
siled cutaneous syndrome

siled cutaneous syndrome (ccs) anedi iteevala moodu arudaina vaarasatvamgaa vacchina charmapu adenexal tumour syndromelaku niyaminchabadina padam, bahula kutumba trichopithelioma (mft1) (epithelioma adenoids systicum mariyu epithelioma adenoids systicum off broke anie kooda pilustaaru), broke-spiegler syndrome (bss), mariyu kutumba sthuupaakaaramu (effsy). charmapu adenexal kanitulu anevi ververu kanajalalanu kaligi unna charma kanitula pedda samuuham (i.ii. muula kanaala nundi paripakvam chendina) saadhaarana charmamlo kanipinche naalugu praadhamika adenexal nirmaanaalalo okadaaniki juttu kudullu, sebasius chemata grandhulu, apocrine chemata grandhulu mariyu acrine chemata grandhulu. sisi kanitulu anevi ventrukala kanitulu.
mft1, bss, effsy roopaala sisi unna vyaktulu germline (ii.ii. oka vyaktiki daariteese suukshmakrimula kanaalalo unde) vaati rendu "siled" (i.ii. siled lysine 63 dubiquitase) janyuvulu. eee vyaktulu charma kanitulanu kaligi untaaru, ivi mft1, bss mariyu/ledhaa effsy rakaluga samuuhamai untaayi, ivi vaari sthaanaalu, samsthalu mariyu suukshmadarshini roopaalalo okadaanikokati bhinnamgaa untaayi. edemaina, sisi latho unna oke kutumbamloni sabhyulu effsy-, mft1-ledhaa bss-rakam namuunaanu vyaktam cheyavachu. inca, eee vibhina namuunaalu sii. sii. latho unna vyaktula rooga niruupana ledhaa korsupai takkuva ledhaa etuvanti prabhavanni chuupavu. mft1, bss, ledhaa effsy hair follicular tumourlu unna vyaktulaku vartinche "siled cutaneous syndrome" anae padaanni modata rajan mariyu itarulu pratipaadinchaaru. 2009loe.
sii. sii. latho unna vyaktulu saadhaaranamgaa vaari yavvanamlo praarambhamai, vaari jeevitaallo ekkuva bhaagam konasaagutuu nirapaayamaina charma kanitula sankhyanu penchutaaru. asaadhaarana sandarbhaallo, vaaru praanaantaka kanitulanu abhivruddhi chestaaru, veetilo chaalaa varaku vaari nirapaayamaina kanitula nundi utpannamavutaayi. aamodayogyam kaani rogalakshana, atyanta vikruteekarinche ledhaa praanaantakamaina kanitulaku shastrachikitsa paddhatulu mariyu praanaantaka ledhaa itara madhyastha parigananala sandarbhaalalo, radiation therapy dwara chikitsa chestaaru. shastrachikitsa dwara cherukoleni kanitula sandarbhaalalo radiation therapyni matrame upayoginchavacchu. praanaantaka kanitula abhivruddhini tanikhi cheyadaaniki sisi unna vyaktulandaruu vaarshikamgaa ledhaa marinta tarachugaa phaalo-up pareekshalu cheyinchukovali. sisi unna vyaktulaku vaari sannihita kutumba sabhyulatho paatu lothaina janyu salahaku praapyatanu andinchaali. eee kanitulaku mandulatho sahaa uttama chikitsalanu kanugonadaaniki parisoedhana avasaram.
pradarsana.
