I chhungkuaah hian colon cancer vei an awm em? Lo kal teh, he genetic condition (Familial Adenomatous Polyposis - FAP) hi han sawi teh ang!

I chhungkuaah hian, kum naupan lai pawh ni se, colon cancer vei an awm em? A nih loh leh doctor-in i colon-ah hian pum natna nen hian thil lian tham (polyps) te te i nei tih a hrilh che em? Chutiang thil chu i hre tawh emaw hriat loh emaw pawh nise, vawiin a kan sawi tur `FAP` an tih dinhmun hi hriat chian a pawimawh hle thei a ni. A chhan chu tlem a tlem hle nachungin, hriat hmasak a nih chuan harsatna lian tak tak a chhanhim thei che a ni.

A awlsam zawngin FAP chu eng nge ni?

A awlsam zawngin, Familial Adenomatous Polyposis, a tawi zawngin FAP tih hi genetic condition a ni a, chu chuan i colon-ah leh a chang chuan i rectum chhungah adenomas an tih, cancer lo ni thei, a lo piang chhuak tenau (polyps) tam tak a siam a ni.

Ngaihtuah teh, mi thenkhat chuan an upat deuh deuh chuan an colon-ah heng tumor pakhat emaw pahnih emaw an nei thin. Chu chu thil pangngai a ni. Mahse `FAP` vei tan chuan heng tumor hi za aia tam, a chang chuan sang tam tak an nei tlangpui a , mahse kum naupang tak atang hian an lo thang tan a, kum sawm mi lek pawh a ni mai thei. Chuvangin, heng tumor zinga pakhat hi cancer (`colorectal cancer`)-ah a chan theihna chance a sang hle a, chu chu dam chhunga a hlauhawmna a ni .

He hlauhawmna hi thunun theih nan doctor-te chuan colon pumpui (total colectomy) chhuah vek tura operation neih an rawt tlangpui. A chang chuan, rectum pawh lakchhuah a ni (proctocolectomy). Hei hi a chhan chu FAP-ah hian heng tumor te hi a thang chak lutuk a, pakhat hnu pakhata lakchhuah a control theih loh vang a ni. Dik tak chuan, he surgery hi tih a nih loh chuan FAP vei tam tak chuan kum chanve velah cancer an vei ang .

FAP vei te hian tumor chi dang an nei thei a, chu chu colon chhungah chauh ni lovin hmun dangah te, vun, soft tissue, ha, leh ruh te pawh a ni thei. Colon lakchhuah a nih hnuah pawh heng tumor dangte enfiah nan screening neih chhunzawm a ngai mai thei a, chutiang bawkin operation pawh i neih a ngai mai thei bawk.

FAP vei tan chuan cancer vei theihna chance eng nge ni?

Enkawl loh chuan FAP vei chu colon cancer vei theihna chance 100% vel a ni . Tin, midang aiin kum naupang zawkin cancer hi an vei thei bawk. Chhungkaw member pakhatin he natna hi a neih chuan an fate chu kum 10 atanga tan kum tin colonoscopy an nei tur a ni .

Colon cancer bakah hian FAP vei te hi cancer chi dang vei theihna hlauhawm tak an ni:

• Duodenal cancer - 8% vel a ni.
• Papillary thyroid cancer - 2% vel a ni.
• Pancreatic cancer - 2% vel a ni.
• Liver cancer ``Hepatoblastoma`` an tih (a bik takin naupangte tan) - 1.5% vel
• Pum cancer - 1% vel a ni.
• Thluak leh ruhroah tumor - 1% aia tlem

FAP chi hrang hrang a awm em?

Ni e, `FAP` chi khat leh chu bakah chuan subtype dang engemaw zat a awm bawk.

• "Classic" FAP: Hei hi colon chhunga adenoma 100 chuang awm vang a ni.
• "Attenuated" FAP (AFAP): Hei hi subtype na deuh deuh a ni. Colon chhungah hian cyst 20 leh 100 inkar a awm a.
• Gardner syndrome: Classic `FAP` ang bawkin colon chhungah hian tumor 100 chuang a awm a. Tin, taksa hmun dangah hian tumor chi dang a lo awm bawk.
• Turcot syndrome: Cancer natna hi thluaka intestine-a tumor tam tak awm rualin a lo awm thin.

Hetiang dinhmun hi engtiang chiahin nge FAP tia koh a nih?

FAP hi a awm lo hle a ni . Mi 8,000 zinga pakhat velin a nghawng a ni. FAP hian colon cancer zawng zawng zinga 0.5% vel a huam a ni. Subtype AFAP, Gardner syndrome, leh Turcotte syndrome te pawh hi a tlem zawk hle.

FAP leh Lynch syndrome hi eng nge an danglamna?

