwesisodwa noma isici, noma noma ngabe une-tumor endocrine eyodwa ehlotshaniswa nama-MEN, udokotela wakho angase akuhlole ezinye izicubu ngaphambi kokuba abe nezimpawu.
Ngokufanayo, umlando womndeni awudingekile ukuba abantu baxilongwe, ngoba umuntu angaba ngowokuqala emndenini ukuthi abe nesifo. Izakhi zofuzo ezibangela abakwa-MEN ziye zabonakala, futhi ukuhlolwa kofuzo kungase kube yindlela yokukhetha ukuhlolwa.
Kubuye kube nokuhlukahluka kwezinga le-syndrome ngayinye ye-MEN-ungase ube nama-MEN ngisho noma ungahambisani kahle neyodwa yamaphethini evamile.
Ukwelapha
Ukwelashwa kwawo wonke ama-syndromes e-MEN kuncike ezintweni ezimbalwa ezihlukile. Akuwona wonke umuntu otholakala ene-MEN 1, MEN 2A, noma i-MEN 2B ehlangabezana nesifundo esifanayo esifanayo nesifo. Ngokuvamile, ukwelashwa kugxile ezintweni ezintathu eziyinhloko, okufaka ukunciphisa izimpawu, ukutholakala kwezicubu ekuqaleni, nokuvimbela imiphumela yezifo ezimbi.
Uma unayo izimpawu ezibangelwa ukungahambi kahle kwe-hormonal, cishe uzodinga ukwelashwa kwalezo zimpawu. Izifo ze-endocrine zivame ukuguquguquka ngokuhamba kwesikhathi, ngoba izitho ze-endocrine zisebenza ngokusekelwe kwesimo esiyinkimbinkimbi sempendulo emihle nebizayo. Lokhu kusho ukuthi ama-hormone asebenza ngokweqile angaqeda ukucindezela noma ukukhuthaza ukukhiqizwa kwamanye ama-hormone emzimbeni. Ngakho-ke, ngisho noma uthola ukwelashwa kwama-hormone okwesikhashana, ungase udinge ukwelashwa okuhlukile noma ukulungiswa kwesilinganiso sakho esikhathini esizayo.
Ezinye izici ezibalulekile zokwelashwa kwe-MEN zibandakanya ukuqapha ngokucophelela nokuqapha ukukhomba izicubu ezintsha futhi ukubona noma yikuphi ukulimala ngokushesha ngangokunokwenzeka. Izicubu ezimbi zingadinga ukwelashwa ngokuhlinzwa noma chemotherapy ngaphambi kokuba zenze izinkinga zempilo ezingaphezulu.
Izwi elivela
Noma yiluphi uhlobo lwe-tumor lungesabeka. Futhi ukuba ne-tumor engaphezu kweyodwa kuyesabisa nakakhulu. Uma utshelwe ukuthi unayo noma ungase ube neoplasia ye-endocrine eminingi, kungenzeka ukuthi ukhathazeke kakhulu ngokuthi yiziphi izimpawu ezingaphuma ngokulandelayo nokuthi ngabe impilo yakho yonke isengozini.
Iqiniso lokuthi ama-syndromes e-MEN ahlonishwa futhi ahlukaniswa enza isimo sakho sibikezeleke nakakhulu kunalokho esingase sibonakale. Naphezu kokuthi lezi zingama-syndromes ezingavamile, ziye zachazwa ngokuningiliziwe futhi kunezindlela eziqinisekisiwe zokuphatha lezi zifo. Ngenkathi ngokuqinisekile uzodinga ukulandelwa kwezokwelapha okuqhubekayo, kunezindlela eziphumelelayo zokulawula isimo sakho futhi ungaqiniseka ngokuphelele ukuthi ngokunakekelwa kwezempilo okusheshayo, ungaba nomphumela onempilo.
> Umthombo:
> Wasserman JD, Tomlinson GE, uDruker H, et al. I-Endocrine Neoplasia ne-Hyperparathyroid-I-Jaw Syndromes Ye-Tumor: Izici Zemitholampilo, I-Genetics, Nezincomo Ze-Surveillance ebuntwaneni. I-Clin Cancer Res . 2017 Jul 1; 23 (13): e123-e132.