Djuricic, Slavisa M. (6603108728)Slavisa M. (6603108728)DjuricicSarajlija, Adrijan (26027638400)Adrijan (26027638400)SarajlijaDjokic, Dragomir (56017672300)Dragomir (56017672300)DjokicSimic, Radoje (16744648200)Radoje (16744648200)Simic2025-06-122025-06-122020https://doi.org/10.5937/scriptamed51-25350https://www.scopus.com/inward/record.uri?eid=2-s2.0-85149462144&doi=10.5937%2fscriptamed51-25350&partnerID=40&md5=fcaf04e642def65aeab386c1f058dc58https://remedy.med.bg.ac.rs/handle/123456789/5071Kaposiform haemangioendothelioma (KHE) is a rare, locally invasive vascular tumour that is commonly associated with the Kasabach-Merritt phenomenon (KMP). A case of a five-month-old female infant admitted for dyspnoea, stridor, and skin haematoma is presented. Computerised tomography of the chest showed a tumour mass occupying mediastinum and most of the left hemithorax, while laboratory analysis revealed a thrombocytopaenia and a consumption coagulop-athy. Histology of tumour biopsy was characteristic of KHE with a component of tufted angioma. Corticosteroid treatment initially induced a reduction in tumour size, but progression occurred four weeks later and led to a fatal outcome despite additional chemotherapy. After a literature search, we found only 18 cases of me-diastinal KHE published so far, with 21 % fatality rate. In the present case several risk factors for adverse outcome were present: onset of disease in early infancy, a large volume of the tumour, mediastinal location, KMP, and partial response to available therapy. © 2020 Djuricic et al.infantkaposiform haemangioendotheliomaKasabach-Merritt phenome-nonmediastinum