sii. sii. latho unna vyaktulu saadhaaranamgaa eee vyaadhi yokka kutumba charitranu kaligi untaaru mariyu vaari thala mariyu mondem meeda tarachugaa bahula (konnisaarlu 100 kante ekkuva) nirapaayamaina juttu kudulla kanitulato untaaru. kanitulu saadhaaranamgaa yuktavayassu vadda praarambhamavutaayi mariyu 1 samvatsaram vayassu unna pillalalo sambhavinchinappatikii yuktavayassu antataa kramamga perukupotayi. teevramaina sandarbhaallo, kanitulu chaalaa varaku vikruteekaristaayi, chaalaa varaku talapai, mukham mariyu/ledhaa itara juttu mose pradaesaalaina pabik praantam antataa vistarinchi untaayi, mariyu asadhaaranamgaa praanaantakamainavi mariyu charmarahita pradaesaalaku vyaapti chendavacchu. milia, ii.ii. muusukupooyina acrine chemata grandhula valla erpade aspashtamaina chinna charma tellati macchalu, sii. sii. laloe kooda sambhavinchavachhu mariyu konnisaarlu oka vyaktiki sii. sii. luu unnayani matrame suuchistaayi; eee vyaktulu jeevitamlo taruvaata kanitulanu abhivruddhi cheyavachu mariyu puurtigaa perigina vyaadhitho pillalanu kaligi unde pramaadam undhi. sii. sii. la pradarsanalu vaati kanitula suukshma hystopathologylaku sambandhinchinavi (tadupari vibhaganni chudandi). ii.ii. vaari kanitulu kutumbetara sthuupaakaaraalu, spiradenomas mariyu/ledhaa trichopitheliomasnu pooli undavacchu. sthuupaakaaram lanty sii. sii. sii kanitulu mruduvaina nodular masga kanipistaayi, ivi saadhaaranamgaa gulabi rangulo untaayi, apaaradarsakamgaa undavacchu, uparitala rakta naalaalanu kaligi undavacchu, chaalaa samvatsaraalugaa kramamga perugutaayi mariyu aneka sem. mea. (i.ii. centimeters) parimaanamlo. eee kanitulalo rendu ledhaa anthakante ekkuva kanitulu vileenam ayi chaalaa pedda parimaanamlo kanitulanu yerparustaayi. spiradenoma lanty sii. sii. ess. kanitulu kooda nodular kanitulugaa kanipistaayi, conny ivi neelam/nalupu rangulo undavacchu, badhakaramga, vaegamgaa perugutaayi mariyu sthuupaakaaraala vale, pedda-parimaana kanitulanu roopondinchadaaniki vileenam kaavacchu. sthuupaakaaram lanty mariyu spiradenoma lanty kanitulu (i.ii. spiradenocylindromas) oke kanitilo sambhavinchavachhu. trichopithelioma lanty sii. sii. kanitulu charmam-rangu, chinna, populesga kanipistaayi, ivi saadhaaranamgaa mukku chuttoo charmamlo, nasolabial madata mariyu/ledhaa nuditi chuttoo untaayi. european puurveekula vyaktulalo, popules saadhaaranamgaa 0.3-0.4 sem. mea. parimaanamlo untaayi, conny african, bharatiya mariyu chainees puurveekula vyaktulalo peddavi kaavacchu.
sisi la yokka mft1 roopam saadhaaranamgaa bahula trichopithelioma-lanty kanitulato untundi, bss roopam sthuupaakaara-, spiradenoma-mariyu/ledhaa trichopithelioma-lanty kanitulu ledhaa popules thoo untundi, mariyu effsy roopam saadhaaranamgaa bahula sthuupaakaara-vanti popules thoo untundi. asadhaaranamgaa, sii. sii. latho unna vyaktulu vaari nirapaayamaina kanitula lopala ledhaa sameepamlo sambhavinche praanaantaka kanitulanu abhivruddhi chestaaru; avi pradhanamgaa sthuupaakaara, spiradenomas, trichopitheliomas kuu praanaantaka pratiruupaalu mariyu varusaga cylindrocarcinomas, spiradenocarcinomas mariyu trichoblastic carcinomas anie pilustaaru. eee kanitulalo konni besal-sell carcinomas ledhaa adenocarcinomasnu pooli untaayi. eee praanaantaka kanitulu lalajala grandhulu (i.ii.lalajala grandhi yokka besal sell adenocarcinomas), kaaleyam, oopiritittulu mariyu/ledhaa emukalu. praanaantaka sii. sii. kanitulu vruddhulalo ekkuvagaa sambhavistaayi mariyu peddavigaa untaayi (antey.ii. vaati nirapaayamaina pratyardhula kante 17.5 centimeters varaku) untundi.
