He tumor hmuh tur awm lo Paraganglioma an tih chungchanga i zawhnate chhanna chu hetiang hi a ni!

A chang chuan i thawkrim lutuk, i thinlung a na hle a, i lu a na hle tih i hre nghal em? A nih loh leh, chhan awm lovin i thisen sang a sang thut ang maiin i inhria em? Hengte hi thil random mai maiah i ngai mai thei a, mahse a chang chuan hei hi chhan dang a awm thei bawk. Paraganglioma hi chutiang natna hmuh tur awm lo mahse hriat tur pawimawh tak pakhat a ni.

Chuti a nih chuan, he paraganglioma hi eng nge ni?

A awlsam zawngin, paragangliomas hi i taksaa tumor awm lo tak tak a ni. I kawr chhunga thisen kalna lian ber carotid artery bulah te, i lu leh kawr chhunga nerve-te bulah emaw, i taksa hmun dangah emaw an lo awm fo thin. Heng tumor te hi cell chi khat bik chromaffin cells an tih atanga siam an ni a . Heng cell te hian hormone catecholamines an siam a, i thisenah an chhuah tir thin.

Kan kal chunga awm adrenal glands hian hormone chi hrang hrang a siam chhuak tih i hria em? Heng catecholamines te hi kan taksaa hna pawimawh tak tak engemaw zat control tu hormone an ni. Eng nge an nih i hria em?

• I heart rate chu.
• Thisen sang a ni.
• Thisen sugar level (`thisen glucose`) a awm.
• Kan taksain stress a chhan dan.

Catecholamine chi hrang hrang pawimawh ber berte chu:

• Dopamine a ni.
• Epinephrine (hei hi adrenaline kan tih pawh a ni).
• Norepinephrine (noradrenaline an ti bawk).

Paragangliomas hi adrenal glands-ah lo awm lo mah se, heng tumor te hi adrenal glands-a awm tissue atanga siam a ni. Chuvangin heng paragangliomas bakah hian catecholamine hormone te pawh thisenah a pung khawm thei bawk. Chutah chuan symptom hrang hrang a lo lang tan ta a ni.

Paraganglioma leh Pheochromocytoma hi eng nge an danglamna?

Heng hming pahnih hi a buaithlak deuh thei a ni lawm ni? Paraganglioma leh pheochromocytoma te hi a chunga kan sawi tak chromaffin cell atanga lo chhuak tumor hmuh tur awm lo tak tak an ni. An pahnih inthlauhna ber chu taksaa an insiam dan hi a ni.

Pheochromocytomas hi i adrenal gland lai takah a lo awm a, chu chu adrenal medulla an ti a ni. Paragangliomas hi adrenal gland pawnah a lo awm thin. Kawr chhunga thisen kalna kawng emaw, nerve emaw bulah an awm fo thin. Chuvangin paragangliomas hi ‘extra-adrenal pheochromocytomas’ an ti fo thin.

Paraganglioma hi cancer a ni em?

Hei hi mi tam takin harsatna an neih a ni. Heng tumor, paragangliomas , tia koh te hi cancer (malignant) emaw noncancerous (benign) emaw pawh a ni thei.A tlangpuiin paraganglioma 20% vel hi cancer vei an ni.

Mahse hetah hian harsatna awm chu, a chang chuan doctor-te tan chuan tumor chu cancer a nih leh nih loh chiang taka hriat a harsa thin. Chu chu, tumor chu surgery hmanga lakchhuah a, a tissue chu microscope hmanga enfiah pawh ni se, a chang chuan chiang taka sawi theih a ni lo tihna a ni. Chuvangin, paraganglioma hi a hnuaia mite hi cancer anga ngaih a ni tlangpui.

• Tumor chu a chhehvel tissue-ah a darh tawh a nih chuan (hei hi `regional spread of paraganglioma` an ti a).
• Hmun hla tak takah a darh tawh a nih chuan, i lung emaw ruh emaw (hei hi `metastasized` an ti).
• A tir lama enkawl leh tihdam a nih hnua a lo kir leh a nih chuan (hei hi `recurred` an ti).

