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Adamantinoma

Adamantinoma. Ted Scriven Sept 15 th , 2008. Definition. Adamantinoma is a malignant bone tumour. Epidemiology. Rare < 1% of primary bone tumours Patents mostly 2 nd and 3 rd decade of life, but wide range overall Predilection for tibia - ~ 80% of cases Usually occurs in diaphysis

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Adamantinoma

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  1. Adamantinoma Ted Scriven Sept 15th, 2008

  2. Definition Adamantinoma is a malignant bone tumour

  3. Epidemiology Rare < 1% of primary bone tumours Patents mostly 2nd and 3rd decade of life, but wide range overall Predilection for tibia - ~ 80% of cases Usually occurs in diaphysis Slight male predominance  1.25:1

  4. Etiology Theory: Adamantinoma arises from aberrant nests of epithelial cells – this would explain why the high occurrence in the subcutaneous proximal tibia Theory: Osteofibrous dysplasia is benign precursor to adamantinoma ? 2 types – classic (>20yo) and differentiated (<20yo – transformed from OFD)

  5. Clinical Slow growing  therefore symptoms may be present for years Most common symptom: Pain Occasionally palpable mass (due to subcutaneous location) 20% present with pathologic fracture

  6. Radiological Xray: Geographic Lucent Eccentric Usually, multiple lesions separated by areas of sclerosis “soap bubbles” Cortical thinning No periosteal reaction Usually, no soft tissue mass

  7. Radiological CT Not as useful as MRI MRI T1: low signal intensity T2: high signal intensity

  8. DDx osteofibrous dysplasia fibrous dysplasia ABC chondromyxoid fibroma chondrosarcoma

  9. Pathology Gross: Lobulated Rubbery May have focal areas of hemorrhage and necrosis May have bone spicules and cysts filled with blood

  10. Pathology Microscopic: “Islands” or “nests” of epithelioid cells in a fibrous stoma May resemble fibrous dysplasia or osteofibrous dysplasia Minimal nuclear atypia, rare mitotic figures Immunohistochemical staining: + for cytokeratins and vimentin

  11. Treatment Wide resection or amputation Radio-resistant Chemo not shown to be effective

  12. Prognosis Prognosis depends on surgical margins Recurrence in 25 - 32% who do not undergo wide resection or amputation (< 10% by wide excision) Mets occur in up to 30% Mets usually in lungs or lymph nodes 85% survival at 10 yrs? Long term follow up very important as tumour is slow-growing

  13. THANK YOU

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