I vunah hian bumps mak tak tak i nei em? Hei hi Muir-Torre Syndrome a ni thei ang em?

I vunah hian bump te te i lo awm tan nghal tawh em? Chûngte chu thil pangngaiah i ngai mai thei. Mahse, a chang chuan heng vun inthlak danglamna hi chhungril lama harsatna nen pawh a inzawm thei bawk. Vawiin hian kan sawi dawn a, thil awm lo tak, mahse hriat tur pawimawh tak kan sawi dawn a ni. Muir-Torre Syndrome an ti a.

Muir-Torre Syndrome hi eng nge ni? A awlsam zawngin...

Muir-Torre Syndrome hi genetic condition hmuh tur awm lo tak a ni a, i dam chhungin cancer chi hrang hrang i vei theihna a tipung a ni. He syndrome vei te hian an vunah hian vun tumor, cancer emaw benign (i.e., benign) emaw an nei tlangpui. Taksa chhungah cancer pakhat emaw, a aia tam emaw an nei thei bawk a, a bîk takin chaw kawng (gastrointestinal tract)-ah .

Ngaihtuah teh, kan taksa hi cell tenau maktaduai tam tak atanga siam a ni. Heng cell te hi an inthen darh hian a chang chuan genes-ah hian inthlak danglamna tenau (mutations) a awm thei a ni. Chu chu he genetic change thlentu chu a ni.

Chuti a nih chuan Muir-Torre Syndrome leh Lynch Syndrome hi a inang em?

A tam zawkah chuan, a ni. Moore-Torre syndrome hi Lynch syndrome chi khat anga ngaih a ni . Lynch syndrome pawh hi genetic change engemaw zat avanga cancer vei theihna tipungtu a ni bawk. A chang chuan Doctor ten Hereditary Non-Polyposis Colorectal Cancer (HNPCC) an ti bawk. Chumi awmzia chu polyp awm lovin colon-ah cancer a lo awm tihna a ni. Chuvangin, Moore-Torre hi Lynch syndrome atanga lo chhuak a ni a, vun ziarang bik a lantir a ni.

Hetiang dinhmun hi engtiang chiahin nge a awm? Sri Lanka-ah hian he thil hi kan ngaihtuah tur em ni?

Moore-Torre syndrome hi a takah chuan natna hlauhawm tak a ni . Khawvel pumah hri kai 200 vel chauh hmuhchhuah a ni. Amaherawhchu, hei hian kan hriat chian loh tur tihna erawh a ni lo. `HNPCC` vei zinga 9.2% vel chuan heng Moore-Torre features te hi an lantir thei a ni. Hmeichhia aiin mipa a nghawng nasa zawk niin a lang bawk (chu chu mipa 3 ah hmeichhia 2 vel tihna a ni). Sri Lanka ramah hian mi engzat nge awm tih hi statistics dik tak awm lo mah se, hetiang dinhmun hi hriat chian a pawimawh hle.

Moore-Torre syndrome natna lan chhuah dan chu engte nge ni? Engtin nge kan hriat chian?

Hei hi vunah lumps emaw inthlak danglamna emaw a lo lang vang leh internal cancer symptoms vang a ni ber . A châng chuan vun lama harsatna a awm hma emaw, a rualin emaw, chhungril cancer a lo awm hnuah emaw a awm thei bawk. Mahse, vun inthlak danglamna hi hriat hmasak ber a ni fo . Cancer dangte chuan a tir lamah chuan symptom an nei lo thei.

Vunah hian eng inthlak danglamna nge i hmuh theih?

Moore-Torre syndrome vei vunah hian a hnuaia mi hi hmuh tur a awm thei:

• Sebaceous adenomas: Hengte hi vun lama harsatna awm tam ber (80% - 99%) an ni. Hengte hi cancer nei lo (benign) tumor an ni. Kan vunah oil glands awm sebaceous glands-ah an lo thang lian a ni. Lu leh kawngah an awm tlangpui a, Moore-Torre natna vei zingah chuan rilru, pum, hip leh hnungzang-ah te an awm tam zawk . An hmel chu bumps te, khauh, sen emaw vun rawng emaw ang mai a ni .
• Sebaceous carcinoma: Hei hi cancer (malignant) tumor a ni a, sebaceous glands-ah a lo awm a. Sebaceous adenoma ang maiin a lang thei. Mahse, a darh nghal vat a, a bik takin mitmeng chhehvelah a darh rang hle . Heng tumor te hian thisen a chhuak thei a, crusty substance a ooze thei bawk.
• Keratoacanthoma: Hei hi vun natna chi dang a ni. Lu, kawr, ruh leh kut ah sam zung bulah a lo thang lian thei a ni. Cancer emaw cancer ni lo emaw pawh a ni thei . A hmel chu zungbun te tak te, a zungpui ang mai a ni a, a châng chuan a chungah thisen kalna kawng hmuh theih a awm a, a laiah keratin knot a awm bawk . A thang chak hle a, kar tlemte chhungin centimetre 3 vel zeta lian a ni a, chutah chuan thla emaw kum emaw chhungin a tlahniam zauh zauh thin. Pakhat emaw, a aia tam emaw pawh a awm thei.
• Fordyce spots: Moore-Torre syndrome-ah hian sebaceous glands hi a inrawlh fo avangin doctor thenkhat chuan heng "Fordyce spots" te hi symptom angin an ngai a. Chûngte chu sebaceous glands sang tak, lian deuh deuh, sam awm lohna hmuna lang, hmui vêl ang chite an ni . Zirna hrang hrangah Moore-Torre syndrome-ah hian an tam zawk tih hmuhchhuah a ni.

