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Case Reports   |    
Jaffe-Campanacci Syndrome A Case Report and Review of the Literature
Mohammad Anwar Hau, MBBS, MMed (Ortho); Edward J. Fox, MD; Justin M. Cates, MD, PhD; Brian E. Brigman, MD; Henry J. Mankin, MD
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Investigation performed at the Orthopaedic Oncology Service and the Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts

Mohammad Anwar Hau, MBBS, MMed (Ortho)
Orthopaedic Department, Hospital Kota Bharu, 15586 Kota Bharu, Kelantan, Malaysia

Edward J. Fox, MD
Department of Orthopaedics, University of Rochester, 601 Elmwood Avenue, Rochester, NY 14642

Justin M. Cates, MD, PhD
75 Templeton Place, White River Junction, VT 05001

Brian E. Brigman, MD
Orthopedic Cell Biology Laboratory, Duke University, 389 Medical Sciences Research Building, Durham, NC 27710

Henry J. Mankin, MD
Orthopaedic Oncology Service, Gray 604, Massachusetts General Hospital, Boston, Massachusetts 02114. E-mail address for H.J. Mankin: hmankin@partners.org

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.

J Bone Joint Surg Am, 2002 Apr 01;84(4):634-638
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Extract

In 1942, Jaffe and Lichtenstein 1 first described nonosteogenic fibroma (now known as nonossifying fibroma) of bone as a frequently encountered benign fibrous lesion of the long bones. In 1958, Jaffe 2 subsequently described a rarely seen clinical entity in which multiple nonossifying fibromas occurred in association with caf�-au-lait spots and axillary freckling, but without accompanying neurofibromas. Jaffe suggested that the disorder was an unusual form of neurofibromatosis. In a subsequent report of the same findings-that is, multiple nonossifying fibromas and caf�-au-lait spots-Holt 3 suggested that they were incidental and unrelated. Schwartz and Ramos 4 attributed the two sets of findings to a syndrome called diffuse mesodermal dysplasia, but the name was never adopted. Several isolated cases of this unusual clinical presentation were reported by Mandell et al. 5 and Pitcock 6 . In 1983, Campanacci et al. 7 reported on ten patients with nonossifying fibromas associated with some features of neurofibromatosis and expressed the opinion that these cases represented a new syndrome. Mirra et al. 8 reported on a patient with similar symptoms in 1982 and, on the basis of their knowledge of the large series reported on later by Campanacci et al., designated the syndrome as Jaffe-Campanacci syndrome. Since then, the syndrome has been widely accepted as a unique clinical entity, but very few cases have been reported in the English-language literature 7,9-13 .
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