kutumbetara sthuupaakaaraalu, spiradenomas, spiradenocylindromas mariyu vaati praanaantaka pratiruupaalu oke vivikta kanitigaa ledhaa, takkuva saadhaaranamgaa, bahula kanitulugaa untaayi. eee kanitulu chedurumadurugaa untaayi (i.ii. vaarasatvamgaa raaledu) mariyu "siled" janyu parivartananu kaligi unna kanaalanu kaligi undavacchu ledhaa kaligi undakapovachhu. okavela unte, utparivartana chendina "siled" janyuvu yokka sthaanam kaniti kanaalaku parimitam cheyabadutundi. eee chedurumaduru kanitulu sii. sii. la yokka aavirbhavaalu ledhaa nirdhaarana kaadu.
hystopathology.
hematoxylene mariyu eosine-tadisina namuunaala suukshma hystopathological roopaanni batti nirnayinchinatlugaa, sisi kaniti kanajaalaalu chedurumaduru sthuupaakaaraalu, spiradenomas ledhaa trichopitheliomasnu pooli untaayi. sthuupaakaaram lanty sii. sii. sii. kanitulu anevi charmamloki vistarinche naan-encopsulated nodular gayalu, ivi besal kanaalanu kaligi untaayi (i.ii. charmam yokka epidermis yokka atyalpa poralo kanipinche maadirigaane chinna, gundrani kanaalu), mariyu mandapati neelamaaliga poralu vaeru chesina sthuupaakaara namoonaalalo ("jigsa lantivi") amarchabadi untaayi. spiderdenoma lanty sii. sii. sii. kanitulu saapekshamgaa avyavastheekruta mariyu dattamaina vistarana basophilic kanaala shrenini kaligi untaayi (i.ii. hematoxylene steyn asadhaaranamgaa pedda mottamlo teesukovadam valla kanaalu neelam rangulo kanipistaayi). lymphocytelu saadhaaranamgaa eee kanitula kanajalalanu janasaandrata penchutaayi. konni sii. sii. s. kanitulu (i.ii. spiradenocylindromas) sthuupaakaaraalu mariyu spiradenomas yokka hystopathological lakshanaalanu vileenam chestundi. trichopithelioma lanty sii. sii. sii. popules unifam basaloid kanaala dveepaalu mariyu teegalatho koodi untaayi (i.ii. fibrus stromalo charma ankurotpatti kanaalanu pooli unde cuboid aakarapu kanaalu), juttu papillanu pooli unde epithelial nirmaanaalu ledhaa asampuurnamgaa erpadina juttu kudullu, chinna caratosistlu (i.ii. charmapu tittulu) stareekruta squamous epithelium mariyu calcification yokka kendraalato kappabadi untaayi. baseloid kanaalu saadhaaranamgaa roganirodhaka vyavasthalo 4 pratirakshakaanni upayoginchi vislaeshanha dwara kanugonabadina epithelial sell samslaeshanha anuvunu vyaktam chestaayi. trichopithelioma lanty sii. sii. kanitula kanajaalamlo sii. keey. 20 proteinnu vyaktam chese merkel kanaalu kooda undavacchu. sthuupaakaara cancer mariyu spiradenocarcinoma kanitulu, vaati nirapaayamaina pratiruupaala maadirigaa kakunda, takkuva-sthaayi ledhaa adhika-sthaayi, asadhaaranamgaa kanipinche kanaalu vaegamgaa vistaristaayi (udaaharanaku, kana vistarana yokka adhika sthaayini choopinche kanaala dwara nirvachinchabadinatlugaa, kanaala protein marker yokka adhika sthaayilu), maib proteinnu vyaktam cheyakapovachhu, mariyu, spiradenocarcinoma kanitulaku sambandhinchi, saadhaaranamgaa lymphocytelu undavu. trichoblastic carcinomalu hypercellular, fibrus, naan-mixoid stromatho kuudina vaati nirapaayamaina pratiruupaala nundi bhinnamgaa untaayi, ivi tarachugaa basal sell carcinomaluga gurtinchabadataayi mariyu adhika-sthaayi kanitulalo necrosis yokka vistrutamaina praantaalu untaayi (i.ii. chanipoyina kanajaalam); eee kanitulalo kanaalu vaegamgaa vistaristunnaayi.
janyusaastram.