I vunah hian lump thar emaw, hetiang hian a danglam emaw i hmuh chuan, a bik takin a lo thang chak a, a rawng a danglam a, a thisen a chhuak a nih chuan doctor i pan ngei ngei tur a ni tih hre reng ang che.

Moore-Torre syndrome nen hian eng cancer nge inzawm?

He syndrome hmang hian cancer chi hrang hrang a awm thei. Cancer natna langsar ber ber leh a lan chhuah dan te chu:

• Colorectal cancer: Hei hi cancer chi khat zingah chuan a tam ber a ni . Mi zawng zawng zinga a chanve vel a nghawng a ni. Colon emaw rectum emaw lining-a cancer cells lo thang lian vang a ni. Chumi danglamna chu Moore-Torre nen chuan he cancer hi a tlangpui aia kum 15-20 hmain a lo awm thei a, a tlangpuiin kum 50 vel a ni a, a darh chak thei bawk . A lan chhuah dan chu pum na, pum na, pum na, chaw ei dan inthlak danglam, leh bawlhhlawh chhunga thisen chhuak te a ni .
• Pum cancer: Hei hi Moore-Torre-a cancer hmuh tur a ni bawk. A lan chhuah dan chu pum na, pum na, bawlhhlawh chhunga thisen, chaw ei harsa, rilru hah, leh ei duh lohna te a ni thei .
• Urinary system cancer natna: 1.1.`(Urothelial carcinoma)` emaw `(transitional carcinoma)` emaw an ti bawk. Hei hian urinary system lining a nghawng a ni. Zun laiin natna a thlen thei a , zun chhungah thisen a awm thei bawk .
• Endometrial cancer: Hei hi endometrium, nausen pum chhunga awm cancer a ni . Pum hnuai lam natna, pelvic na, leh vaginal discharge emaw thisen chhuah emaw danglam tak a thlen thei a ni .

Chu bakah, cancer chi dang engemaw zat chu he syndrome nen hian a inzawm thei a, chung zingah chuan:

• Ovarian cancer a ni
• Prostate cancer a ni
• Bladder cancer a ni
• Kidney cancer a ni
• Small intestine cancer a ni
• Liver cancer a ni
• Lung cancer a ni
• Thisen cancer a ni
• Brain cancer leh chi dang a awm bawk.

He list hi a hlauhawm hmel hle mai. Mahse a pawimawh ber chu heng cancer zawng zawng hi mi zawng zawngah a lo awm vek dawn lo tih hi a ni. Tin, a hmaa hmuhchhuah a nih chuan enkawl theih an ni bawk .

Engvangin nge he Moore-Torre syndrome hi a lo awm? Eng nge a chhan?

A awlsam zawngin, Moore-Torrell syndrome hi i gene pakhata mutation avanga lo awm a ni . Mutations chu kan DNA sequence inthlak danglamna a ni. He DNA hi kan cell te inthen darh a, cell thar an siam hian copy a ni. A chang chuan tihsual a awm thei. Chutiang cell pangngai lo chu a inthen chhunzawm zel chuan natna chi hrang hrang a thlen thei a, cancer pawh a thlen thei a ni.

Eng genetic variation nge hei hi nghawng?

A tlangpuiin chi hnih a awm a:

• Type 1 Moore-Torre syndrome (Type 1 MTS): Hei hi i DNA sequence-a tihsual siamtu gene pakhata mutation avanga lo awm a ni . Heng genes te hian tihsual an siam that theih loh chuan tissue ah hian cell pangngai lo tak tak an pung khawm thin. 90% ah chuan MSH2 an tih gene hi a nghawng a ni. Mahse, gene dang engemaw zat, MLH1, MSH6, leh PMS2 te chu a inrawlh thei a ni.
• Type 2 Moore-Torrell Syndrome (MTS): Hei hi MUTYH gene-a mutation awm vang a ni. He gene hian kan DNA a oxidative damage siamthat hna a thawk a ni. MTS vei hmun thuma ṭhena hmun khat vel chuan he chi hi an nei a ni. Oxidation hian DNA molecule a tidanglam a, chu chuan cell growth control loh (i.e., cancer) a thlen thei a ni. Mutated gene hian he chhiatna hi a siam that theih loh chuan a chhiatna chu a awm reng a ni.

