Kaposiform haemangioendothelioma: case report and review of the literature

Kaposiform haemangiondothelioma (KHE) is a rare vascular tumour, predominantly of infancy and early childhood, that has a close association with Kasabach-Merritt syndrome. Despite benign histology, this tumour frequently behaves aggressively, causing significant morbidity and mortality as a result of the compression and invasion of surrounding structures as well as from associated haematological and lymphoproliferative syndromes. There is a need for a high index of suspicion when presented with large, enlarging or abnormal vascular lesions in infancy and, less commonly, in adulthood. An early diagnosis of KHE can lead to prompt treatment, which may be life saving. To date, there have been only four reported cases of KHE occurring in patients over the age of 18 years. We report an otherwise well 26-year-old woman who presented with a KHE of the left thigh, and briefly review the literature. We believe this to be the second reported case of KHE in the UK and the first in an adult patient.