I taksaah heng lumps mak tak tak te hi i nei em? Von Hippel-Lindau Disease (VHL) chungchang hi han sawi ila.

Engvangin nge ka taksa pum puiah hian hnawih mak tak tak ka awm fo thin tih i ngaihtuah mai thei, a nih loh leh natna thenkhat chu a reh loh vang mai mai tih i ngaihtuah mai thei. A chang chuan a chhan hi kan ngaihtuah aiin a buaithlak deuh thei bawk. Vawiin hian kan sawi dawn a, thil awm lo tak, mahse hriat tur pawimawh tak tak kan sawi dawn a ni. Chu chu von Hippel-Lindau natna a ni a, a tawi zawngin `(VHL)` kan ti a ni.

Von Hippel-Lindau (VHL) hi eng nge ni? A awlsam zawngin i hrethiam ang u.

Okay, tunah chuan he `(VHL)` hi eng nge ni tih i ngaihtuah mai thei. A awlsam zawngin von Hippel-Lindau hi genetic condition hmuh tur awm lo tak a ni. I taksa chhunga gene thenkhatah `(genetic mutation)` emaw, inthlak danglamna emaw a awm chuan a thleng thin. Chumi avang chuan i taksa peng hrang hrangah `(malignant)` tumor te, chubakah `(benign)` tumor leh cyst te i nei tam zawk thin. Doctor-te chuan he natna hi `(von Hippel-Lindau syndrome` an ti \hin.

Research atanga a lan dan chuan he genetic mutation nei zinga 97% chuan kum 65 an tlin chuan heng leh VHL nena inzawm tumor dangte hi an nei ang a, a tam zawkah chuan VHL nena inzawm tumor enkawlna ber chu operation tihchhuah a ni.

VHL natna nen hian eng cancer nge inzawm thei?

Hetiang `(VHL)` natna hi i neih chuan a hnuaia cancer chi khat emaw a aia tam emaw i vei theihna a sang zawk a ni:

• Clear cell renal carcinoma (ccRCC): Hei hi kal cancer chi khat zingah chuan a tam ber a ni. Mithiamte sawi dan chuan VHL vei zinga 25% atanga 60% inkar velin he cancer hi an vei thei a ni. Kan taksaah hian kal (kidney) hi kan taksa peng pawimawh ber pakhat a ni tih hre reng ang che, chuvangin a chhunga cancer hi kan ngaih pawimawh em em a ni.
• Pancreatic neuroendocrine tumors (pancreatic NETs): Hengte hi tumor chi khat, i pancreas-a endocrine cells atanga intan a ni. VHL vei zinga 9% leh 17% inkarah an vei thei a ni.
• Pheochromocytoma: Hei hi tumor awm lo mahse enkawl theih a ni a, adrenal gland-ah a lo awm thin. Heng tumor te hi noncancerous tumor angin a intan thei a, mahse a hnuah cancer a ni thei bawk. VHL vei zinga 10% atanga 20% ah an awm thin.
• Broad ligament cystadenomas: He natna hian hmeichhia a nghawng a ni. VHL vei hmeichhia 10% vel ah a awm thin. Fallopian tube bula lo awm tumor a ni.

VHL nena lo awm cancer ni lo tumor te chu engte nge ni?

Cancer chauh ni lovin, cancer ni lo tumor, chu chu taksa peng dangah a darh lo (metastasize) te pawh VHL a awm chuan a lo awm thei a ni. Chung zinga pawimawh ber chu hemangioblastoma a ni.Hengte hi cancer ni lo tumor an ni a, thluak thisen kalna kawngah, ruhroah emaw, retina-ah emaw a lo awm a ni. An darh lo nachungin an lian thei a, a chhehvel tissue te a nghawng thei a, hriselna lama harsatna lian tak a thlen thei a ni.

`(VHL)` nena inzawm hemangioblastomas chi hrang hrang hmuh theihte chu:

• Retinal hemangioblastoma: Hei hi mit atanga lo chhuak tumor chi khat a ni. Mit a hloh phah hial a ni. Research atanga a lan dan chuan (VHL) vei zinga 60% ah a awm thin.
• Brain stem leh cerebellar hemangioblastomas: Hengte hi thluaka tumor awmte an ni. I balance a nghawng thei a, harsatna dang a thlen thei bawk. VHL vei zinga 13% leh 72% inkar vel an vei a ni.
• Spinal cord hemangioblastoma: Hetiang hemangioblastoma hi (VHL) vei zinga 13% leh 50% inkar ah a awm thin.

Tin, VHL i neih chuan cancer ni lo tumor leh cyst te pawh i nei thei bawk a, chungte chu:

• Epididymal cystadenomas: Hei hi mipa natna tuar thei tumor a ni. Testicles bulah hian epididymis, tube ang chi, sperm dahkhawmna, testicles bulah an lo thang lian a ni. VHL vei mipa 25% leh 60% inkar ah an awm thin.
• Endolymphatic sac tumors (ELST): Hengte hi tumor awm lo tak tak an ni. VHL i neih chuan beng chhungril lamah an lo thang lian thei. He natna hian VHL vei zinga 10% leh 25% inkar a nghawng a ni.
• Cysts: Hei hi tuiin a khat cyst a ni. VHL vei te hian kal leh pancreas-ah heng cyst te hi an nei thei a ni.

