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Adamantinoma Ted Scriven Sept 15 th, 2008. Adamantinoma is a malignant bone tumour Definition.

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Presentation on theme: "Adamantinoma Ted Scriven Sept 15 th, 2008. Adamantinoma is a malignant bone tumour Definition."— Presentation transcript:

1 Adamantinoma Ted Scriven Sept 15 th, 2008

2 Adamantinoma is a malignant bone tumour Definition

3 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 Slight male predominance  1.25:1 Epidemiology

4 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) Etiology

5 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 Clinical

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

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

8 osteofibrous dysplasia fibrous dysplasia ABC chondromyxoid fibroma chondrosarcoma DDx

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

10 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 Pathology

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

12 Prognosis depends on surgical margins Recurrence in % 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 Prognosis

13 THANK YOU


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