Jaterní infantilní hemangioendoteliom je vzácný benigní vaskulární tumor pocházející z mezenchymální tkáně jater. Přestože jde o nádor benigní, může způsobit až život ohrožující komplikace. V této kazuistice popisujeme případ 5týdenního kojence s tumorózním ložiskem v pravém jaterním laloku. Zobrazovací metody ukazovaly na diagnózu infantilního hemangioendoteliomu, ale přítomnost hepatoblastomu nebylo možné zcela vyloučit. Vzhledem k riziku život ohrožujících komplikací byl nádor chirurgicky odstraněn. Histologický rozbor potvrdil diagnózu infantilního hemangioendoteliomu.
Infantile hepatic hemangioendothelioma (IHH) is a rare, benign vascular tumor that originates from mesenchymal tissue in the liver. Although IHH is benign, it may develop life-threatening complications. We describe a 5-week-old female with a tumorous mass in the right hepatic lobe. Imaging methods pointed to an IHH diagnosis, but we could not rule out hepatoblastoma. Due to the risk of life-threatening complications, the tumor was surgically removed. Histological analysis confirmed the diagnosis of IHH.
- Klíčová slova
- infantilní hemangioendoteliom,
- MeSH
- hemangioendoteliom * chirurgie diagnóza MeSH
- lidé MeSH
- nádory jater * chirurgie diagnóza MeSH
- novorozenec MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
12letý chlapec s mnohočetnými komorbiditami byl přijat pro výraznou slabost, apatii, bledost. V krevním obraze dominovala pancytopenie - makrocytární anémie s těžkým poklesem hemoglobinu, leukopenie a trombocytopenie se zvýšenými markery hemolýzy. Kostní dřeň byla hypocelulární s dyserytropoetickými a myelodysplastickými rysy s absencí megakaryocytů. Současně byly přítomné velmi nízké sérové hladiny vitaminu B12 a kyseliny listové, spolu s nízkými hladinami dalších vitaminů a nutričních parametrů. Léčba parenterálně podávaným vitaminem B12 vedla rychle k postupnému obnovení funkce kostní dřeně a spolu s léčbou přidružené malabsorpce a karence dalších vitaminů k úpravě celkového stavu.
12-years-old boy with multiple comorbidities was admitted to the hospital for major weakness, lethargy and palor. There was pancytopenia in the blood count - macrocytic anemia with deep decrease of hemoglobin, leucocytopenia and trombocytopenia and higher level of hemolysis parameters. Bone marrow was hypocellular with marks of dyserythropoesis and myelopoesis and with no megakaryocytes. There were also low levels of vitamin B12 and folate and also low levels of other vitamins and nutritional parameters in the serum. After parenteral vitamin B12, other vitamins and makronutrients delivery quick improvement of clinical status and bone marrow function was reached.
