Difuzni infantilni hepatalni hemangioendoteliom: prikaz bolesnika

Scientific paper - Case study

• Title (english): Diffuse infantile hepatic hemangioendothelioma: a case report
• Author: Ivica Sjekavica
• Author: Marko Petrovečki
• Author: Vice Šunjara
• Author: Maja Pavlović
• Author: Ranka Štern Padovan
• Author's institution: University of Zagreb; School of Medicine
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences

Abstract (english)

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.

Abstract (croatian)

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.

• Keywords (english): Fatal Outcome; Heart Arrest / etiology; Hemangioendothelioma / complications; Hemangioendothelioma / pathology; Hemangioendothelioma / therapy; Humans; Infant; Liver / pathology; Liver Neoplasms / complications; Liver Neoplasms / pathology; Liver Neoplasms / therapy; Male; Tumor Burden
• Language: croatian
• Publication type: Scientific paper - Case study
• Publication status: Published
• Peer review: Peer review
• Publication version: Published version
• Journal title: Liječnički vjesnik
• Numbering: vol. 135, no. 9-10, pp. 242-245
• p-ISSN: 0024-3477
• URN:NBN: urn:nbn:hr:105:232472
• Publication: 2013-09
• Document URL: http://lijecnicki-vjesnik.hlz.hr/lijecnicki-vjesnik/difuzni-infantilni-hepatalni-hemangioendoteliom-prikaz-bolesnika/
• Type of resource: Text
• Access conditions: Open access
• Created on: 2024-03-27 20:07:20