MULTICENTRIC FIBROMATOSIS WITH METAPHYSEAL DYSPLASIA

A new association between multicentric fibromatosis and a bone dysplas ia is reported. Three patients are described, each of whom had multice ntric fibromatosis of the lower extremity and a skeletal dysplasia. On e of the patients had dysplastic changes restricted to the involved ex tremity. The other two had dysplastic changes of all the long bones. T he dysplasia consisted predominantly of undertubulation reminiscent of the findings seen in Pyle disease. No clinical or radiographic abnorm alities of the marrow were found in any of these patients. It is belie ved that this association represents mesenchyme that is predisposed to develop both dysplastic bone changes and tumor.