- MeSH
- apatie MeSH
- dítě MeSH
- lidé MeSH
- makrocytární anemie etiologie MeSH
- nedostatek vitaminu B12 diagnóza komplikace terapie MeSH
- pancytopenie * diagnóza etiologie terapie MeSH
- podvýživa diagnóza etiologie MeSH
- vitamin B 12 aplikace a dávkování terapeutické užití MeSH
- výsledek terapie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
BACKGROUND: We designed the EURAMOS-1 trial to investigate whether intensified postoperative chemotherapy for patients whose tumour showed a poor response to preoperative chemotherapy (≥10% viable tumour) improved event-free survival in patients with high-grade osteosarcoma. METHODS: EURAMOS-1 was an open-label, international, phase 3 randomised, controlled trial. Consenting patients with newly diagnosed, resectable, high-grade osteosarcoma aged 40 years or younger were eligible for randomisation. Patients were randomly assigned (1:1) to receive either postoperative cisplatin, doxorubicin, and methotrexate (MAP) or MAP plus ifosfamide and etoposide (MAPIE) using concealed permuted blocks with three stratification factors: trial group; location of tumour (proximal femur or proximal humerus vs other limb vs axial skeleton); and presence of metastases (no vs yes or possible). The MAP regimen consisted of cisplatin 120 mg/m(2), doxorubicin 37·5 mg/m(2) per day on days 1 and 2 (on weeks 1 and 6) followed 3 weeks later by high-dose methotrexate 12 g/m(2) over 4 h. The MAPIE regimen consisted of MAP as a base regimen, with the addition of high-dose ifosfamide (14 g/m(2)) at 2·8 g/m(2) per day with equidose mesna uroprotection, followed by etoposide 100 mg/m(2) per day over 1 h on days 1-5. The primary outcome measure was event-free survival measured in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT00134030. FINDINGS: Between April 14, 2005, and June 30, 2011, 2260 patients were registered from 325 sites in 17 countries. 618 patients with poor response were randomly assigned; 310 to receive MAP and 308 to receive MAPIE. Median follow-up was 62·1 months (IQR 46·6-76·6); 62·3 months (IQR 46·9-77·1) for the MAP group and 61·1 months (IQR 46·5-75·3) for the MAPIE group. 307 event-free survival events were reported (153 in the MAP group vs 154 in the MAPIE group). 193 deaths were reported (101 in the MAP group vs 92 in the MAPIE group). Event-free survival did not differ between treatment groups (hazard ratio [HR] 0·98 [95% CI 0·78-1·23]); hazards were non-proportional (p=0·0003). The most common grade 3-4 adverse events were neutropenia (268 [89%] patients in MAP vs 268 [90%] in MAPIE), thrombocytopenia (231 [78% in MAP vs 248 [83%] in MAPIE), and febrile neutropenia without documented infection (149 [50%] in MAP vs 217 [73%] in MAPIE). MAPIE was associated with more frequent grade 4 non-haematological toxicity than MAP (35 [12%] of 301 in the MAP group vs 71 [24%] of 298 in the MAPIE group). Two patients died during postoperative therapy, one from infection (although their absolute neutrophil count was normal), which was definitely related to their MAP treatment (specifically doxorubicin and cisplatin), and one from left ventricular systolic dysfunction, which was probably related to MAPIE treatment (specifically doxorubicin). One suspected unexpected serious adverse reaction was reported in the MAP group: bone marrow infarction due to methotrexate. INTERPRETATION: EURAMOS-1 results do not support the addition of ifosfamide and etoposide to postoperative chemotherapy in patients with poorly responding osteosarcoma because its administration was associated with increased toxicity without improving event-free survival. The results define standard of care for this population. New strategies are required to improve outcomes in this setting. FUNDING: UK Medical Research Council, National Cancer Institute, European Science Foundation, St Anna Kinderkrebsforschung, Fonds National de la Recherche Scientifique, Fonds voor Wetenschappelijk Onderzoek-Vlaanderen, Parents Organization, Danish Medical Research Council, Academy of Finland, Deutsche Forschungsgemeinschaft, Deutsche Krebshilfe, Federal Ministry of Education and Research, Semmelweis Foundation, ZonMw (Council for Medical Research), Research Council of Norway, Scandinavian Sarcoma Group, Swiss Paediatric Oncology Group, Cancer Research UK, National Institute for Health Research, University College London Hospitals, and Biomedical Research Centre.
- MeSH
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- kombinovaná terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- nádory kostí farmakoterapie mortalita MeSH
- osteosarkom farmakoterapie mortalita MeSH
- předškolní dítě MeSH
- protokoly protinádorové kombinované chemoterapie terapeutické užití MeSH
- senioři MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- klinické zkoušky, fáze III MeSH
- multicentrická studie MeSH
- randomizované kontrolované studie MeSH
- srovnávací studie MeSH
- MeSH
- adjuvantní chemoterapie metody MeSH
- dítě * MeSH
- kolorektální nádory * diagnóza farmakoterapie chirurgie MeSH
- lidé MeSH
- mladiství * MeSH
- Check Tag
- dítě * MeSH
- lidé MeSH
- mladiství * MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