Cancer a nih chuan engtin nge a darh zel?

He paraganglioma hi cancer a nih chuan a tan standard staging system a awm lo. Chu ai chuan hetiang hian a sawifiah zawk a ni:

• Localized paraganglioma: Tumor hi hmun khatah chauh a awm a.
• Regional paraganglioma: Cancer hi a bul tanna bul hnaia lymph node emaw tissue dang emaw ah a darh tawh a.
• Metastatic paraganglioma: Cancer hi i taksa peng dang, i liver, lung, ruh emaw, lymph node hla tak takah emaw a darh tawh a ni. Cancer natna paragangliomas 35% leh 50% inkar hian hetiang hian a darh (metastasize) thei a ni.
• Recurrent paraganglioma: Cancer hi enkawl leh tihdam a nih hnuah a lo let leh tawh a ni. A hmaa a awmna hmunah a awm thei a, taksa peng dangah pawh a awm thei bawk.

Heng nut te hi engtiang chiahin nge an lo thang chak?

A tlangpuiin paragangliomas hi a lo thang zawi zawi hle. Mahse, hei hi mi tin a inang lo thei. Mi thenkhat chu an thang chak deuh mai thei.

Hetiang dinhmun hian tute nge a nghawng nasa ber?

He natna, paraganglioma , tia koh hi kum eng pawhah a lo awm thei a ni. Mahse, kum 30 leh 50 inkar ah a tam ber a, chubakah naupang 10% velin an vei tih hmuhchhuah a ni bawk.

Paraganglioma hi engtiang chiahin nge a awm?

Hei hi a takah chuan tumor hmuh tur awm lo tak a ni. Statistically chuan mi maktaduai khatah mi pahnih vel chauh hian paraganglioma an vei niin an chhut. Chuvangin hei hi a tlem zia chu i ngaihtuah thei ang.

Paraganglioma natna lan chhuah dan chu engte nge ni?

Paragangliomas symptoms hi tumor hian a chunga kan sawi tak adrenaline emaw noradrenaline emaw i thisenah a chhuah tam lutuk vang a ni. Mahse, paraganglioma thenkhat chuan he extra hormone hi an siam chhuak lo va, chuvangin symptom engmah an nei lo (asymptomatic an ti).

Symptom hmuh tam berHi:

• Thisen sang (hypertension) a lo awm nghal vat.
• Lu na.
• Chhan awm lova thawk hah lutuk.
• Thinlung thawk rang tak, mumal lo tak, thinlung thawk ri hriat theih khawpa ring.
• I taksa a nghing ang maia inhriatna.

Heng bakah hian symptoms langsar lo zawk pawh a awm :

• I hmel lan dan pangngai aia paler zawka awm.
• Nausea leh/ emaw, luak chhuak.
• Kawthalo.
• Ek khal.
• Thisen thlum sang (hyperglycemia).
• Thisen sang tlahniam nghal a, ding chhuakin luak chhuak (hei hi orthostatic hypotension an ti a).
• Chhan awm lova thinrim.

Mi thenkhat chuan heng symptoms te hi an nei tlem hle thei a, a nih loh leh burst-ah pawh an nei thei bawk. Ngaihtuah teh, a chang chuan thil dik lo a awm lo va, chutah chuan heng symptoms te hi a lo lang nghal a, chutah chuan rei vak lo hnuah a bo leh thin. Hei vang hian a chang chuan hei hi hriat a tlai thin.

Eng thilin nge paraganglioma hi a thlen?

