The Journal of Bone and Joint Surgery (American). 2005;87:399-403.
doi:10.2106/JBJS.D.02117
© 2005 The Journal of Bone and Joint Surgery, Inc.
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow CME: Take the activity for this article:
 Oncology Test 3: Spring 2006 (publication date May 15, 2006; expiration dat...
Right arrow Letters to the Editor: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Letters to the Editor are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow Rights and Permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Papagelopoulos, P. J.
Right arrow Articles by Papaparaskeva, K. Th.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Papagelopoulos, P. J.
Right arrow Articles by Papaparaskeva, K. Th.
Related Collections
Right arrow Case Report
Right arrow Oncology
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Facebook   Add to Technorati   Add to Twitter  
What's this?

Malignant Fibrous Histiocytoma of Bone Associated with Type-1 Neurofibromatosis

A Case Report

Panayiotis J. Papagelopoulos, MD, DSc1, Andreas F. Mavrogenis, MD2, Evanthia C. Galanis, MD, DSc3, George D. Chloros, MD4 and 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

Investigation performed at the Department of Orthopaedic Surgery, Athens University Medical School, Athens, Greece

The first 150 words of the full text of this article appear below.


   Introduction
 
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.

The most serious, and often fatal, complication of the disease is a malignant peripheral nerve sheath tumor. Most cases are located within the soft tissue of the . . . [Full Text of this Article]


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Facebook Facebook   Add to Technorati Technorati   Add to Twitter Twitter    What's this?