ccs anedi athani ledhaa aame rendu "siled" janyuvulalo okadaanilo nishkriyaatmaka utparivartananu kaligi unna tallidandrula nundi vaarasatvamgaa vacchina autosomal dominant desorder. mutation anedi juttu kudulla muula kanaalanu sthuupakanaalu-, spiradenoma-, trichopithelioma-mariyu/ledhaa spiradenocylindroma-vanti gayalanu yerparuchukune kanaalugaa vaeru cheyadaaniki kaaranamavutundi. chochukupovadam (i.ii. vyaadhi lakshanaalanu pradarsinche sii. sii. latho unna vyaktula saatam vividha adhyayanaalalo 44 saatam nundi <ii. dii. 1> (mottam sagatu, 72 saatam) varaku undhi. ayithe, eee adhyayanalu saadhaaranamgaa "siled" janyuvu dwara encode cheyabadina marnah yokka vyakteekarana kosam daani unikini anchanaa vaeyadaaniki rakta leukocyte namuunaalanu pareekshistaayi. iteevali adhyayanalu eee paddhati dwara gurtinchabadani aneka sisi kesulalo utparivartanaalu unnayani kanugonnayi, endukante avih 1) mosaic utparivartanalu ivi raktamlooni leukocytelaku "siled" nuu pampinhee chestaayi, conny juttu kudullaku takkuva ledhaa aemee pampinhee cheyavu; 2) lothaina intronic utparivartanalu (i.ii. siled" janyuvu yokka intraanlalo lothugaa sambhavinche utparivartanaalu) kriyaaseela protein kosam code cheyani conny "saadhaarana" siled marnaga gurtinchabade marnas erpadataaniki daariteestaayi; mariyu 3) vividha kanajaalaalo mariyu lopala vistrutamgaa maarutuu unde siled marnah sthaayila abhivruddhiki daariteese sankhya vaividhyam utparivartanalu coppy cheyandi mariyu raktamlo leukocytela namuunaalu saanukuula phalitaalanu ivvadaaniki taginanta ekkuvagaa undavacchu, conny juttu kudullalo kaniti erpadataanni nivaarinchadaaniki chaalaa takkuvagaa untaayi. rakta rakta kanitula kante charma kanitulanu vislaeshimchae janyu pareekshalu, rendu ledhaa anthakante ekkuva charma kanitulu ledhaa rakta rakta kanitula namuunaalanu vislaeshimchaayi mariyu/ledhaa "siled" janyuvu yokka sunnitamaina tadupari taram sequencingnu upayoginchina janyu pareekshalu "siled" janyu utparivartanaalanu nishkriyam cheyadaaniki aadhaaraalanu kanugonnayi, lekapothe "siled" janyuvu sanukoolamgaa pariganinchabadutundi mariyu anduvalla ccs kaadu.
sisi latho unna 14 mandhi vyaktula iteevali adhyayanalu vaari kaniti kanaalu rendu "siled" janyuvula vyakteekarananu kolpoyayani suuchistunnaayi. sisi kaniti kanaalalo eee double janyu mutation anni nirapaayamaina mariyu konni praanaantaka sisi kanitulalo kanugonabadindi, idhi sisi laloe sthiramaina lakshanamgaa undavacchani suuchistumdi. eee drushtilo, sii. sii. latho unna vyaktulu oka "siled" janyuvulo vaarasatvamgaa vacchina, germline mutationnu kaligi untaaru, idhi sareeram antataa kanaalaku vistrutamgaa pampinhee cheyabadutundi mariyu vaarasatvamgaa pondabadadu, rendava "siled" janyuvulo pondina mutation (heterozygosityni chudandi) vaari sii. sii. kaniti kanaalaku parimitam cheyabadindi. sisi kanitula abhivruddhiki juttu kudulla muula kanaalalo rendu "siled" janyuvula nashtam avasaram kaavacchu.