A tam zawkah chuan paragangliomas awm chhan bik a awm lo. Chu chu random takin an thleng tihna a ni. Mahse, mi 25% atanga 35% vel chuan chhungkaw hrang hranga kal thin genetic condition (hereditary condition) avanga paragangliomas an vei thin. Chung dinhmun thenkhat chu:

• Multiple endocrine neoplasia 2 syndrome, chi A leh B (MEN2A leh MEN2B)) te a ni.
• Von Hippel-Lindau (VHL) natna a ni.
• Neurofibromatosis chi khat (NF1) a ni.
• Hereditary paraganglioma natna hi a ni.
• Carney-Stratakis dyad (chutah chuan paraganglioma hi gastrointestinal stromal tumor (GIST) nen a awm thei).
• Carney triad (chutah chuan paraganglioma, GIST, leh lung tumor chi khat pulmonary chondroma an tih te pawh a tel thei).

Engtin nge Paraganglioma hi hmuhchhuah a nih?

Paraganglioma hi hriat a harsa thei hle a, a chhan chu tumor hmuh tur a awm lo va, a chang chuan symptom a awm lo. A châng chuan, doctor-te chuan chhan dang vangin test an tih laiin paragangliomas an hmu ṭhin.

Doctor chuan a hnuaia thilte hi a ngaihtuah hnuah paraganglioma a ringhlel thei a ni:

• I medical history kimchang, a bik takin i chhungkuaa tu pawhin pheochromocytoma emaw paraganglioma emaw an vei tawh em tih te.
• Taksa leh damdawi lam enfiahna kimchang.
• Symptom thenkhat awm dan. Entirnan, damdawi pangngaiin a thunun theih loh thisen sang i neih chuan.

Hei atan hian eng test nge tih thin?

I doctor chuan paraganglioma i vei em tih hriat nan heng test leh procedure te hi a hmang thei ang:

• Taksa enfiah: I doctor chuan a enfiah ang che a, i hriselna tlangpui, i thisen sang ang chi te chu a enfiah ang che. Nang leh i chhungte damdawi lam chanchin, a bik takin hormone nena inzawm harsatna eng pawh a zawt bawk ang.
• Darkar 24 chhunga zun test: Hei hi darkar 24 chhunga i zun sample lakkhawm a, adrenal hormone catecholamines an tih zat tehna a ni. Heng hormone te hi a chhiat chuan thil lo awm thin te pawh a zawng thin. Heng catecholamines te hi i zun ah hian a level pangngai aia sang a awm chuan paraganglioma chhinchhiahna a ni thei.
• Thisen catecholamine test: Heng test te hian i thisena catecholamines awm zat a teh thin. Sawi tawh ang khan heng hormone te hi a chhe vek a, thil lo chhuak thin te pawh an zawng thin. Hengte hi thisenah a tlangpui aia tam a awm chuan paraganglioma chhinchhiahna a ni thei bawk.
• PET scan (positron emission tomography scan): PET scan ah hian i taksaah radioactive chemical (radiotracer) him tak inject a, PET scanner an tih device hmangin i taksa peng leh tissue te thlalak lak a ni. He scan hian chemical hi active tak tak cell leh tumor te a hmuchhuak a ni. Hei hian hriselna lama harsatna a awm leh awm loh hriat theih nan a pui thei a ni. He scan hi paraganglioma awmna hmun hriat chian nan a tha hle.
• CT scan (computer tomography scan): CT scan chu angle hrang hrang atanga X-ray hrang hrang lak atanga i taksa chhungril chipchiar taka thlalak siamna a ni. I doctor chuan CT scan hmangin tumor awmna hmun (a tlangpuiin kawr chhungah) a hriat theih nan a rawt thei ang.
• MRI scan (magnetic resonance imaging): MRI hian magnet, radio wave leh computer hmangin i taksa chhung lam thlalak chipchiar tak tak a siam a. I doctor chuan tumor awmna hmun chu a chiang zawka hriat theih nan MRI neih a rawt mai thei.

I doctor-in paraganglioma a vei tih a hriat hnuah, i taksa hmun dangah a darh tawh em tih enfiahna an nei leh thei ang.

Genetic Testing hi a tul em?

Paraganglioma i vei tih hmuhchhuah i nih chuan, i doctor chuan inherited syndrome i neih leh neih loh leh a kaihhnawih cancer dang vei theihna hlauhawm i nih leh nih loh hriat nan genetic counselling neih a rawt mai thei.