Lynch syndrome hi hereditary condition dang a ni a, colon cancer leh cancer dang vei theihna a tipung a ni. Lynch syndrome hi HNPCC (hereditary nonpolyposis colorectal cancer syndrome) an ti bawk. A hming ang bawkin, hei hian colon tumor tam tak a lo awm tihna a ni hauh lo .

Lynch syndrome vei te hian colon-a tumor pakhat emaw a aia tam emaw awm pawhin cancer an vei thei a ni. Tin, tumor leh cancer te hi FAP aiin a tlai deuh a, a hlauhawmna pawh a hniam deuh bawk. Heng dinhmun pahnih hi gene mutation hrang hrang avanga lo awm a ni .

FAP natna lan chhuahna chu engte nge ni? Engtin nge kan hriat chian?

FAP-ah chuan colon polyp hi mipui zawng zawng aiin a lo piang hma hle a, a tam zawk chu tleirawl lai a ni . Heng polyp te hian hlauhawm taka an lian hma chuan symptoms an nei lo mai thei . Mahse colonoscopy i neih chuan i doctor chuan a hmu thei ang.

`FAP` vei te hian an colon ah polyp za tam tak emaw sang tam tak emaw an nei thei . `AFAP` vei te hian 20 tal an nei a, `FAP` ah hian polyp hi a tam zawk a, a thang chak zawk avangin polyp aiin symptoms a thlen tam zawk. A lan chhuah dan chu:

• Rectal atanga thisen chhuak
• Kawthalo
• Chronic pum na

FAP vei te hian pawn lam atanga doctor-te hriat theih tur symptom an nei thei a:

• Dermatofibromas: Vun hnuaia fibrous, scar ang chi hring hring.
• Epidermal cysts: Keratin-a khat, vun hnuaia awm, zungbun zungbun ang maia awm.
• Osteomas: Cancer ni lo tumor, ruh chhunga lo awm. Jawbone emaw luruh emaw-ah an awm tam ber.
• Ha dang emaw, ha impacted emaw:Hengte hi ha X-ray-ah hmuh theih a ni.
• Congenital hypertrophy of the retinal pigment epithelium (CHRPE): Hengte hi mit enfiahnaah hmuh theih a ni. Mahse, a tlangpuiin mit lama harsatna an thlen lo.

Kum engzat nge FAP symptoms hi a intan tlangpui?

FAP-ah chuan colon hi kum 16 vel atanga tan a ni tlangpui a , AFAP-ah chuan a hnu deuh deuhin a intan thei. I nu leh pa leh doctor ten he natna hi i hlauhawm tih an hriat chuan kum 10 i tlin hnuah an test tan mai thei che a, i test loh chuan i symptoms avang hian he natna hi i nei tih hmuhchhuah a ni thei bawk.

FAP awm chhan ber chu eng nge ni?

FAP thlentu ber chu gene mutation vang a ni . He gene hi adenomatous polyposis coli (APC) gene an ti a. Tumor suppressor gene an ti bawk. Chumi awmzia chu he gene hian cell te chu control theih loha lo thang lianin tumor siam turin a veng tihna a ni. Gene mutation a awm chuan he gene hnathawh hi a tibuai a ni.

FAP thlentu gene mutation hi germline mutation a ni. Hei hian dam chhunga thil thleng ni lovin, nau pai lai thil a ni tihna a ni . Hei hi thlah khat atanga thlah khata kal zel thil a ni. I nu leh pa zinga pakhatin he mutation hi a neih chuan 50% in rochun theihna chance i nei a ni . Mahse, heng mutations zinga 30% vel chu thil thar (original mutations) a ni a, chu chu rochun a ni lo tihna a ni . Chuvangin FAP vei hmuhchhuah zinga 30% vel chuan chhungkaw history-ah he natna hi an nei lo thei a ni.

FAP avanga harsatna awm thei te chu engte nge ni?

FAP ah hian complication enkawl tur pawimawh ber chu colon cancer a ni . I colon (colectomy) lakchhuahna tur operation (colectomy) hian he hlauhawmna hi nasa takin a tihziaawm thei a ni. Mahse, colon tel lova nun hian i nunah nghawng engemaw zat a nei thei a, a chhan chu i bawlhhlawh kal dan a inthlak danglam thin. Colon ai chuan ileostomy pouch emaw ileal pouch emaw hman a ngai mai thei.

