Management of a Kaposiform haemangioendothelioma of the kidney with Kasabach-Meritt phenomenon without chemotherapy

Kaposiform haemangioendothelioma (KHE) is a benign vascular tumour. While it is not associated with distant spread, it can be locally aggressive and associated with Kasabach-Merritt syndrome (KMS). There were only 2 prior reports of KHE isolated to the kidney, with both having been managed with neoadjuvant chemotherapy. We present a case report of renal KHE in a paediatric patient managed instead with upfront surgical resection, and discuss the close interdisciplinary diagnostic and management approach undertaken to facilitate this, and the considerations in the approach.