I doctor chuan heng thil thlengah hian genetic test neih a rawt thei ang:

• Nangmah emaw i chhungkaw zinga mi emaw hian hereditary pheochromocytoma emaw paraganglioma syndrome emaw nena inzawm symptoms i neih chuan.
• I thisenah catecholamines level pangngai aia sang i neih emaw, cancerous paraganglioma chhinchhiahna i neih chuan.
• Kum 40 i tlin hmaa paraganglioma i vei tawh chuan.

I genetic counselor-in i test result-ah gene inthlak danglamna a hmuh chuan i chhungkaw member dangte (asymptomatic ni lo mahse hlauhawma awmte pawh) te pawh test tir turin a rawt mai thei.

Paraganglioma hi engtin nge enkawl a nih?

Paraganglioma enkawl dan hi thil engemaw zatah a innghat a, chung zingah chuan:

• Tumor lian leh te.
• Tumor chu cancer (malignant) emaw, benign (benign) emaw a nih pawhin.
• Catecholamines level sang lutuk avanga symptom i neih leh neih loh.
• Tumor hi hmun khatah chauh a awm em, a nih loh leh taksa peng dangah a darh (metastasized) em?
• Tumor hi a vawi khatna atana hmuhchhuah a nih leh nih loh, a hmaa tihdam a nih hnuah a lo lang leh tawh em tih te.

Hormone tam lutuk avanga symptoms siamtu paraganglioma i neih chuan i doctor chuan chutiang symptoms control nan damdawi a pe ang che. Heng damdawi te hi a hnuaia mi ang hian a ni thei:

• I thisen sang control thei damdawi, entirnan alpha-blockers .
• I heart rate a normal level-a awm tir thei damdawi, entirnan beta-blockers .
• I adrenal gland(s) atanga hormone chhuak tam lutuk te nghawng tikhawlo thei damdawi.

Paraganglioma enkawl dan tur chu:

• Surgery hmanga tumor lakchhuah a ni.
• Radiation hmanga enkawlna a ni.
• Chemotherapy hmanga enkawl a ni.
• Ablation hmanga enkawl a ni.
• Targeted therapy hmanga enkawlna.

Nang leh i medical team te chuan nangmah leh i dinhmun nena inmil ber treatment plan chu in ruat dun ang.

Surgery hmanga tumor lakchhuah a ni

Paraganglioma enkawlna ber chu operation a ni. Tumor lakchhuahna tur surgery neih chhung hian i surgeon chuan a chhehvel tissue leh lymph node te chu tumor chu a darh tawh em tih a enfiah ang. A neih chuan surgeon chuan a natna tissue chu a la chhuak ang, a theih chuan.

Paragangliomas tam zawk hi minimally invasive technique hmangin lakchhuah theih a ni a, chu chu laparoscopic surgery . Hei hian i vunah hliam tenau tlemte siam a, hmanraw bik hmanga tumor chu lakchhuah a huam a ni. Mahse, tumor lian zawk tan chuan traditional open surgery a ngai mai thei.

Operation hnuah i doctor chuan i thisen emaw, i zun emaw-a catecholamines awm zat a enfiah ang. Catecholamines level a normal leh chuan hei hi paraganglioma cell zawng zawng lakchhuah vek a nih tawhna chhinchhiahna a ni.

Radiation hmanga enkawlna a ni

Radiation therapy hi cancer enkawlna a ni a, high-energy beam of radiation hmangin cancer cells te chu a that emaw, an thanlenna tur a titawp emaw a ni. Hei hi a chhehvela tissue hrisel tihchhiat tlem nan tih a ni.

Radiation therapy hi chi hnih a awm a, chungte chu:

• External radiation therapy: Hei hi i taksa pawn lama machine hmanga cancer awmna hmunah radiation direct a ni.
• Internal radiation therapy: Hei hian radioactive substance chu needle, chi, wire emaw catheter emaw-ah dah a ni a, chu chu doctor-in tumor chhungah emaw, a bulah emaw direct-in a dah a ni.

