Multiple fibrous cortical defects/non-ossifying fibroma
30 years old male patient presented with multiple Cafe au Lait patches & axillary freckling.
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Multiple bilateral tibial & femoral sharply demarcated, asymmetrical, cortically based lesions with thin margins.They are located mainly in the metaphysis, adjacent to the physis.
Multiple bilateral intramuscular enhancing nodules are seen (neurofibromas).
Multiple NOFs are associated with NF1, fibrous dysplasia, and Jaffe-Campanacci syndrome.
The association of multiple non-ossifying fibromas with cafe au lait skin patches has been named Jaffee-Campanacci syndrome (JCS).
It remains intriguing whether the Jaffe-Campanacci syndrome is a particular form of NF1 or a separate entity. In the 2013 “WHO Classification of tumours of soft tissue and bone”, it is defined as the association of NOFs and NF1.
Other authors suggested the syndrome might be a manifestation of the variability of NF1.
Criteria for Jaffe-Campanacci syndrome, as proposed by Mirra et al., including axillary freckles, café-au-lait spots and multiple NOFs.
- 1. Fletcher, Christopher DM, et al. "WHO Classification of Tumours of Soft Tissue and Bone. 2013." Lyon: IARC: 305-310.
- 2. Mirra JM, Gold RH, Rand F. Disseminated nonossifying fibromas in association with café-au-lait spots (Jaffe-Campanacci syndrome). Clin. Orthop. Relat. Res. 1982; (168): 192-205. Pubmed citation