"siled" janyuvu yokka charya yokka vidhanam.
siled" janyuvu chromosome 16 yokka podavaina (ledhaa "q") bhujampai 12.1 bandlo undhi. idhi kaniti anichivese janyuvugaa vargeekarinchabadindi, antey.ii. kanaala perugudalanu niyantrinche janyuvu mariyu mutation dwara nishkriyam cheyabadinappudu aniyantrita kana perugudala mariyu kanitula erpadataaniki daariteestundi. nishkriyaatmaka "siled" janyu utparivartanalu tii-sell acute lymphoblastic leukemia, multiple myloma, hepatocellular carcinoma, neuroblastoma, pancreatic cancer, garbhasaya cancer, kadupu cancer, peddapregu cancer, oopiritittula cancer mariyu maanava papillomavirus-sambandhita cancerlalo sambhavistaayi. sisi laloe 95 utparivartanaala adhyayanamlo, 48 saatam frame shift, 27 saatam ardhamlenivi, 12 saatam missense, mariyu 11 saatam splies sait utparivartanalu. asadhaaranamgaa, sii. sii. latho unna vyaktulu lothaina antaryuddha utparivartanalanu kaligi untaaru (i.ii. exon-intron sarihaddu nundi 100 kante ekkuva bays jatala dhooramlo unna intraanlo sambhavinche utparivartanalu) ledhaa chaalaa ledhaa anni "siled" janyuvulanu tolaginche pedda ettuna utparivartanalu. eee utparivartanalanni utparivartanalanu nishkriyam chestunnayi.
siled" janyuvu yokka encode cheyabadina utpatti, siled lysine 63 dubiquitase protein (siled protein), oka dubiquitating enjaim, i.ii. konni proteinla nundi ubiquitin nuu tolaginchi, tadwara eee proteinla karyakalapalanu niyantrinche protease. siled protein nf-kebi, doubluenty, naach, tgf-bii, mariyu jnk sell signalling maargaalanu niyantrinchadamlo palgone proteinla nundi ubiquitin nuu tolagistundi; eee margalu saadhaaranamgaa juttu nirmaanam, kana perugudala, kana manugada, taapajanaka pratispandanalu mariyu/ledhaa kaniti abhivruddhini niyantrinchadaaniki panicheestaayi. nf-kebi signalling yokka siled protein niyantrana kolpovadam anedi sisi kanitula abhivruddhiki keelaka dohadam cheyavachu. kanaala yokka cytosol loe daani kriyaarahita sthaanam nundi kana kendrakaaniki nf-kebi kadalikanu prothsahinche proteinlanu sarvavyaapti cheyadaaniki aneka rakaala uddeepanalu kaaranamavutaayi. nf-kebi anedi oka transcription karakam, idhi kendrakamlo unnappudu vividha janyuvula vyakteekarananu prerepistundi, idhi apoptosis mariyu necroptosis dwara kana maranaanni prothsahistundi mariyu kana perugudalanu nirodhistundi. siled protein nundi ubiquitines vidudala avtayi mariyu tadwara proteinlanu (udhaa.zii. ikbg, nemo protein anie kooda pilustaaru) idhi kendrakaaniki cytosolic nf-κb kadalikanu prothsahistundi. sisi kaniti kanaalu nf-kebi kadalikalapai eee samyamanam kaligi undavani, anduvalla asadhaaranamgaa deerghakaalika manugada, adhika vruddhi raetlu mariyu bahusha kaniti erpadataaniki dohadapade itara lakshanaalanu kaligi untaayani pratipaadinchabadindi.
rooga nirdhaarana.
sisi la nirdhaarana daani kanitula pradarsanalu mariyu suukshma hystopathologylu mariyu sisi la yokka kaniti beararla kutumba charitrala dwara gattiga suuchimchabadutumdi. rooga nirdhaarana nirdhaaranaku "siled" janyu utparivartanalanu nishkriyam cheyadaanni gurtinchadaaniki janyu pareeksha mukhyam. praanaantaka sii. sii. kanitulu vyaktula vyaktigata mariyu kutumba charitra, metastases uniki mariyu praanaantaka mariyu metastatic kanitula suukshma hystopathologyla aadhaaramgaa nirdhaarana cheyabadataayi. rooga nirdhaarananu nirdhaarinchadaaniki metastatic kanitulanu "siled" utparivartanala kosam neerugaa pareekshinchavacchu. cylindromas, spiradenomas, trichopitheliomas ledhaa vaati praanaantaka pratyardhula yokka chedurumaduru kesula nundi sisi lanu vaeru cheyadaaniki, "siled" janyuvu kosam pareekshanu kaneesam oka kanitini hystologicalga dhruveekarinchina rendu ledhaa anthakante ekkuva cylindromas, spiradenomas ledhaa trichopitheliomas ledhaa 2) oke (aadarsamgaa hystologicalga dhruveekarinchabadina) cylindroma, spiradenoma ledhaa trichopithelioma mariyu janyuparamaina ledhaa hystopathological moolyaankanala dwara dhruveekarinchabadina sisi la kutumba charitra kaligina vyaktulalo nirvahinchaali. rakta rakta kanajaalaala janyu pareeksha aadhaaramgaa saadhaarana "clyde" janyuvu unikini choopinche vyaktulalo, kaniti kanajaalam yokka janyu pareeksha anedi sii. sii. la nirdhaarananu nirdhaarinchagaladu. eee paddhatulu siklanu chedurumaduru sthuupaakaaraalu, spiradenomas, trichopitheliomas mariyu vaati praanaantaka pratiruupaala nundi matrame kakunda, burt-hogg-dube syndrome, tuberose sclerosis, neurophybromatosis, couden syndrome, maerii-unnaa hypotrichosis mariyu bahula scalp trichelem malabaddhakam vanti vividha charma kaniti rugmatala nundi kooda vaeru chestaayi.
