MALIGNANT FIBROUS HISTIOCYTOMA ASSOCIATED WITH A BONE INFARCT IN A PATIENT WITH HEREDITARY BONE DYSPLASIA

Hereditary bone dysplasia (HBD) is an extremely rare clinicopathologic al entity manifested by diaphyseal medullary stenosis and cortical bon e 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 autoso mal dominant. In this paper we present the case of a 19-year-old man w ith hereditary bone dysplasia who was unaware of his underlying condit ion until he presented with malignant transformation arising in an are a of bone infarct of the left tibia.