A 13 years old child presents with a 3-year history of gradual swelling of the maxilla with medial displacement of turbinates with mild proptosis. What is the most likely diagnosis:
Correct Answer: Fibrous dysplasia
Description: Ans. D Fibrous dysplasia Ref- Image - Clinical Features Patients with small, monostotic lesions may be asymptomatic, with the osseous abnormality identified incidentally on radiologic studies obtained for unrelated reasons. However, bone pain, swelling, and tenderness are common presentations in symptomatic patients. Endocrine disturbances may be the initial presentation in some patients. This section will briefly review the common affected locations of fibrous dysplasia, fibrous dysplasia deformity and fracture, and malignant transformation for this lesion. Affected locations The most common sites of skeletal involvement in monostotic fibrous dysplasia are the ribs, proximal femur, and craniofacial bones, typically the posterior maxilla. The lesion may involve only a small segment of bone or it may occupy its entire length. In polyostotic fibrous dysplasia, the spectrum of involvement varies from 2 bones to more than 75% of the skeleton. Polyostotic fibrous dysplasia is most commonly found in the femur, tibia, pelvis, and foot. Other sites less commonly affected include the ribs, skull, and bones of the upper extremity. Uncommonly affected bones include the lumbar spine, clavicle, and the cervical spine. Deformity and fracture The most common physical deformities are leg-length discrepancy, facial asymmetry due to hemicranial involvement, and rib deformities. Fracture is the most common complication in fibrous dysplasia. It is seen in more than half of the patients with the polyostotic form of the disease. Deformities in weight-bearing bones can occur. Almost 75% of patients with polyostotic fibrous dysplasia are symptomatic, with pain, deformity, or pathologic fractures. Malignant transformation Malignant transformation of fibrous dysplasia occurs very infrequently, with reported prevalences ranging from 0.4% to 4%. Previous irradiation has been documented in more than half of the cases with malignant transformation. The most common malignant tumors are osteosarcoma, fibrosarcoma, and chondrosarcoma, and the majority of patients are older than thirty years when the sarcoma is diagnosed. The craniofacial region is the most common site of involvement, followed by the femur, tibia, and pelvis. The rate of malignant transformation is higher for polyostotic lesions than for monostotic lesions.
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