DIFUZNI INFANTILNI HEPATALNI HEMANGIOENDOTELIOM: PRIKAZ BOLESNIKA

Vaskularni tumori najčešći su benigni tumori jetre, a među njima najčešći je infantilni hepatalni hemangioendoteliom (IHHE), koji dobro reagira na terapiju i ima nisku stopu mortaliteta. U rijetkim slučajevima IHHE mogu biti rezistentni na terapiju te svojom veličinom i volumenom krvnog protoka mogu...

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Published inLiječnički vjesnik Vol. 135; no. 9-10
Main Authors Sjekavica, Ivica, Petrovečki, Marko, Šunjara, Vice, Pavlović, Maja, Štern Padovan, Ranka
Format Journal Article
LanguageCroatian
English
Published Hrvatski liječnički zbor 30.10.2013
Subjects
Hemangioendoteliom – dijagnoza, komplikacije, terapija, mortalitet
Hemangioendothelioma – diagnosis, complications, therapy, mortality
Jetreni tumori – dijagnoza, komplikacije, terapija, mortalitet
Liver neoplasms – diagnosis, complications, therapy, mortality
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Abstract Vaskularni tumori najčešći su benigni tumori jetre, a među njima najčešći je infantilni hepatalni hemangioendoteliom (IHHE), koji dobro reagira na terapiju i ima nisku stopu mortaliteta. U rijetkim slučajevima IHHE mogu biti rezistentni na terapiju te svojom veličinom i volumenom krvnog protoka mogu dovesti do kardiorespiratorne dekompenzacije i smrti. U ovom radu prikazuje se tromjesečno muško dojenče koje je klinički obrađeno zbog učestalih respiracijskih infekcija, bronhoopstrukcija i tumorske tvorbe u trbuhu. Slikovnim prikazima (CT, MR i DSA) prikazao se morfološki tipičan difuzni IHHE koji je bio dokazan patohistološkom biopsijom. Tumor je bio rezistentan na standardnu medikamentnu terapiju, svojom je veličinom uzrokovao kardiorespiratornu dekompenzaciju i arest sa smrtnim ishodom u dobi od 13 mjeseci. U rijetkim slučajevima tumora rezistentnih na lijekove trebalo bi učiniti transplantaciju jetre što ranije, prije razvoja kardiorespiratornih komplikacija.
AbstractList Vaskularni tumori najčešći su benigni tumori jetre, a među njima najčešći je infantilni hepatalni hemangioendoteliom (IHHE), koji dobro reagira na terapiju i ima nisku stopu mortaliteta. U rijetkim slučajevima IHHE mogu biti rezistentni na terapiju te svojom veličinom i volumenom krvnog protoka mogu dovesti do kardiorespiratorne dekompenzacije i smrti. U ovom radu prikazuje se tromjesečno muško dojenče koje je klinički obrađeno zbog učestalih respiracijskih infekcija, bronhoopstrukcija i tumorske tvorbe u trbuhu. Slikovnim prikazima (CT, MR i DSA) prikazao se morfološki tipičan difuzni IHHE koji je bio dokazan patohistološkom biopsijom. Tumor je bio rezistentan na standardnu medikamentnu terapiju, svojom je veličinom uzrokovao kardiorespiratornu dekompenzaciju i arest sa smrtnim ishodom u dobi od 13 mjeseci. U rijetkim slučajevima tumora rezistentnih na lijekove trebalo bi učiniti transplantaciju jetre što ranije, prije razvoja kardiorespiratornih komplikacija.
Abstract_FL Infantile hepatic hemangioendothelioma (IHHE) is the most common benign hepatic tumor in children (12% of hepatic tumors), that usually responds well to therapy and has low mortality rates. In extremely rare cases of diffuse tumors resistant to therapy, size and blood flow can lead to cardiorespiratory failure and death. A 3-month-old male infant presented with respiratory infections and an abdominal mass, with frequent broncho-obstructive attacks. The CT, MRI and DSA exams showed typical morphology of hemangiendothelioma that was proven on histopathological biopsy. The tumor was resistant to standard medical therapy, and its volume led to cardiopulmonary arrest and death at 13 months of age. In rare cases of diffuse therapy-resistant IHHE a liver transplantation should be considered as an earlier treatment.
Author Šunjara, Vice
Sjekavica, Ivica
Petrovečki, Marko
Štern Padovan, Ranka
Pavlović, Maja
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Snippet Vaskularni tumori najčešći su benigni tumori jetre, a među njima najčešći je infantilni hepatalni hemangioendoteliom (IHHE), koji dobro reagira na terapiju i...
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SubjectTerms Hemangioendoteliom – dijagnoza, komplikacije, terapija, mortalitet
Hemangioendothelioma – diagnosis, complications, therapy, mortality
Jetreni tumori – dijagnoza, komplikacije, terapija, mortalitet
Liver neoplasms – diagnosis, complications, therapy, mortality
Title DIFUZNI INFANTILNI HEPATALNI HEMANGIOENDOTELIOM: PRIKAZ BOLESNIKA
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Volume 135
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