Table 3

Differences between Osteofibrous dysplasia and Adamantinoma

Features

Osteofibrous dysplasia

Adamantinoma


Nature

Benign condition

Locally aggressive

Age

Less than 10 years

2 year to 86 years

Site

May involve both tibia and fibula.

90% tibial involvement, In 10–15% cases, ipsilateral fibular involvement, Rarely pretibial soft tissue and other bones

Clinical presentation

• Pain, swelling, pseudoarthrosis, bowing, pathological fracture may occur

• With or without Pain, swelling, pathological fracture in 25% cases

• seldom progresses during childhood, and any progression of the lesion stops after puberty

• progressive during adult age

Predisposing history of trauma

Absent

Present

Radiology

Periosteal reaction present

Periosteal reaction is variable

Intra cortical

15% of cases, there is extracortical extension into soft tissues

Limited to anterior cortex

Single or multiple nodular lesions in one or more foci in medulla

Well marginated with marginal sclerosis, ground glass appearance

Sharply or poorly delineated osteolytic lesion. with septations and peripheral sclerosis, characteristic "soap-bubble" appearance

Histopathology

Zonal phenomenon present

Scattered epithelial cells recognized on IHC

Absent

Presence of epithelial cells forming small nests/strands recognized in H&E

Recurrence

Local recurrence in 25%.

Tends to recur in 18–32%

Metastasis

No metastasis

Metastases may occur in 15–30%, Lung and Lymph nodes usually involved

Regression

Spontaneous regression at puberty in 33% cases

Regression ± [45]


Jain et al. Diagnostic Pathology 2008 3:8   doi:10.1186/1746-1596-3-8

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