Malignant fibrous histiocytoma associated with a bone infarct in a patient with hereditary bone dysplasia

Hereditary bone dysplasia (HBD) is an extremely rare clinicopathological entity manifested by diaphyseal medullary stenosis and cortical bone thickening associated with a propensity for fractures affecting the long tubular bone. Malignant transformation has been reported to occur at an alarming frequency. The hereditary pattern appears to be autosomal dominant. In this paper we present the case of a 19-year-old man with hereditary bone dysplasia who was unaware of his underlying condition until he presented with malignant transformation arising in an area of bone infarct of the left tibia.