Odontogenic myxofibroma synchronous with primary angiosarcoma of the spleen

Odontogenic myxofibroma of the temporomandibular joint (TMJ) is a rare tumour, moreover, primary splenic angiosarcoma (PAS) in paediatric patients is extremely rare We report on a 1 5-year-old boy who presented with right TMJ swelling and subsequently developed epigastric and right upper quadrant pain The TMJ swelling proved to be odontogenic myxofibroma and the abdominal pain was a result of primary splenic angiosarcoma with hepatic metastasis We report for the first time the synchronous presentation of PAS and odontogenic myxofibroma in a paediatric patient, and we describe the radiological features along with the histological diagnosis and clinical outcome Uptake in F-18-2-fluoro-2-deoxy-D-glucose positron emission tomography is also described for the first time for both these tumours