Advanced epithelioid haemangioendothelioma in a child presenting as deep vein thrombosis

Venous thromboembolism (VTE) is a rare event in children and does not usually trigger investigation for malignancy. We report the case of a previously healthy female teenager presenting with unilateral leg swelling. Colour-Doppler ultrasound confirmed deep vein thrombosis (DVT), and the thrombophilia workup was negative. Cross-sectional imaging identified multiple liver and lung lesions, diagnosed as epithelioid haemangioendothelioma (EHE) at biopsy. Lifelong anticoagulation was commenced; the patient was initially observed and then started on sirolimus at disease progression. We describe the first published case of EHE presenting with DVT in a child. Clinicians need to be mindful of the association between cancer and thrombosis, even in paediatric patients.