COPYRIGHT © 2005 BY THE JOURNAL OF BONE AND JOINT SURGERY, INCORPORATED Malignant Fibrous Histiocytoma of Bone Associated with

on Recklinghausen disease, or neurofibromatosis, is a congenital and familial disorder with an autosomal dominant pattern of inheritance. The disease prima-rily affects ectodermal tissues arising from the neural crest. It is divided into two basic forms: peripheral (type 1) and central (type 2), characterized by distinct skeletal and soft-tissue manifestations1-6. Type-1 neurofibromatosis usually manifests in early childhood and is more frequent than the type-2 form of the disease7,8. Skeletal abnormalities and dysplasias, including erosive or pressure bone defects, pseudarthroses, and cranial defects, are diagnosed in approximately 80 % of patients with type-1 neurofibromatosis. In addition, multiple nonossify-ing fibromas that predominantly affect the metaphyseal parts of the long tubular bones tend to develop in patient