Pleuropulmonalni blastom - prikaz slučaja
DOI:
https://doi.org/10.13112/pc.1122Ključne riječi:
pleuropulmonalni blastom; neoplazme pluća; djecaSažetak
Cilj: Cilj rada je prikazati izazove u postavljanju dijagnoze i liječenju pleuropulmonalnog blastoma.
Prikaz slučaja: Petomjesečno muško dojenče hospitalizirano je zbog desnostranog pneumotoraksa u sklopu bronhiolitisa uzrokovanog respiratornim sincicijskim virusom. Slikovna obrada prikazala je bulu u desnom plućnom krilu. Nakon resekcije, patohistološkom analizom postavljena je dijagnoza kongenitalne cistične adenomatoidne malformacije pluća tipa I. U dobi od 2.5 godine, tijekom liječenja pleuropneumonije, kod pacijenta je otkrivena solidno-cistična tumorska tvorba desnog hemitoraksa. Ultrazvučno vođenom iglenom biopsijom postavljena je dijagnoza sarkoma iz obitelji Ewing. Nakon neoadjuvantne kemoterapije učinjena je desnostrana bilobektomija. Patohistološka analiza potvrdila je pleuropulmonalni blastom tipa II, a genomsko sekvenciranje patogenu varijantu DICER1 gena. Ista mutacija dokazana je i u zametnoj liniji, čime je potvrđen DICER1 sindrom. Sedam mjeseci nakon završetka terapije pacijent je bez znakova bolesti te urednog tjelesnog i psihomotornog razvoja.
Zaključak: Pleuropulmonalni blastom rijedak je i agresivan intratorakalni tumor koji treba razmotriti u diferencijalnoj dijagnozi kongenitalnih malformacija pluća, plućnih ili bronhalnih cisti te pneumotoraksa. Pravodobno prepoznavanje i multidisciplinarno liječenje, koje uključuje kirurški zahvat i kemoterapiju, ključni su za povoljnu prognozu bolesti.
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Autorska prava (c) 2025 Maja Pavlović, Iva Tkalčec, Jelena Roganović
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