I doctor-in radiation therapy a rawt chi chu i cancer chu localized emaw, regional emaw, distant emaw, a lo kir leh emaw a innghat a ni. Doctor-te chuan cancer natna paragangliomas enkawlna atan external beam radiation therapy leh/ emaw 131I-MIBG therapy an hmang tam ber. 131I-MIBG therapy hi cancer cell chi hrang hrangah radioactive substance pek dan a ni a, chu chu taksaah inject a ni a, a radiation chhuah chuan cell te chu a that ta a ni.

Chemotherapy hmanga enkawl a ni

Metastatic paraganglioma enkawlna standard chu chemotherapy a ni. Hei hian cancer cell te tihhlum nan te, an inthen darh loh nan te, a nih loh leh an lo thang lian zel tur tihtawp nan emaw damdawi hman a huam a ni. Chemotherapy hi thisen kal tlanga pek a ni tlangpui. Hei hi a tlangpuiin enkawlna tha tak ni mahse, side effect a nei thei a ni.

Ablation Therapy hmanga enkawl a ni

Ablation therapy hi minimally invasive treatment option a ni a, temperature sang tak emaw, hniam tak emaw hmangin tumor tihchhiat a ni. Cancer cell leh cell pangngai lo tihlum thei ablation therapies te chu:

• Radiofrequency ablation: Hei hi radio wave hmanga cancer cell leh cell pangngai lo tihlum leh tihchhiat a ni. Radio wave te hi electrode (electric conduct tu hmanrua tenau) hmanga thawn a ni.
• Cryoablation: He enkawlna hian liquid nitrogen emaw liquid carbon dioxide hmangin cancer cell leh cell pangngai lo te chu a freeze a, a tichhia a ni.

Targeted Therapy hmanga tih a ni

Targeted therapy hi damdawi emaw thil dang emaw hmanga cancer cell bik chauh beih nan, cell hriselte tichhe lovin, enkawlna kawng khat a ni. Doctor-te chuan targeted therapy hmangin paragangliomas hla tak leh lo lang leh ṭhin chu an enkawl ṭhin.

Tunah hian zirchiangtute chuan tyrosine kinase inhibitor sunitinib an tih chu an zirchiang mek a ni.Damdawi chi khat chu distant paragangliomas a tihdam theih dan tur zirchian mek a ni. Tyrosine kinase inhibitor therapy hi targeted therapy chi khat a ni a, tumor thanna a titawp thei a ni.

Paraganglioma lo awm tur hi ven theih a ni em?

Vanduaithlak takin paragangliomas hi ven theih a ni lo. Mahse, inherited syndrome leh paragangliomas vei theihna hlauhawma siam thei gene thenkhat i neih chuan genetic counseling hian paragangliomas test tir turin a pui thei che a, a hma pawhin i man thei bawk.

I first-degree chhungte (unau leh nu leh pa) te hian paraganglioma emaw pheochromocytoma emaw an vei a nih chuan, leh/ emaw, heng genetic condition te hi i neih chuan i doctor nen inbia ang che:

• Multiple endocrine neoplasia 2 natna a ni.
• Von Hippel-Lindau (VHL) natna a ni.
• Neurofibromatosis chi khat a ni.
• Hereditary paraganglioma natna hi a ni.
• Carney-Stratakis te chu an inkawp a.
• Carney triad a ni.

Hetiang dinhmunah hian eng prognosis nge awm?

Paragangliomas, chu chu dam leh dam theihna chance hi thil engemaw zat a zirin a inang lo. Chung zingah chuan:

• I taksaa tumor awmna leh a lian zia.
• Cancer a ni emaw, taksa peng dangah a darh tawh emaw.
• Surgery hmanga tumor chu lakchhuah a ni em, a chhuah a nih chuan operation neih laiin tumor engzat nge lakchhuah?

