Spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia: clinical and computed tomographic features in 8 cases.

Objective: The aim of this study was to evaluate the patient demographic and computed tomography (CT) imaging findings of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia.

Study Design: Ten cases of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia diagnosed during 1993-2013 were reviewed. Eight cases with CT images were reviewed.

Results: The mean age of diagnosis of osteosarcoma was 37.8 years (range, 8-55 years). The presence of a soft tissue extension component beyond the area of cortical bone destruction was demonstrated radiographically in 7 out of 8 cases. Mineralization of tumor matrix was seen in 6 cases. Periosteal reaction was demonstrated in only 1 case; 1 out of 6 cases showed significant enhancement on postcontrast CT images. Nine patients had surgical resections of tumor. Seven of them died of tumor, with a mean survival time of 5.3 years.

Conclusions: This study provides CT imaging features with clinical information of spontaneous osteosarcoma in craniomaxillofacial fibrous dysplasia.