Fibrous Dysplasia, Adamantinoma, and Osteofibrous Dysplasia
摘要
All fibrous osseous tumours are rare and have similar radiological appearance with lytic expansile appearance and endosteal scalloping. Also, clinical features such as swelling, pain, and potential pathological fracture are all common features. Fibrous dysplasia is an often asymptomatic skeletal disorder that may present in different forms, ranging from isolated monostotic lesions to complex syndromes, such as McCune-Albright syndrome. While the disorder itself is benign, its complications, including bone deformities, fractures, and endocrine abnormalities, can significantly affect quality of life. Adamantinoma is a low-grade malignant bone tumour that primarily affects the long bones, particularly the tibia. It is typically present between the second and fifth decades with a slight male predilection. Almost all cases occur in long bones, and up to 85% occur in the mid-tibial shaft. There are two types: classic adamantinoma and differentiated adamantinoma (osteofibrous dysplasia-like adamantinoma). Osteofibrous dysplasia (OFD) is a benign fibro-osseous lesion that primarily affects the long bones, particularly the tibia and fibula. It is characterized by the replacement of normal bone with fibrous tissue and immature woven bone. OFD is most commonly diagnosed in children and adolescents, with a slight predilection for males. OFD is often asymptomatic but can present with pain, swelling, or pathological fractures. Radiological imaging plays a crucial role in the diagnosis of osteofibrous dysplasia. The prognosis is generally favourable, particularly for the classic form. However, there is a risk of recurrence, especially if the lesion is not completely excised.