Ntau tus kab mob endocrine neoplasia (MEN) II

Ntau tus kab mob endocrine neoplasia, hom II (MEN II) yog ib qho kev tsis sib haum xeeb dhau los ntawm cov tsev neeg nyob rau hauv uas ib lossis ntau dua ntawm cov qog endocrine txuas dhau los yog tsim lub qog. Cov qog endocrine feem ntau muaj xws li:

• Cov qog adrenal (kwv yees li ib nrab ntawm lub sijhawm)
• Parathyroid caj pas (20% ntawm lub sijhawm)
• Mob qog (yuav luag txhua lub sijhawm)

Ntau tus kab mob endocrine neoplasia (MEN I) yog ib yam mob uas cuam tshuam.

Qhov ua rau MEN II yog ib qho kev tsis txaus ntseeg ntawm cov gene hu ua RET. Qhov kev tsis zoo no ua rau ntau cov qog tshwm sim hauv tib tus neeg, tab sis tsis tas yuav nyob rau tib lub sijhawm.

Kev koom tes ntawm lub qog adrenal yog feem ntau nrog cov qog hu ua pheochromocytoma.

Kev koom tes ntawm cov ua cov thyroid caj dab feem ntau nrog cov qog hu ua medullary carcinoma ntawm cov thyroid.

Cov hlav nyob rau hauv lub qog, adrenal, lossis parathyroid qog yuav tshwm sim ntau xyoo sib txawv.

Qhov tshwm sim no yuav tshwm sim thaum muaj hnub nyoog, thiab cuam tshuam rau txiv neej thiab poj niam sib npaug. Qhov kev pheej hmoo tseem ceeb yog tsev neeg keeb kwm ntawm MEN II.

Muaj ob qhov subtypes ntawm MEN II. Lawv yog MEN IIa thiab IIb. MEN IIb feem ntau tsis tshua pom.

Cov tsos mob yuav txawv. Txawm li cas los xij, lawv zoo ib yam li cov ntawm no:

• Lub cev nqaij daim tawv mob cancer ntawm lub qog
• Pheochromocytoma
• Parathyroid adenoma
• Parathyroid hyperplasia

Txhawm rau kuaj xyuas tus mob no, tus kws saib xyuas kev noj qab haus huv nrhiav kev hloov mus rau hauv cov RET gene. Qhov no tuaj yeem ua tau los ntawm kev kuaj ntshav. Cov kev ntsuam xyuas ntxiv tau ua tiav los txiav txim siab cov tshuaj hormones twg tseem ceeb dhau los.

Kev kuaj mob lub cev yuav qhia:

• Ntug loj lymph node nyob rau hauv lub caj dab
• Ua npaws
• Ntshav siab
• Lub plawv dhia nrawm
• Lub qog nodules

Kev ntsuam xyuas duab uas siv los txheeb cov qog yuav suav nrog:

• Mob plab hauv TV
• Kev ua haujlwm ntawm lub raum lossis ureters
• MIBG scintiscan
• MRI ntawm lub plab mog
• Lub qog kuaj ntshav
• Ultrasound ntawm lub qog

Kev kuaj ntshav yog siv los xyuas seb qee lub caj pas hauv lub cev ua haujlwm zoo npaum li cas. Lawv suav nrog:

• Qib Calcitonin qib
• Ntshav phwj hauv alkaline phosphatase
• Ntshav calcium
• Ntshav parathyroid theem
• Ntshav phosphorus
• Zis catecholamines
• Zis metanephrine

Lwm yam kev ntsuam xyuas lossis cov txheej txheem uas yuav ua tau muaj xws li:

• Adrenal kuaj mob
• Electrocardiogram (ECG)
• Lub qog ua ntshav

Kev phais mob yog qhov xav tau kom tshem tawm pheochromocytoma, uas tuaj yeem tsim kev phom sij rau lub neej vim yog cov tshuaj hormones nws ua.

Rau mob nqaij hlav hauv koj lub qog, cov thyroid caj pas thiab cov qog ntshav yuav tsum muab tshem tawm tag nrho. Kev hloov kho cov thyroid hormones txaus yog muab tom qab kev phais mob.

Yog paub tias menyuam yaus nqa tus mob RET hloov mus rau lwm tus, phais yuav tshem tawm cov qog ua ntej nws yuav mob qog nqaij hlav. Qhov no yuav tsum tau sab laj nrog tus kws kho mob paub txog tus mob no. Nws yuav ua rau thaum me nyuam yaus (ua ntej hnub nyoog 5) hauv cov neeg paub MEN IIa, thiab ua ntej hnub nyoog 6 lub hlis rau cov neeg mob MEN IIb.

Pheochromocytoma feem ntau tsis mob qog noj ntshav (benign). Kev mob kheesxaws hauv lub cev cov thyroid yog qhov muaj mob qog noj ntshav thiab yuav ua rau neeg tuag taus, tab sis kev kuaj mob ntxov thiab phais mob feem ntau tuaj yeem kho tau. Kev phais tsis kho tus pib MEN II.

Kev sib kis ntawm cov qog hlwb yog qhov ua kom muaj teeb meem tshwm sim.

Hu rau koj tus kws kho mob yog tias koj pom muaj mob MEN II lossis yog ib tus neeg hauv koj tsev neeg tau txais kev kuaj mob.

Kev txheeb ze cov neeg txheeb ze ntawm cov neeg muaj MEN II yuav ua rau tus neeg muaj mob xiam hlwb thaum ntxov thiab paub txog. Qhov no yuav tso cai rau cov qib ua los tiv thaiv cov teeb meem.

Sipple syndrome; TXIV NEEJ II; Pheochromocytoma - MEN II; Mob qog noj ntshav - pheochromocytoma; Parathyroid mob cancer - pheochromocytoma

• Cov qog endocrine

Lub Tebchaws National Comprehensive Cancer Network lub vev xaib. Cov chaw txhaj tshuaj xyaum ua raws li oncology (NCCN kev qhia): neuroendocrine hlav. Version 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Hloov kho thaum Lub Peb Hlis 5, 2019. Nkag mus rau Lub Peb Hlis 8, 2020.

Newey PJ, Thakker RV. Ntau tus kab mob endocrine neoplasia. Hauv: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Phau Ntawv ntawm EndocrinologyCov. Xab 14th. Philadelphia, PA: Elsevier; 2020: cha 42.

Nieman LK, Spiegel AM. Cov teeb meem polyglandular. Hauv: Goldman L, Schafer AI, eds. Goldman-Cecil TshuajCov. 26th ed. Philadelphia, PA: Elsevier; 2020: cha 218.

Tacon LJ, Learoyd DL, Robinson BG. Ntau yam tshuaj endocrine neoplasia yam 2 thiab mob qog nqaij hlav cancer. Hauv: Jameson JL, De Groot LJ, de Kretser DM, li al, eds. Endocrinology: Cov Neeg Loj thiab PediatricCov. 7 tus ed. Philadelphia, PA: Elsevier Saunders; 2016: cha 149.