VHL natna hi engtiang chiahin nge a hluar?

Hei hi a takah chuan thil awm lo tak a ni. Mi 36,000 zinga pakhat velin a nghawng a ni. VHL vei tam zawk chuan kum 20 bawr vel atang khan symptoms an nei tan thin. Hei hian kum naupang takah he thil hi i fimkhur a ngai tihna a ni.

Von Hippel-Lindau natna lan chhuah dan chu engte nge ni?

Hetiang dinhmun hian i taksa pum puiah tumor a siam theih avangin symptoms a inang lo thei. Tumor awmna leh a lian leh teah a innghat a ni. Hetiang symptoms hi i nei thei ang:

• Lu na fo thin.
• Hriatna hloh emaw beng chhunga ri reng reng (tinnitus).
• Thisen sang a sang.
• Kal laiin balance hloh.
• Muscle chakna tlahniam emaw, chetna inrem harsa emaw.
• Vision chungchang a ni.
• Luak chhuak.

Ngaihtuah teh, heng chhinchhiahna pakhat emaw pahnih emaw hi kan neih chuan engtiang chiahin nge kan lungkham ang? Chuvangin vawi khatah heng symptoms engemaw zat i neih chuan doctor thurawn lak a pawimawh tak zet a ni.

VHL natna hi eng nge thlen?

Von Hippel-Lindau syndrome hi rochun natna a ni a . I nu leh pa pakhat hnen atanga tumor suppressor gene VHL an tih copy danglam tak i rochun hian a thleng thin.

A awlsam zawngin sawi ila, heng ``tumor suppressor genes`` te hian cell te inthen rang lutuk chu a titawp a, chu chuan cancer a thlen thei a ni. Car brake ang mai a ni. Mahse heng genes te hi mutated a nih chuan brake lakchhuah a, accelerator dah ang mai a ni. Cell thanna chu thunun theih lohvin a chak zawk.

He natna hi autosomal dominant inheritance pattern hmanga kai a ni. Chumi awmzia chu i nu leh pa zinga pakhatin VHL gene pangngai lo a neih chuan i rochun theihna chance leh von Hippel-Lindau natna vei theihna chance 50% i nei tihna a ni. Mahse, zirchianna atanga a lan dan chuan VHL vei zinga 10% vel chuan chhungkaw history-ah he natna hi an nei lo. Chumi awmzia chu genetic mutations thar pawh a awm thei tihna a ni.

VHL natna hi engtin nge an hriat theih?

Doctor-te chuan VHL vanga natna lan chhuah dan, hemangioblastoma emaw clear cell renal carcinoma emaw i neih chuan VHL i nei tih an ringhlel thei. Mahse hei hi finfiah theihna awmchhun chu genetic testing hmanga tih a ni. I chhungte zingah Von Hippel-Lindau natna vei an awm chuan i doctor hnenah i tan genetic testing chungchang zawh a pawimawh.

VHL natna enkawl dan tur engte nge ni?

VHL tumor emaw cyst chi hrang hrang a zirin enkawl dan pawh a inang lo. I doctor chuan i tana tha tur enkawlna kawng hrang hrang a hrilhfiah ang che. Enkawlna hman tlanglawn ber berte chu:

• Surgery hmanga tumor lakchhuah a ni.
• Chemotherapy hmanga enkawl a ni.
• Radiation hmanga enkawlna a ni.
• Targeted therapy-ah hian peptide receptor radionuclide therapy (PPRT) leh tyrosine kinase inhibitors (TKI) ang chi thil te a tel a ni.
• Immunotherapy hmanga enkawl a ni.
• Hormone hmanga enkawl a ni.

A pawimawh ber chu natna hi hriat hmasak a, a enkawl dan tur dik tak tan a ni. Chumi hnuah chauh chuan result tha ber i hmu thei ang.

VHL natna hi a dam kim thei em?

Vanduaithlak takin tun dinhmunah hian von Hippel-Lindau natna hi damdawi a la awm lo. Enkawlna tum ber chu a rang thei ang bera tumor zawng chhuak leh paih chhuah a ni. I doctor chuan enkawlna dang bakah tumor te chu paih chhuah nan operation neih a rawt mai thei.

VHL ka neih chuan eng nge ka beisei ang?

Hetiang dinhmun hi i neih chuan natna thenkhat chhinchhiahna awm leh awm loh enfiah nan test neih fo a ngai dawn a ni. Tumor hmuhchhuah hmasak leh enkawl hian von Hippel-Lindau natnain i nunah nghawng a neih chu a tihziaawm thei a ni. Chuvangin i doctor-in a rawtna test neih a pawimawh hle.

VHL natna hi ven theih a ni em?

