35. Fibrous Dysplasia
Definition
Fibrous dysplasia is a dysplastic bone disorder wherein immature woven bone forms directly from abnormal fibrous connective tissue. Fibro-osseous tissue within affected bones expands as a lesion of growing bone. The term “dysplasia” refers to the person’s inability to form mature lamellar bone.
Incidence
The true incidence is not known, but there are estimates that about 10% of benign bone tumors are the result of fibrous dysplasia.
Etiology
Fundamentally fibrous dysplasia appears to be a disorder of postnatal cancellous bone maintenance or an abnormality of bone-forming mesenchyme.
Signs and Symptoms
• Abnormal cutaneous pigmentation
• Albright syndrome
• Cushing’s disease
• Hyperparathyroidism
• Hyperthyroidism
• Hypophosphatemic rickets