Adamantinoma*

By Ted Scriven

Collected by
Sidho Hantoko
 Definition
Adamantinoma is a malignant bone tumour
 Epidemiology
1. Rare
2. < 1% of primary bone tumours
3. Patents mostly 2nd and 3rd decade of life, but wide range overall
4. Predilection for tibia - ~ 80% of cases
5. Usually occurs in diaphysis
6. Slight male predominance 1.25:1
 Etiology
1. Theory:
Adamantinoma arises from aberrant nests of epithelial cells
this would explain why the high occurrence in the
subcutaneous proximal tibia
2. Theory:
Osteofibrous dysplasia is benign precursor to adamantinoma
? 2 types classic (>20yo) and differentiated (<20yo
transformed from OFD)
 Clinical
1. Slow growing therefore symptoms may be present for years

2. Most common symptom: Pain

3. Occasionally palpable mass (due to subcutaneous location)

4. 20% present with pathologic fracture

 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
 Radiological
CT
Not as useful as MRI

MRI
T1: low signal intensity
T2: high signal intensity
 DDx
1. Osteofibrous dysplasia
2. Fibrous dysplasia
3. Abc
4. Chondromyxoid fibroma
5. Chondrosarcoma
 Pathology
Gross:
Lobulated
Rubbery
May have focal areas of
hemorrhage and necrosis
May have bone spicules and
cysts filled with blood
 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
 Treatment
1. Wide resection or amputation
2. Radio-resistant
3. Chemo not shown to be effective
 Prognosis
1. Prognosis depends on surgical margins
2. Recurrence in 25 - 32% who do not undergo wide resection or
amputation (< 10% by wide excision)
3. Mets occur in up to 30%
4. Mets usually in lungs or lymph nodes
5. 85% survival at 10 yrs?
6. Long term follow up very important as tumour is slow-growing