FAP thlentu gene mutation chu i taksa cell tinah a awm avangin i taksa khawi hmunah pawh tumor a lo awm thei a ni . Tumor dang pawh colon pawnah a lo awm thei a, a chang chuan cancer a ni thei bawk. I doctor chuan heng tumor chi hrang hrangte hi screening schedule siam turin a rawt thei ang:

• Duodenal/periampullary polyp awmte chu:Chungte chu i small intestine chunglam (duodenum)-ah emaw, bile duct emaw pancreatic duct emaw small intestine nena inzawmna hmunah emaw a lo awm thin. FAP nei zawng zawng deuhthaw hian hengte hi an siam chhuak thin. Mi tlemte chuan duodenal cancer, ampullary cancer, pancreatic cancer in the duct, emaw bile duct cancer emaw an vei thei a ni.
• Stomach polyps: FAP vei zinga 90% velin stomach polyps an nei a, mahse heng zinga 1% chauh hi cancer-ah an chang thung.
• Desmoid tumors: Hei hi cancer ni lo tumor a ni a, connective tissue-ah a lo awm a. FAP vei zinga 15% vel ah an awm thin. Cancer nei lo mah se, a chang chuan (5% vel) chuan hriselna lama harsatna lian tak an thlen thei a, thihna thlengin an thlen thei bawk. An chetsual hle thei a, a bul hnaia thil awmteah an darh thei a, thisen kalna kawng, taksa peng leh nerve te chu an press on emaw block emaw thei bawk. An paih chhuah harsa a, a lo lang leh thei bawk.
• Papillary thyroid cancer: Hetiang thyroid cancer hi FAP vei zinga 2% velin an nei thin. A hlauhawm lo deuh a, tihdam theih a ni fo bawk.
• Liver cancer: A bik takin FAP vei naupangte hian liver cancer hmuh tur awm lo, hepatoblastoma an tih chu an vei theihna chance a tlem hle.
• Medulloblastoma: Hei hi thluak cancer a ni. Turcot syndrome nen a awm thei a, hei hi FAP nen a inzawm a ni. FAP nen pawh hian a tlem hle.
• Rectal polyps: I colon nen i rectum lakchhuah i nih loh chuan rectal polyps vei theihna i nei a, chuvangin i dam chhungin proctoscopy exam i neih a ngai dawn a ni.

Engtin nge FAP i nei tih chiang taka i hriat theih ang?

APC gene mutation hi i rochun tawh em tih hriat i duh chuan genetic testing hmangin i hre thei ang . Genetic test hian i DNA (thisen emaw, hmui emaw ang chi) sample a la a, gene mutations bik a zawng thin. Mutation i neih chuan a dawt leh tur chu colonoscopy hmanga adenomas enfiah a ni.

FAP hi polyp 100 tal (AFAP a nih chuan 20) a awm a, APC gene mutation a awm chuan hriat theih a ni. FAP hi adenomatous polyps thlentu hi hereditary condition awmchhun a ni lo. MUTYH-associated polyposis hi chutiang bawk chu a ni a, mahse gene dang MUTYH an tih a mutation vang a ni.

FAP vei tan chuan enkawl dan tur eng nge ni?

FAP enkawlna ruahmannaah hian dam chhung enkawlna leh operation tih ngei ngei tur a ni.A tir lamah chuan polyp (or AFAP) tlemte i neih chuan i doctor chuan colonoscopy (polypectomy) neih laiin pakhat zel a la chhuak thei a ni. Polyp te hi a lian lutuk emaw, cancer a nih chuan operation a ngai mai thei.

Inzai

Classic FAP vei tam zawk chuan kum sawmhnih hnuai lam emaw kum sawmthum tir lam emaw ah total colectomy an neih a ngai dawn a ni . I doctor chuan i surgery neih hun tur chu i risk factor bikte a zirin a rel ang. Colectomy chi hrang hrang i neih theih dan tur pawh a sawipui ang che.

I colon lakchhuah a nih chuan i small intestine leh rectum (or anus) inkarah gap a awm a. A châng chuan, surgeon chuan heng a tawp pahnih hi a thlunzawm leh thei ṭhin. Tichuan i rectum kaltlangin a hma ang bawkin bowel movement i nei thei ang. Chu chu a theih loh chuan ‘ostomy’ an siam ang a, chu chu i pum chhunga i bawlhhlawh chhuah theihna tur kawngkhar thar a ni.

Screening neih a ni

I medical team chuan tumor chi hrang hrang screening test vawi engzat nge i neih tur tih an hrilh ang che, chu chu i personal risk factors a zirin a ni. Classic FAP tan chuan kum 10 atanga colectomy thleng kum tin colonoscopy neih a tha . AFAP tan chuan kum tin screening tan tur a ni a, kum 20 atanga tan tur a ni.

I colectomy hnuah sigmoidoscopy exam i nei chhunzawm zel tur a ni a, chu chuan i GI tract hnuai lam a enfiah thin. I rectum hmun thenkhat i neih chuan thla 6 atanga thla 12 danah i check tir tur a ni. I rectum chu lakchhuah a, ileal pouch hmanga thlak a nih chuan kum 1 atanga kum 4 danah i check tir tur a ni.

