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Non-ossifying fibroma, fibrous cortical defect and Jaffe–Campanacci syndrome: a biologic and clinical review

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Abstract

Non-ossifying fibroma of bone (NOF) is a common entity, more frequently found in male children and consisting of a solitary eccentric, lytic expanded lesion in the metaphysis of a long bone. The disorder is benign and most often asymptomatic but may result in a fracture requiring therapy. Of some importance is to distinguish NOF from another very similar but smaller lesion, fibrous cortical defect, which is almost always asymptomatic and eccentrically located. Even more striking is a very rarely encountered lesion known as Jaffe–Campanacci syndrome, which also occurs in children who present with typical non-ossifying fibromatous tumors but in multiple sites. In addition, these patients have some systemic and dermal findings resembling those seen in patients with Type 1 neurofibromatosis.

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Acknowledgments

The study was performed using information from our MGH Orthopaedic Oncology computer system, a program, which has been approved by our Institutional Review Board. We did not violate patient confidentiality. Several of the images are from the Jaffe Orthopaedic Pathology Collection…and once again, we did not in any way violate patient confidentiality.

Conflict of interest statement

The authors declare that they have no conflict of interest related to the publication of this manuscript.

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Authors and Affiliations

  1. Orthopaedic Research Laboratories, Massachusetts General Hospital, 1122A Jackson Building, Boston, MA, 02114, USA

    Henry J. Mankin, Carol A. Trahan, Gertrud Fondren & Carole J. Mankin

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  1. Henry J. Mankin
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Correspondence to Henry J. Mankin.

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Mankin, H.J., Trahan, C.A., Fondren, G. et al. Non-ossifying fibroma, fibrous cortical defect and Jaffe–Campanacci syndrome: a biologic and clinical review. Musculoskelet Surg 93, 1–7 (2009). https://doi.org/10.1007/s12306-009-0016-4

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  • DOI: https://doi.org/10.1007/s12306-009-0016-4

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