Abstract
Objective
A systematic review was undertaken to examine the transformation of craniofacial fibrous
dysplasia (FD) into osteosarcoma (OS).
Study Design
Five databases were searched, and data were evaluated in two subsets:1. Frequency
analysis involving only cross-sectional studies that specifically examined patients
for osteosarcomatous transformation, thereby allowing a calculation of the frequency
of transformation of craniofacial FD, and 2. Case analysis of case reports and case
series of FD that underwent osteosarcomatous transformation. Quality assessment of
the studies in the frequency and case analyses was performed.
Results
Seven cross-sectional studies involving 779 patients in the frequency analysis documented
transformation of craniofacial FD into OS in 13 patients, for a frequency of 1.67%.
Of the 18 separate cases of osteosarcomatous transformation in the case analysis,
11 (61.1%) occurred in females, 11 (61.1%) developed from monostotic FD, and 7 (38.9%)
arose in the mandible, with 5 in the maxilla (27.8%) and 6 (33.3%) in other craniofacial
sites. The mean age of the patients at the time of malignant diagnosis was 36.4 years,
with an average of 11.3 years from FD diagnosis to the appearance of OS. The quality
of the articles ranged from fair to excellent.
Conclusions
The risk of osteosarcomatous transformation of craniofacial FD is low, but the possibility
of transformation should be taken into consideration by clinicians, as this will guide
treatment and follow-up regimens.
Keywords
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Article info
Publication history
Accepted:
December 16,
2022
Received in revised form:
November 10,
2022
Received:
April 28,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2022 Elsevier Inc. All rights reserved.