Test tur ruahman dangte chu:

• Upper endoscopy: Hei hi colonoscopy nen a inang a, mahse i digestive system chunglam ah tih a ni. Endoscope chu i rilru atanga i pum leh i small intestine (duodenum) chunglam lamah polyps awm leh awm loh enfiah nan an pass thin. A awm chuan i doctor chuan a tih dan rualin a la chhuak thei bawk.
• Thyroid emaw liver cancer emaw a awm leh awm loh ultrasound test.
• CT scan (computed tomography scan) emaw MRI (magnetic resonance imaging) hmangin desmoid tumor a awm leh awm loh enfiah a ni.

FAP hi ven theih a ni em?

FAP condition hi i fate hnena i thlen theih dan hriatthiam nan genetic counselling neih a niA pui thei a ni. Chûng hlauhawmte chungchâng sawi chuan i chhûngkaw ruahmanna siam tûrin a ṭanpui thei che a ni. A tlangpuiin naupai laiin genetic mutation a awm loh nan ven theih a ni lo va, mahse family planning option hrang hrang i ngaihtuah theih a awm.

FAP vei dam rei zawng chu engzat nge ni?

Enkawl loh chuan a dam rei zawng chu kum 42 vel a ni . Mahse, damdawi lam enkawlna leh enkawlna dik tak i neih chuan nun pangngaiin i nung thei a ni . I colon lakchhuah hnuah i risk lian ber chu digestive system cancer dang leh harsatna nei zawk ‘desmoid’ tumor atanga lo chhuak a ni. Hengte hi colon cancer aiin a tlem zawk hle.

Hetiang dinhmun hi ka neih chuan eng nge ka beisei ang?

FAP i vei tih hmuhchhuah i nih chuan, i dam chhung zawnga damdawi lam test neih i beisei thei a, a nih loh leh operation vawi engemaw zat neih a nih pawhin tumor te chu paih chhuah i beisei thei bawk . I colon pawna tumor lo awmte hi i colon chhunga polyp awmte aiin cancer a nih theihna a tlem zawk. Desmoid tumor hi cancer ni lo mah se, a chang chuan harsatna tenau emaw, tibuaitu lian tak emaw pawh a ni thei.

I dam tirh lamah total colectomy neih i beisei thei . Chumi hnuah chuan i intestines chu a inzawm leh thei a, ileal pouch emaw ostomy emaw i nei thei bawk. Heng duhthlanna tin hian harsatna bik a thlen theihna a keng tel vek a ni. Mahse, colectomy hnuah pawh nun rei tak, hrisel takin i la nung thei tho.

Damdawi lama enkawl ngai tur genetic condition i nei tih i hriat hian lungngaihna leh lungkhamna i neih hi thil pangngai a ni. Mahse, i hriat hnuah chuan i cancer hlauhawmna chu i enkawl theih nân hma i la thei ang . Surgery hi i hmalam hun tur a nih ngei laiin, doctor-te chuan a awlsam thei ang bera siam an tum ang.

Mi tam tak chuan laparoscopic procedure hmangin colon an la chhuak thei a, chu chu incision tenau hmanga tih a ni a, a dam nghal vat thin . Ileal pouch nei te hian operation vawi tam tak an neih a ngai mai thei a, mahse a tawpah chuan a hma ang bawkin toilet an hmang thei tawh dawn a ni.

Kan sawiho tawh a\anga hriat reng tur pawimawh ber berte (Take-Home Message) .

Okay, chuti a nih chuan, `FAP` chungchang kan sawi tawh atang chuan, i hriat reng tur pawimawh ber berte chu hetiang hi a ni:

• FAP hi genetic condition a ni a, thlah kal zelah a kal zel thei a ni.
• Hei hian colon chhungah polyp za tam tak emaw, sang tam tak emaw a siam a, chu chu cancer a ni thei.
• Enkawl loh chuan colon cancer vei theihna a sang hle .
• Kum naupang (kum 10-12) atanga colonoscopy test neih tan a pawimawh hle .
• Enkawlna ber chu colon lakchhuahna operation (colectomy) a ni.
• Colon lakchhuah a nih hnuah pawh taksa hmun dangah tumor lo awm leh awm loh enfiah nan dam chhung screening neih a ngai a ni.
• Hei hi dam chhung zawnga enkawl ngai ni mah se, damdawi lam enkawlna leh test dik tak neih chuan nun pangngai, hrisel tak nun theih a ni .

Nangmah emaw, i chhungte zinga tu emaw chuan heng natna lan chhuah dan hi i neih chuan, emaw, he thil chungchang hi hriat belh i duh chuan, damdawi lam thurawn zawng ngei ang che . Hriat hmasak leh hmalak hmasak hi a pawimawh ber a ni.

` FAP, Chhungkaw Adenomatous Polyposis, Genetic Diseases, Colon Polyps, Colon Cancer, APC Gene, Colonoscopy, Colectomy, Surgery tih te a ni