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Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia--a case report
N M Smith1, R W Byard, B Foster
1Department of Histopathology, Adelaide Children's Hospital, South Australia.
Insights
Congenital ossifying fibroma (osteofibrous dysplasia) is a rare benign leg tumor in newborns. This case highlights its presentation and non-surgical management, showing potential for spontaneous healing.
Area of Science:
- Orthopedic Surgery
- Pediatric Oncology
- Pathology
Background:
- Ossifying fibromas (OFs) are benign bone tumors typically affecting the jaw in adults.
- OFs of the long bones are rare, particularly in the pediatric population, and histologically resemble jaw OFs.
- Congenital presentation of long bone OFs is exceptionally rare.
Observation:
- A neonate presented with a congenital bowed right lower leg, limb length discrepancy, and an anterior tibial mass.
- Radiographic imaging revealed a mixed lytic and sclerotic lesion in the proximal tibia.
- Histopathological examination confirmed features consistent with ossifying fibroma (osteofibrous dysplasia).
Findings:
- The congenital ossifying fibroma in the neonate showed no tumor growth.
- Radiological evidence indicated healing of the tibial lesion at one year follow-up.
- Conservative management was successful without surgical intervention.
Implications:
- This case underscores the importance of considering ossifying fibroma in the differential diagnosis of congenital tibial lesions.
- It demonstrates the potential for non-operative management and favorable outcomes in congenital long bone ossifying fibromas.
- Highlights the clinicopathological features of this unusual pediatric bone tumor.
Abstract:
Ossifying fibromas of the long bones of the leg are benign lesions occurring in the pediatric age group identical in histological appearance to the similarly named tumor of the jaw in adults. Most frequently presentation occurs after minor trauma with symptoms of a swelling of the tibia or fibula which may be painful. Pathological fracture or limp are also occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate who presented at birth with a bowed right lower leg. The limb was 1 cm shorter than the other side, with tibia vara and a firm mass situated anteriorly. X-ray showed a mixed lytic and sclerotic lesion in the proximal metaphysis of the tibia. Biopsy showed collagenous stroma containing spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. As the appearances were typical of an ossifying fibroma (osteofibrous dysplasia) no surgical treatment was given. The patient was well with no growth of the tumor and with radiological evidence of healing at 1 year follow up. This case is presented to draw attention to the clinicopathological features of this unusual lesion which must be considered in the differential diagnosis of congenital lesions of the tibia.