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.