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

Chir Organi Mov. 2009 May;93(1):1-7. doi: 10.1007/s12306-009-0016-4. Epub 2009 Apr 29.


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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Axilla
  • Bone Neoplasms / diagnosis*
  • Bone Neoplasms / pathology
  • Cafe-au-Lait Spots / etiology
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Fibrous Dysplasia, Polyostotic / diagnosis*
  • Fibrous Dysplasia, Polyostotic / pathology
  • Fractures, Spontaneous / etiology
  • Histiocytoma, Benign Fibrous / diagnosis*
  • Histiocytoma, Benign Fibrous / pathology
  • Humans
  • Infant
  • Intellectual Disability / etiology
  • Magnetic Resonance Imaging
  • Male
  • Melanosis / etiology
  • Middle Aged
  • Neurofibromatosis 1 / diagnosis
  • Phenotype
  • Registries
  • Syndrome
  • Young Adult