Paraganglioma te tak te, taksa hmun danga darh lo (metastasized lo) nei te hi kum nga chhunga dam rei zat chu 95% vel a ni. Mahse, paraganglioma vei, enkawl hmasak hnua lo lang leh emaw, taksa hmun danga darh tawh (metastasized) nei te chu kum nga chhung dam rei zawng chu 34% leh 60% inkar a ni.

Tin, paraganglioma na tak tak thenkhat pawh a awm a, hla takah a darh lo a nih pawhin a chhehvel tissue-ah a darh tawk tawh avangin operation hmanga lak chhuah vek theih a ni lo. Chutiang hunah chuan adrenaline leh noradrenaline chhuak tam lutuk hi a hlauhawm thei a, enkawl harsa tak a ni thei bawk.

A pawimawh ber chu paragangliomas hi cancer emaw ni lo emaw pawh nise, enkawl loh chuan adrenaline leh noradrenaline tam lutuk a chhuah avangin harsatna lian tak, nunna atana hlauhawm tak pawh a thlen thei a ni.

Chung harsatna thenkhat chu:

• Lung ruh natna (cardiomyopathy) a ni.
• I thinlung ruh (`myocarditis`) a inflammation.
• I thluaka thisen chhuak thunun loh (cerebral hemorrhaging).
• I lungah tui a lo awm (pulmonary edema).
• Lung natna (`myocardial infarction`) a ni.
• Thai.
• Coma ah a awm.
• Thihna.

Engtikah nge Doctor ka hmuh ang?

Paraganglioma i vei tih hmuhchhuah a nih a, rinhlelh awm tak tak i neih chuan doctor pan nghal rawh.

Paraganglioma symptoms, thisen sang leh lu na te i neih chuan i doctor nen inbia ang che. Paraganglioma hi a tlem avangin thisen sang enkawl hi a la pawimawh hle a, i vei a rinawm loh pawhin thisen sang enkawl hi a la pawimawh hle.

I chhungte first-degree (unau, nu leh pa) zinga pakhatin ``Multiple endocrine neoplasia 2 syndrome`` emaw ``Von Hippel-Lindau (VHL) disease`` emaw ang chi genetic syndrome a nei tih i hriat chuan i doctor hnenah genetic testing chungchang zawt la, hei hian paraganglioma vei theihna a tipung thei a ni.

I doctor hnenah eng zawhna nge i zawh tur?

Paraganglioma i vei tih hmuhchhuah a nih chuan i doctor hnenah heng zawhnate hi zawh a \angkai thei ang:

• Engvangin nge paraganglioma ka neih?
• Ka fate leh/ emaw ka chhungte hian paraganglioma an vei thei em?
• Eng enkawlna kawng nge ka neih?
• Enkawlna hrang hrang hian eng side effect nge a neih?
• Engtin nge ka symptoms te hi ka enkawl theih ang?

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

Paraganglioma hi natna hlauhawm tak a ni a, mahse hriat reng a pawimawh. Hriatthiam harsa tak tak, a bik takin thisen sang rang tak, thawk hah, emaw, thinlung thawk chak tak i neih chuan, chu chu thil thleng mai mai anga hnawl ai chuan damdawi lam thurawn lak chu a finthlak zawk.

Paraganglioma awm chhan hi hriat chian ni lo fo mah se, thlahtu natna thenkhat nen a inzawm tlat a ni. Chuvangin i chhungkuaa mi tu emaw chuan paraganglioma emaw pheochromocytoma emaw a vei tawh a nih chuan, genetic testing hian i hlauhawmna a hriat theih nan a pui thei che a, chubakah hriselna lama harsatna dang i neih theih leh theih loh pawh a hriat theih bawk. Hemi chungchanga zawhna i neih chuan i doctor nen inbiakna neih hreh suh. I tanpui turin an lo kal a ni.

` Paraganglioma, Paraganglioma, Pheochromocytoma, Chromaffin cells, Catecholamines, Hormones, Tumors, Cancer, Symptoms, Thisen sang, Genetic Diseases