Multiple fibrous cortical defects/non-ossifying fibroma

Discussion:

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 tumors 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.

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