chikitsa mariyu rooga niruupana.
sii. sii. ess anedi vaarasatvamgaa vacchina, jeevitaantam unde, kutumba vyaadhi, idhi prastutam nayam cheyalenidi. eee janyuvulo nishkriyaatmaka utparivartananu kaligi laeni vaatini gurtinchadaaniki sisi kutumbaala sabhyulandarinee "siled" janyuvu kosam pareekshinchaali. vyaadhi yokka kaneesa aadharalu unna sandarbhaallo eee janyuvu kosam pareeksha mukhyamgaa mukhyamainadi (udhaa.zii. milia, pradarsana vibhaganni chudandi) mariyu mosaic, lothaina intronic ledhaa coppy number vaividhyam utparivartanalu unna vyaktulalo rakta leukocyte namuunaalanu pareekshinchadaaniki badulugaa kanitini pareekshinchaalsina avasaram undavacchu (janyu vibhaganni chudandi). nishkriyaatmaka kutumba "siled" janyu utparivartananu kaligi unnatlu kanipinche vyaktulandaruu janyu salahanu pondaali, indulo vyaktulu, kutumbaalu mariyu taraala madhya sisi teevratanu anchanaa vese saamardhyamlo parimitulapai samacharam; kutumba pranaalika; pri-implantation janyu nirdhaarana; mariyu phaalo-up moolyaankanam avasaram. sii. sii. latho unna vyaktulu puurti charmam mariyu bahusha itara avayavalanu pondaali (udhaa.zii. lalajala grandhi) pareekshalu vaarshikamgaa saadhaarana sandarbhaalalo nirvahinchabadataayi, prati 2-3 nelalaku okasari vaari kanitulu vaegamgaa perugutaayi ledhaa aneka kotta charma kanitulu erpadataayi, mariyu ventane vaari kanitulu praanaantaka sankethaalanu abhivruddhi chese sandarbhaalalo (udhaa.zii. kaniti pundu, chaalaa vaegamgaa perugudala, noppi, adapadadapa raktasraavam, rangu maarpulu, ledhaa antarleena emukaku tethering ledhaa oopiritittula metastagelanu pratibimbimchae swaasa teesukovadamlo ibbandi vanti avayava panicheyakapovadam vanti sanghatanalu akasmaattugaa praarambhamayye sandarbhaallo).
nirapaayamaina sii. sii. ess. kanitulu.