Ni lo, a dang thei lo. Hei hi a chhan chu von Hippel-Lindau natna hi nu leh pa pakhat atanga nu leh pa dang hnena genetic mutation kaltlanga lo awm a nih vang a ni. I chhungkuaa mi pakhatin VHL a vei a nih chuan, genetic testing hmangin nang pawhin mutation hi i nei em tih hriat theih nan a pui thei che a ni.

Von Hippel-Lindau natna hi i veng thei lo nain, i risk hriat leh `(VHL)` hian engtin nge a nghawng theih tih hriatthiam a \angkai hle. I hlauhawm tih i hriat chuan doctor-te chuan `(VHL)` nena inzawm tumor chhinchhiahna te chu an en thei a, a hmaa lakchhuah tumin hma an la thei bawk.

(VHL) gene i neih a, fa neih i tum a nih chuan genetic counselor nen inhmukhawmin i fain gene a rochun theih dan tur sawipui a tha ang.

VHL nena inzawm natna hrang hrangte screening test bik a awm em?

Hemi atan hian screening guideline bik a awm lo a, cancer chi dang (US Preventive Services Task Force-in cancer screening a rawt ang chi) a awm ang bawkin. Mahse, genetic testing-in VHL gene dik lo i nei tih a finfiah chuan i healthcare team chuan harsatna hriat hmasakna tura pui turin test thenkhat an rawt thei ang. Entirnan, VHL nena inzawm cancer, clear cell renal carcinoma ang chi te enfiah nan kum hnih danah abdominal magnetic resonance imaging scan (MRI) neih tir turin an rawt mai thei.

Engtin nge ka in enkawl ang? Engtin nge ka tih theih ang?

VHL nena nun tih awmzia chu rinhlelhna engemaw zat neia nun tihna a ni. VHL thlentu genetic mutation i rochun chuan 50% in he natna hi i vei theihna chance i nei a ni. Chutianga i tih chuan cancer chi hrang hrang leh natna khirh tak dang i vei theihna chance 97% i nei a ni.

Mahse, `(VHL)` hian i nun a nghawng dan tur dik tak chu tumahin an sawi lawk thei lo. Heng harsatnate hi i hmachhawn theihna tura pui thei tur thil thenkhat chu hetiang hi a ni:

• Mental health support dawng rawh: Zirna pakhatah chuan VHL hmuhchhuah hian rilru lam nghawng a nei thei a, chu chu lungkhamna leh panic attack te hi a ni. Chuvang chuan rilru lam doctor emaw, counselor emaw nena inbiakna neih a pawimawh hle.
• I hriselna zawng zawng humhim rawh: Ei leh in inthlau tak ei rawh – sa thau lo, whole grains, leafy greens, leh thei chi hrang hrang tam tak ei rawh. Exercise (chu chuan stress a tihziaawm thei bawk). I medical team te hnenah i tana tha tur vaccine te zawt la.

Engtikah nge damdawi lam thurawn ka lak ang?

Hetiang symptoms i neih chuan doctor i call nghal tur a ni:

• I mit emaw, i hriatna emaw a inthlak danglam.
• Lu na fo thin.
• Natna dang avanga awm nia ngaih loh nausea emaw, luak chhuak emaw.
• Thisen sang a san thut.
• I kal harsat thut a nih chuan, balance vawn reng tur, emaw, chetna inrualrem thei tur i neih chuan.

Doctor hnenah eng zawhna nge ka zawh ang?

I chhungkuaa mi pakhatin von Hippel-Lindau natna a vei tih hrilh che kha han mitthla teh. Chutiang a nih chuan, i doctor-a zawhna i zawh theih ṭhenkhat chu hetiang hi a ni:

• Ka genetic test result hian eng nge a sawi?
• Ka chhungkaw dangte hian genetic testing an nei tur em ni?
• `(VHL)` nena inzawm thei dinhmun chhinchhiahna hmasa ber hriat theih nan engzat nge test ka neih ang?

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Von Hippel-Lindau natna (VHL) hi genetic disorder hmuh tur awm lo tak a ni. Mi 36,000 zinga pakhat a nghawng a ni. Chuvangin, chutiang mi 36,000 zinga mi i nih thu hriat chu thil mak tak a ni thei a, a bik takin i chhungte zingah he natna hi tu mahin an la vei lo a nih chuan.

Genetic testing-in (VHL) i nei tih a nemnghet a nih chuan, thuthang chu process turin hun engemaw chen pe rawh. Tichuan, i diagnosis awmzia hrethiam turin hun hmang rawh. Entirnan, cancer leh tumor chi hrang hrang i vei theihna tur chungchangah zawhna i nei thei a, i natna hmuhchhuah hian i chhungkaw midangte a nghawng dan tur chungchangah zawhna i nei thei bawk. I medical team-te chu eng nge an beisei tih zawt hreh ngai suh. Tin, (VHL) nena khawsakna avanga lungkhamna lo awm thinte enkawl turin tanpuina dil theihnghilh suh. Nangmah chauh i ni lo va, he zinkawngah hian tanpui thei tur che mi tam tak an awm bawk.

` Von Hippel-Lindau, VHL, genetic disease, cancer, tumor, symptoms, enkawl dan