Congenital ossifying fibroma (osteofibrols dysplasia) of the tibia - A case report

Abstract

Ossifying fibromas of the large bones of the leg are benign lesions occurring in the paediatric age group, identical in histological appearance to the similarly named tumour of the jaw in adults They present most frequently after minor trauma with symptoms of swelling of the tibia or fibula which may or may not be painful. Pathological fracture or limp are occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate in whom at birth the right tibia was noted to be twisted, of normal length, with a firm mass anteriorly. Plain x-rays showed a mixed lytic and sclerotic lesion in the proximal metaphysis. Biopsy showed collagenous stroma containing plump spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. A diagnosis of ossifying fibroma (osteofibrous dysplasia) was made and so no further surgical treatment was undertaken. The differential diagnosis includes fibrous dysplasia, ossifying fibroma, adamantinoma and chronic osteomyelitis. Lesions such as pseudarthrosis associated with neurofibromatosis, aneurysmal bone cyst, fibrous histiocytoma, fibromatosis, eosinophilic granuloma, myofibromatosis, and malignant fibrous histiocytoma, have all been described arising in a congenital form and need to be excluded. A conservative therapeutic approach is generally recommended. Close clinical and radiological follow-up in the reported case over six months has shown evidence of regression of the lesion. If surgery is indicated, for example where the risk of pathological fracture is high, wide local excision is the favoured surgical treatment, as local recurrence frequently occurs after curettage. There is no place for radiotherapy or chemotherapy.