Comparison of characteristic features and local recurrence in syndromic versus non-syndromic multifocal non-ossifying fibroma.

BACKGROUND

Multiple non-ossifying fibromas (MNOF) could be presented with other extraskeletal anomalies (syndromic) or not (non-syndromic). In this study, we aimed to compare characteristic features and local recurrence between symptomatic syndromic and non-syndromic MNOFs.

METHODS

Thirty-five patients with symptomatic MNOF were included in this study, comprised of 30 patients without the café-au-lait spot (non-syndromic) and five with café-au-lait spots plus other signs of neurofibromatosis type 1 (syndromic). Characteristic features of the patients and lesions were compared between syndromic and non-syndromic MNOFs. The lesions were treated with curettage and bone graft. The rate of local recurrences was also compared between the two groups of syndromic and non-syndromic MNOF.

RESULTS

Study population included 19 (54.3%) males and 16 (45.7%) females with the mean age of 7.63 ± 3.1 years (range 4-11). The mean follow-up of the patients was 65.6 ± 38.2 months (range 24-96). The lesion was bilateral in 13.3% of non-syndromic MNOFs and 80% of syndromic MNOFs. This difference was statistically significant (p = 0.01). After curettage and bone grafting, the lesion recurred in 6.7%of non-syndromic MNOFs and 60% of the syndromic MNOF. This difference was statistically significant, as well (p = 0.01). No other significant difference was found between syndromic and non-syndromic MNOFs.

CONCLUSIONS

The syndromic form of MNOF is much less prevalent and is associated with a higher rate of recurrence after surgical removal. Therefore, a more rigorous removal of the MNOF lesions might be necessary when presented in a syndromic context.