Ama-endocrine neoplasia amaningi (AMADODA) II

I-endocrine neoplasia eminingi, uhlobo II (MEN II) yisifo esidluliswa emindenini lapho eyodwa noma eziningi zezindlala ze-endocrine zisebenza ngokweqile noma zakha isimila. Izindlala ze-Endocrine ezivame ukubandakanyeka zifaka:

• Indlala ye-Adrenal (cishe isigamu sesikhathi)
• Indlala yeParathyroid (20% yesikhathi)
• Indlala yegilo (cishe sonke isikhathi)

I-endocrine neoplasia eminingi (MEN I) yisimo esihlobene.

Imbangela ye-MEN II ukukhubazeka kufuzo olubizwa nge-RET. Lokhu kukhubazeka kubangela ukuthi izimila eziningi zivele kumuntu ofanayo, kodwa hhayi ngasikhathi sinye.

Ukubandakanywa kwendlala ye-adrenal kuvame kakhulu ukuba nesimila esibizwa nge-pheochromocytoma.

Ukubandakanywa kwendlala yegilo kuvame kakhulu ukuba nesimila esibizwa ngokuthi yi-medullary carcinoma yegilo.

Amaqhubu endlala yegilo, ye-adrenal, noma ye-parathyroid angenzeka ahlukane iminyaka.

Lesi sifo singavela nganoma yisiphi isikhathi, futhi sithinta abesilisa nabesifazane ngokulinganayo. Isici esiyingozi enkulu umlando womndeni we-MEN II.

Kunezinhlobo ezimbili ezingaphansi kwe-MEN II. Bangama-MEN IIa no-IIb. AMADODA IIb ajwayelekile kakhulu.

Izimpawu zingahluka. Noma kunjalo, ziyefana nalezi ze:

• I-medullary carcinoma yegilo
• Pheochromocytoma
• Parathyroid adenoma
• I-Parathyroid hyperplasia

Ukuthola lesi simo, umhlinzeki wezokunakekelwa kwezempilo ubheka ushintsho kuhlobo lwe-RET. Lokhu kungenziwa ngokuhlolwa kwegazi. Ukuhlolwa okungeziwe kuyenziwa ukuthola ukuthi yimaphi ama-hormone akhiqizwa ngokweqile.

Ukuhlolwa komzimba kungaveza:

• Ama-lymph node akhulisiwe entanyeni
• Imfiva
• Umfutho wegazi ophezulu
• Ukushaya kwenhliziyo okusheshayo
• Amaqhubu endlala yegilo

Ukuhlola ukuhlolwa okusetshenziselwa ukukhomba izicubu kungafaka:

• Iskena se-CT esiswini
• Ukufanekisa izinso noma ureters
• I-MIBG scintiscan
• I-MRI yesisu
• Iskena sendlala yegilo
• I-Ultrasound yegilo

Ukuhlolwa kwegazi kusetshenziselwa ukubona ukuthi izindlala ezithile emzimbeni zisebenza kahle kangakanani. Zingafaka:

• Izinga leCalcitonin
• I-alkaline phosphatase ene-alkaline
• I-calcium yegazi
• Izinga lama-hormone e-parathyroid
• I-phosphorus yegazi
• Amakhemikhali e-urine
• Umchamo metanephrine

Olunye uvivinyo noma izinqubo ezingenziwa zifaka:

• I-adrenal biopsy
• I-Electrocardiogram (ECG)
• I-biopsy yegilo

Ukuhlinzwa kuyadingeka ukukhipha i-pheochromocytoma, engabeka impilo yakho engozini ngenxa yamahomoni eyenzayo.

Kwi-medullary carcinoma yegilo, indlala yegilo kanye nama-lymph node azungezile kufanele asuswe ngokuphelele. Ukwelashwa kwehomoni ye-thyroid kunikezwa ngemuva kokuhlinzwa.

Uma ingane yaziwa ukuthi ithwala ukuguqulwa kwezakhi zofuzo i-RET, ukuhlinzekwa ukususa i-thyroid ngaphambi kokuba kube nomdlavuza kubhekwa. Lokhu kufanele kuxoxwe nodokotela ojwayelene nalesi simo. Lokhu kuzokwenziwa esemncane (ngaphambi kweminyaka yobudala engu-5) kubantu abane-MEN IIa eyaziwa, nangaphambi kwezinyanga eziyisithupha kubantu abane-MEN IIb.

I-Pheochromocytoma imvamisa ayisiwo umdlavuza (ubungozi). I-medullary carcinoma yegilo ngumdlavuza onolaka kakhulu futhi ongabulala, kepha ukuxilongwa nokuhlinzwa kusenesikhathi kungaholela ekwelapheni. Ukuhlinzwa akuyelaphi i-MEN II engaphansi.

Ukusabalala kwamangqamuzana anomdlavuza kuyinkinga engenzeka.

Shayela umhlinzeki wakho uma ubona izimpawu ze-MEN II noma uma othile emndenini wakho ethola ukuxilongwa okunjalo.

Ukuhlola izihlobo eziseduze zabantu abane-MEN II kungaholela ekutholeni i-syndrome ngokushesha nangomdlavuza ohlobene nayo. Lokhu kungavumela izinyathelo zokuvimbela izinkinga.

Isifo se-Sipple; AMADODA II; I-Pheochromocytoma - AMADODA II; Umdlavuza wegilo - pheochromocytoma; Umdlavuza we-Parathyroid - i-pheochromocytoma

• Izindlala ze-Endocrine

Iwebhusayithi ye-National Comprehensive Cancer Network. Imihlahlandlela yokusebenza kwemitholampilo ku-oncology (imihlahlandlela ye-NCCN): izicubu ze-neuroendocrine. Inguqulo 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Kubuyekezwe ngoMashi 5, 2019. Kufinyelelwe ngoMashi 8, 2020.

UNewey PJ, uThakker RV. Ama-neoplasia amaningi we-endocrine. Ku: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, abahleli. Incwadi kaWilliams ye-Endocrinology. Umhlaka 14. IPhiladelphia, PA: Elsevier; 2020: isahluko 42.

UNieman LK, uSpiegel AM. Ukuphazamiseka kwePolyglandular. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 218.

UTacon LJ, uLearoyd DL, uRobinson BG. Izinhlobo eziningi ze-endocrine neoplasia type 2 kanye ne-medullary thyroid carcinoma. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, abahleli. I-Endocrinology: Eyabantu Abadala Neyengane. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 149.