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Malignant Fibrous Histiocytoma of Bone Associated with Type-1 NeurofibromatosisA Case Report
Panayiotis J. Papagelopoulos, MD, DSc1; Andreas F. Mavrogenis, MD2; Evanthia C. Galanis, MD, DSc3; George D. Chloros, MD4; Kleo Th. Papaparaskeva, MD5
1 Department of Orthopaedic Surgery, Athens University Medical School, 4 Christovassili Street, Neo Psychikon, 15451 Athens, Greece. E-mail address: pjp@hol.gr
2 33 25-Martiou Street, Holargos 155-62, Athens, Greece. E-mail address: andreasfmavrogenis@yahoo.gr
3 Division of Medical Oncology, Mayo Clinic, 200 First Street S.W., Rochester, MN 55905. E-mail address: galanis.evanthia@mayo.edu
4 52 Papaflessa Street, 14671 Nea Erythrea, Athens, Greece. E-mail address: gchlorosdoc@uk2.net
5 Division of Anatomic Pathology, Hygeia Athens Medical Center, 4 Erithrou Stavrou Street, 15123 Marousi, Athens, Greece. E-mail address: kleopap@yahoo.com
View Disclosures and Other Information
The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.
Investigation performed at the Department of Orthopaedic Surgery, Athens University Medical School, Athens, Greece

The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 2005 Feb 01;87(2):399-403. doi: 10.2106/JBJS.D.02117
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Extract

Von Recklinghausen disease, or neurofibromatosis, is a congenital and familial disorder with an autosomal dominant pattern of inheritance. The disease primarily 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 nonossifying fibromas that predominantly affect the metaphyseal parts of the long tubular bones tend to develop in patients with this type of the disease1.
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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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