nirapaayamaina sii. sii. sii. kanitula chikitsalu aamodayogyam kaani lakshanaalanu kaliginche kanitulanu tolaginchadampai nirdesinchabadataayi (udhaa.zii. noppi, vranotpatti, raktasraavam, vikruteekarana, laingika panicheyakapovadam ledhaa chevi kaaluva vanti maargam yokka moosukupovadam). eee tolagimpula paddhatulalo shastrachikitsa tolagimpu, curatage, dermabration, cryotherapy ledhaa carbon dioxide, orgaon ledhaa lazer shastrachikitsato vidyudvislaeshanha unnaayi. soundarya ledhaa kriyaatmaka parigananala dwara suuchimchabadina chota, eee chikitsalanu plaastik mariyu/ledhaa flap shastrachikitsa (i.ii. rakta sarafaraatho saadhaarana kanajaalam chekkuchedarakunda daata sait nundi ettiveyabadutundi mariyu shastrachikitsa saitku taralinchabadutundi). eee chikitsalu tarachugaa aneka kanitulu, punaraavruta kanitulu ledhaa kottagaa erpadina kanitulu unna vyaktulalo padepade upayoginchabadataayi; chikitsalu kooda vistrutamgaa undavacchu, udaaharanaku, sii. sii. latho 4 mandiloo 1 varaku vaari scalpsnu puurti shastrachikitsa tolaginchadam avasaram.
praanaantaka sii. sii. sii. kanitulu.
praanaantakamainavi ledhaa andubaatulo unde metastatic kanitulugaa telisina ledhaa anumaaninchabadina praathamika sisi kanitulaku prastutam vistruta sthaanika tolagimpulatoe chikitsa chestaaru, taruvaata tarachugaa sahayaka radiotherapy chestaaru. anni praanaantaka kanaalanu tolaginchadaaniki vistruta sthaanika tolagimpu upayoginchabadutundi, ayithe shastrachikitsa tarvaata migili unna edainaa praanaantaka kanaalanu champadaaniki sahayaka radiotherapy upayoginchabadutundi. praanaantaka sii. sii. kanitulu asadhaaranamgaa suduura, charmarahita pradaesaalaku vyaapti chendutaayi. hystopathologicalga nirvachinchabadina takkuva-sthaayi kanitulu vaati adhika-sthaayi pratiruupaala kante takkuva tarachugaa metastasize avtayi. praanaantaka sii. sii. kanitula chikitsapai gatha adhyayanalu tarachugaa cherchabadinanduna, conny sporadic mariyu family cylindrocarcinoma, spiradenocarcinoma mariyu trichoblastic carcinoma kanitula madhya spashtamgaa thedaanu gurtinchaledu mariyu praanaantaka sii. sii. kanitulu chaalaa arudu kabatti, praanaantaka sii. sii. kanitula saatampai spashtamaina dayta ledhu, ivi punaraavrutamavutaayi, metastasize avtayi mariyu praanaantakam avtayi. ayithe, spashtamgaa, ccs praanaantaka kanitulu eee avaanchaneeya phalitaalalo prati okkati utpatti cheyagalavu. sii. sii. la praanaantaka kanitulaku uttama chikitsa niyamaalu aspashtamgaa unnaayi mariyu marinta adhyayanam avasaram.
sii. sii. kanitulaku aushadha chikitsalu.
vichaarana adhyayanalu sisi kanitulapai nf-kebi marganni nirodhinche aushadhaala chikitsa prabhavalanu pareekshinchaayi. eee mandulalo samayochita (i.ii. neerugaa kanitulaku) aspirin ledhaa itara salycylic aamlam lanty mandulaku vartinchandi. maroka aushadha pareeksha adhyayanamlo, truck graahakaala nirodhakam, samayochita pegcontratinib, adevidhamgaa pareekshinchabadindi. eee chikitsalalo edhee appudappudu swalpakaalika prabhaavaalaku minchi gananeeyamaina kaniti-nirodhaka charyalanu kaligi undadani niroopinchabadaledu. sisi kanitulato unna oka vyaktiki smooth receptor yokka inhibitor ayina vismodegibto chikitsa cheyabadindi, tadwara headjag signalling maargam mariyu rendava vyaktiki oral aspirin plus adalimamab (tumour necrosis phyaaktar alphanu tatastheekarinche monoclonal antibody) thoo chikitsa cheyabadindi. aspirin plus adalimab thoo chikitsa pondina vyakti thana bahula metastatic kanitulalo etuvanti merugudalalu chuupimchanappatikii, eee iddaru vyaktulu akkada charma kanitulalo nemmadigaa abhivruddhi chendutunna, niraadambaramaina merugudalalanu chuupimchaaru. sii. sii. kanitulaku chikitsa cheyadamlo eee ledhaa itara mandulu prabhaavavantamgaa unnaya anie nirnayinchadaaniki tadupari adhyayanalu avasaram.