Hei hi thlah kal zel atanga lo chhuak thil a ni em?

A nihna takah chuan, a ni, rochun aṭanga lo chhuak a ni . Mahse, a chang chuan mi pakhatin gene mutation thar a siam thei a, chu chu chhungkaw zingah tu mahin an nei hmasa lo. Damlo 60% vel chuan chhungkaw history-ah Moore-Torre syndrome an hmu thei a ni. Mahse, he gene mutation nei zawng zawng hian symptom an nei vek lo a, chuvang chuan he chhungkaw inzawmna hi a lang chiang lo.

Mi thenkhat chuan he natna hi symptom nei lovin an nei thei a, an hre lo pawh a ni thei. Tin, a chang chuan immune system a chak loh chuan he `latent MTS` hi a activate thei bawk. Mi thenkhat chuan `solid organ transplant` an dawn hnuah leh immunosuppressant damdawi an ei hnuah he natna hi an nei thin.

Engtin nge hei hi thlah kal zelah a lo kal zel?

• Type 1 MTS hi ``autosomal dominant'' tih a ni. Hei hian a awmzia chu he natna hi rochun tur hian mutated gene copy pakhat chauh i neih a ngai tihna a ni. I nu leh pa zinga pakhatin he gene mutation hi a neih chuan nang pawhin i rochun theihna chance 50% i nei a ni. Mahse, symptom i neih dan chu i gene i rochun nu leh pa nen a danglam thei.
• Type 2 MTS hi ``autosomal recessive`` tih natna a ni. Chumi awmzia chu natna i rochun theih nan i nu leh pa pahnih hian gene mutation inang an neih a ngai tihna a ni. Hei hi a tlem hle. Mahse, he ``autosomal recessive`` gene copy khat chauh nei nu leh pate hian symptom an nei lo mai thei a, chuvangin an nei tih an hre lo mai thei.

Doctor-te hian engtin nge Muir-Torre Syndrome hi an hriat theih?

A chunga kan sawi ang hian vun hring i neih chuan, a bik takin i trunk-ah emaw, kum naupang deuha lo lang tawh emaw a nih chuan doctor chuan Moore-Torrell syndrome a ringhlel mai thei. Tin, internal cancer tilang thei symptom dang te, i chhungkuaah cancer history nei tawh leh awm loh te pawh a zawt bawk ang che.

Hemi atan hian eng ang test nge tih thin?

I doctor chuan genetic test neih a rawt hmain preliminary test eng emaw zat a ti thei ang. Entir nan:

• Immunohistochemistry (IHC): I vunah lump sample te tak te lak a, special dye hmanga stain a, microscope hmangin sample-ah hian genetic mutations bik a awm em tih enfiah a ni.

Heng test result leh thu dangte hmang hian i doctor chuan Moore-Torre syndrome nena inzawm gene-a mutation awm leh awm loh enfiah turin genetic test neih a rawt thei ang. Chumi hnuah i thisen sample an la ang a, mismatch repair genes MSH2, MLH1, MSH6, PMS2, emaw base excision repair gene MUTYH emaw-a mutations an zawng ang.

Moore-Torre syndrome vei chuan eng ang cancer screening nge a neih fo ang?

He syndrome vei te tan chuan cancer screening neih fo a pawimawh hle , a chhan chu a hmaa hmuhchhuah hian enkawlna hlawhtling tak neih theihna a tipung a ni. Doctor-te’n test an rawt theihte chu:

• Colonoscopy: Intestine lian tak enfiahna.
• Upper endoscopy (EGD): Chaw ei kawng, pum leh small intestine hmun hmasa ber enfiah.
• Prostate exam ( mipa tan) 1.1.
• Mammogram hmanga enfiah: 1.1.Breast cancer screening (hmeichhia tan) .
• Pelvic exam ( hmeichhia tan) .
• Pap smear: Cervical cancer enfiahna (hmeichhia tan) .
• Urine cytology: Urinary system-a cancer awmte tan.
• Liver hnathawh dan test a ni
• Thisen chhiar kim (CBC) 1.1.
• Taksa enfiahna
• Skin exam a ni
• Chest X-ray neih a ni

I doctor chuan heng test te hi engzat nge i tih tur tih a rel ang.

Moore-Torre syndrome hi eng enkawlna nge ni?

