Very rare mediastinal location of Kaposiform haemangioendothelioma: A case report and a brief review of the previously published cases

Abstract

Kaposiform 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 coagulopathy. 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 mediastinal 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.

Keywords

• kaposiform haemangioendothelioma
• kasabach-merritt phenomenon
• mediastinum
• infant