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FIBROUS DYSPLASIA

FIBROUS DYSPLASIA. DEFINITION. Uncommon, benign disorder characterized by a tumor-like proliferation of fibro-osseous tissue. AETIOLOGY. Cause unknown. Tissue in the tumour is immature, woven bone that cannot differentiate in to mature, lamellar bone. INCIDENCE. Relatively common

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FIBROUS DYSPLASIA

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  1. FIBROUS DYSPLASIA Fibrous Dysplasia

  2. DEFINITION Uncommon, benign disorder characterized by a tumor-like proliferation of fibro-osseous tissue Fibrous Dysplasia

  3. AETIOLOGY • Cause unknown Tissue in the tumour is immature, woven bone that cannot differentiate in to mature, lamellar bone Fibrous Dysplasia

  4. INCIDENCE • Relatively common • Usually monostotic • Mainly children & adolescents • Median age of onset = 8 yrs • MALE > FEMALE Fibrous Dysplasia

  5. CLINICALLY( McCune-Albrights ) • Polyostotic disease (Usually unilateral) • Skin pigmentation (Café au lait) • Precocious puberty (endocrinopathy) • Presents earlier • Malignant transformation 4% (chondrosarcoma or osteosarcoma) Fibrous Dysplasia

  6. SITES • Ribs commonest 40% • Lower limbs > upper limbs • Craniofacial --> skull deformity • Epiphyses usually spared • Polyostotic -> pain , fracture , deformity Fibrous Dysplasia

  7. RADIOLOGY • Lucent lesion in medullary space • Sclerotic margin • Ground glass appearance typical • No periosteal reaction • Shepherds crook deformity • Expansion of cortex Fibrous Dysplasia

  8. RADIOLOGY Fibrous Dysplasia

  9. PATHOLOGY • Bone displaced by firm , gritty whitish tissue • Vascular tumour , poorly orientated bone trabeculae • Bone is woven rather than lamellar • Lack of osteoblastic rimming Fibrous Dysplasia

  10. DIFFERENTIAL DIAGNOSIS • Pagets disease • FCD • Hyper parathyroidism • Osteoblastoma • Osteosarcoma Fibrous Dysplasia

  11. TREATMENT • Monostotic -> curettage and grafting if symptomatic • Polyostotic -> symptomatic treatment • May require osteotomy for deformity or lengthening Fibrous Dysplasia

  12. PROGNOSIS • Monostotic lesions cease activity at puberty • May be activated by pregnancy • Polyostotic 85% -> pathological # • Malignant change occurs after radiotherapy Fibrous Dysplasia

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