Aggressive bone tumorous lesion in infancy: osteofibrous dysplasia of the tibia and fibula

The clinical experiences of 10 patients with osteofibrous dysplasia were reviewed. Mean age of the patients was 7.3 years; all had an intracortical, eccentric lesion in the anterior aspect of the shaft of the tibia. Seven of the patients underwent either an excision or an en bloc resection; the lesion recurred in four of the seven, and one showed pseudarthrosis. Surgical outcomes were unsatisfactory, especially in the younger patients. In contrast, the remaining three patients who did not undergo surgery showed satisfactory results. Nonsurgical treatment is generally recommended except in cases in which impending deformities seem certain. Osteofibrous dysplasia was characterized histologically by osteoid rimmed by osteoblasts, sinusoid surrounding osteoid, many osteoclasts in the sinusoid, and fibrous stroma. Ultrastructurally, stroma cells revealed the characteristics of preosteoblasts. Osteogenic potentiality was well-documented. This tumorous condition is likely attributed to dysremodelling, in which osteoclastosis is dominant to osteogenesis. Abnormal blood circulation in the periosteum is the likely pathogenesis of osteofibrous dysplasia.