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Paratiroidni karcinom
Klinički centar Srbije, Klinika za endokrinologiju dijabetes i bolesti metabolizma, Beograd

e-adresadrmariamiletic@gmail.com
Ključne reči: paratiroidni karcinom; primarni hiperparatiroidizam; hiperkalcemija
Sažetak
Paratireoidni karcinom (PC) je neuobičajena maligna bolest, u literaturi je zabeleženo manje od 1.000 slučajeva, pošto ga je de Quervain 1904. godine opisao po prvi put kao nefunkcionalni metastatski karcinom. Sa procenjenom prevalencijom od 0,005% svih karcinoma PC je najređi endokrini karcinom i čini tek 0,5-5% svih slučajeva primarnog hiperparatireoidizma (PHPT). Patogeneza PC nije poznata. etiologija PC-a je nepoznata i nema dokaza da PC nastaje transformacijom već postojećih paratiroidnih lezija. Aberantni profil ekspresije mikroRNK i metilacije nedavno identifikovanih putem PCR-a pružaju dokaze da su maligni oblici paratireoidne žlezde različiti od benignih lezija. PC može biti sporadičan ili biti deo genetskog sindroma. Sporadični PC je najčešće povezan sa izlaganjem spoljnoj radijaciji, a retko sa sekundarnim i tercijarnim HPT zbog hronične bubrežne insuficijencije. Takođe je zabeležena udruženost PC-a, sinhronog ili metahronog sa istorijom hiperplazije PT žlezda, paratiroidnog adenoma (PA) ili karcinoma štitne žlezde sa istovremenim PA. PC se obično manifestuje indolentnim, ali upornim kliničkim simptomima; pacijenti često pokazuju simptome i komplikacije teškog PHPT-a kao što su anksioznost, depresija, slabost, gubitak težine, bolesti kostiju i bubrega, bolovi u stomaku, mučnina, pankreatitis i peptični ulkus [4, 6, 8, 44]. U prezentaciji 50% pacijenata pokazuje manifestacije bubrežne i koštane patologije, uključujući osteopeniju, osteoporozu, osteofibrozu, osteitis fibrosa cistica, subperiostalnu resorpciju i patološke frakture. Bubrežna bolest se manifestuje uglavnom kao nefrolitijaza i bubrežna insuficijencija sa prevalencijom od 56, odnosno 84%. Primećeno je da su nivoi kalcijuma u serumu često viši od 3,5 mmol / l (nasuprot <2,8 mmol / l kod benigne bolesti), dok su nivoi PTH u serumu obično 3 do 10 puta veći od gornje normalne granice (benigna PT bolest može pokazivati umereniji porast), iako ne postoji dogovoreni prag nivoa PTH i nivoa kalcijuma u serumu za definisanje PT maligniteta. Kao i kod benigne bolesti, vizuelizacija je neophodna za lokalizaciju tumora, ali ne može pouzdano razlikovati benignu od maligne bolesti. Više metoda snimanja, najčešće kombinacija MIBI i ultrazvučnog pregleda vrata, povećava dijagnostičku osetljivost i tačnost. Neke ultrazvučne karakteristike mogu predvideti malignitet i pomoći u identifikovanju patoloških limfnih čvorova ili invazije na obližnje strukture: veličina > 3 cm treba da izazove sumnju na PC. Opisani su intraoperativni nalazi suspektni na PC. U većini serija, medijana maksimalnog prečnika PC je 3-3,5 cm (<10% veći od 4 cm), u poređenju sa približno 1,5 cm za PA. Izvešteno je da 21% karcinoma ima cističnu komponentu. Nijedan univerzalni sistem kliničkog i patološkog stadijuma za PC nije usvojen, jer ne postoji korelacija prečnika tumora ili statusa limfnih čvorova sa preživljavanjem, a bolest se veoma retko dijagnostikuje preoperativno ili čak intraoperativno. Schulte i saradnici su predložili sistem određivanja na osnovu dve klasifikacione šeme, diferencirane klasifikacije kao u okviru TNM klasifikacije i klasifikacije visokog rizika/ niskog rizika koja izgleda da pokazuje značajnu moć predviđanja za preživljavanje ili recidiv. Istorijski gledano, skoro 96% pacijenata sa PC je lečeno hirurški, a hirurška intervencija je jedina efikasna terapija za kontrolu hiperkalcemije, kako u početnoj resekciji tako i u vreme ponovne pojave metastaza. Optimalno lečenje hiperkalcemije je važno pre operacije. Prikazana je pacijentkinja sa dugo neprepoznatim primarnim hiperparatiroidizmom uprkos ispoljavanju klasičnih znakova i simptoma oboljenja: bilateralne nefrokalkuloze, hipertenzije, pojačane žeđi, opstipacije, depresivnosti, bolova u lumbalnom delu kičme. Po prijemu u Kliniku za endokrinologiju i bolesti metabolizma učinjene su biohemijske analize: eGFR: 46 ml/min/1,73 m2, kalcijum: 3.34mmol/l [2.15-2.65], kalcijum, jonizovani:1.89mmol/l [1.15-1.35], fosfor: 0.74 mmol/l [0.8-1.55], magnezijum: 0.72 mmol/l, PTH 1276 ng/l, vitamin D 14.2 nmol/l. Nakon hitne terapije hiperkalcemije bisfosfonatima i.v., pacijentkinja je upućena na hirurško lečenje s obzirom na ispunjavanje operativnih kriterijuma. Učinjena je ekstrakcija gornje desne PT žlezde, a histopatološki nalaz je ukazao na paratiroidni karcinom.
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O članku

jezik rada: srpski
vrsta rada: neklasifikovan
DOI: 10.5937/medgla2181077M
objavljen u SCIndeksu: 01.07.2021.
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