Moore-Torre syndrome enkawlna atana thil pawimawh ber chu cancer a lo awm chuan zawn chhuah leh tihbo hi a ni . Cancer screening neih fo a ngai ang a, tissue rinhlelh awm apiang biopsy i lak a ngai bawk ang. I doctor-in cancer a hmuh chuan a chi leh a stage a zirin an enkawl ang. Cancer lakchhuahna tur operation hi enkawlna hmasa ber a ni tlangpui .

Cancer enkawlna dangte chu:

• Chemotherapy hmanga enkawl a ni
• Radiation hmanga enkawlna a ni
• Hormone hmanga enkawl a ni
• Immunotherapy hmanga enkawl a ni
• Targeted therapy hmanga enkawlna
• Bone marrow thlak danglam a ni

Tin, oral retinoid isotretinoin an tih hian vun natna thar lo awm lo turin a pui thei bawk. I colonoscopy-ah hian colon polyp tam tak (cancer vei theihna hlauhawm tak) a awm a nih chuan, i doctor chuan prophylactic colectomy tih a rawt thei a, chu chu i colon hmun thenkhat emaw, a pum emaw lakchhuah a ngai a ni.

Moore-Torre syndrome vei tan chuan eng prognosis nge a awm?

I prognosis chu cancer i neih zat leh a darh zau zawngah a innghat a ni. Statistics atanga a lan dan chuan Moore-Torre syndrome vei zinga a chanve vel chuan cancer pakhat aia tam an vei a ni. A chanve aia tam chuan metastatic cancer (taksa hmun dangah a darh tawh a, enkawl harsa tak cancer) an vei a ni.

Moore-Torre syndrome vei cancer hi kum 50 vel an ni tlangpui a , heng cancer te hi mipui zahawm tak aiin a lo thang lian thei a, a na zual nghal vat thei bawk . Chuvangin, a hmaa hriatchhuah a pawimawh . Tin, cancer lakchhuah hnuah pawh a lo let leh theihna chance a sang hle bawk . Chuvangin inenkawl zawha check-up mumal neih a pawimawh hle.

Hei hi ven dan tur a awm em?

A tlangpuiin a ni lo. Hei hi i pianpui dinhmun a nih vang a ni. Mahse, mi zawng zawngin symptom an nei vek dawn lo. Symptoms nei ve thote pawh hi an nu leh pate aiin natna hian a tuar tam emaw, a tuar tlem emaw pawh a ni thei. Scientist te hian engvangin nge hetiang thil hi a thlen tih an la hre chiang lo. Mahse, immune system a chak loh chuan a natna chu a zual thei tih hmuhchhuah a ni .

He genetic mutation hi i nei tih hriat hian invenna tur thutlukna thenkhat siam turin a pui thei che a ni. Entirnan, genetic testing leh genetic counseling te hian i fate hnena he natna hi i thlen loh nan a pui thei che a ni. Tin, hei hi hriat chian hian cancer hriat hmasak leh enkawl tan theihna a siam thei bawk a, a rah chhuah tha lo tur a veng thei bawk.

Moore-Torre syndrome hi a awm lo hle nachungin hetiang diagnosis hi hmachhawn a awlsam lo. Kan DNA-a innghat thil pakhat do chu thil harsa tak angin a lang thei. Mahse, hriatna chu thiltihtheihna a ni . A hun taka hriatchhuah leh enkawl a nih chuan nang leh i doctor hian he harsatna hi i hmachhawn thei ang.

A tawp berah chuan take-home message

Okay, chuvangin kan sawi tawh atang khan i hriat reng tur thenkhat chu khaikhawm ila:

• Muir-Torre Syndrome hi genetic condition a ni a, vun tumor leh internal cancer (a bik takin digestive tract-ah) vei theihna a tipung a ni.
• I vunah, a bik takin i trunk-ah lump thar, danglam tak i hmuh chuan doctor lam pan nghal rawh. Engmah a ni lo thei a, mahse check out tir hi a tha ber.
• Hei hi Lynch syndrome chi khat a ni. I chhungte zingah cancer vei tawh an awm chuan i doctor hnenah pawh hrilh bawk ang che.
• Hriat hmasak leh screening neih fo hi a pawimawh hle. Hei hian cancer hi a hmaa hriatchhuah a, hlawhtling taka enkawl a pui thei a ni.
• Genetic testing leh counseling hian he natna hi a awm leh awm loh leh chhungkaw member-te a nghawng em tih hriat theih nan a pui thei a ni.
• Hlau suh, fimkhur zawk rawh. Nangmah chauh i ni lo va, doctor-te pawhin tanpui turin an awm reng a ni.

He thu hi i tan a tangkai ngei ka beisei. Hrisel takin awm rawh!

` Muir-Torre Syndrome, Lynch Syndrome, Vun Cancer, Genetic Diseases, Gastrointestinal Cancer, Cancer Screening, Genetic Mutations