Table 6

Differential diagnosis of Adamantinoma

Lesions

Age (yrs)

Sex M:F

Site

Location

Clinical Features

Radiological Features

Gross

Microscopy

IHC

Treatment

Remarks


Aneurrysmal bone cyst

10–15

1:1

Vertebrae, flat bones, humerus, tibia

Metaphysis

Usually history of trauma, f/b gradually increasing swelling with little pain. There may be pathological fracture or spinal pressure symptoms

Well defined radiolucent, eccentric cyst

Spongy hemorrhagic mass

Fibrous tissue, vascular spaces

IGF-1

Curettage with bone grafting

May heal spontaneously Benign

Unicamaral bone cyst

10–20

3:1

Humerus, femur

Metaphysis

Usually asymptomatic

Well demarcated, radiolucent cyst extending up to physeal plate

Cystic mass

Well vascularised fibrous tissue with hemosiderin and cholesterol clefts

NA

Curettage with bone grafting

Benign

Fibrous dysplasia

10–30

3:2

Neck of femur, tibia, base of skull

Metaphysis diaphysis

May be mono or polyostotic, pathological fractures and progressive deformity

Cystic areas in metaphysis, lucent patches typically have ground glass appearance

Coarse gritty, Grayish yellow

Loose cellular fibrous tissue with widespread patches of woven bone and scattered giant cells

NA

Depending upon location and type of deformity

Locally aggressive, rarely sarcoma Develops, Associated Albright syndrome

Chondromyxoid Fibroma

10–25

1:1

Tibia, fibula, femur, feet, pelvis

Metaphysis

Asymptomatic, pathological fracture

Eccentrically placed lytic lesion with well defined sclerotic margins

Solid yellowish white or tan

Patches of myxomatous tissue with stellate cells, islands of hyaline cartilage, fibrous tissue

S-100

Excision

Benign

Giant cell tumor

20–40

4:5

Epiphysis and metaphysis

Femur, tibia, radius

Pain with swelling, pathological fracture

Eccentric, cystic lesion in mature bone, extending up to the subchondral plate, soap bubble appearance

Reddish fleshy mass

Multinucleated giant cells, stromal cells, cellular atypia with mitotic figures

Muscle actin, alpha-smooth muscle actin and CD68

Depending upon severity of lesion, curettage with bone graft to excision

Potentially malignant, 50% recur, 10% metastasize

Eosinophilic granuloma

5–10

2:1

Metaphysis

Flat bones, mandible, spine and long bones

Local pain, swelling and tenderness

Well demarcated oval radiolucent area, associated with marked reactive sclerosis

Soft, granular or gelatinous mass

Sheets of Langerhan's cells

CD-1a, S-100

Excision or curettage

Usually heals spontaneously

Osteomyelitis

Any age

Male outnumbers female

Metaphysis, diaphysis

Distal femur, proximal tibia, proximal femur and proximal humerus

Discharging sinus, fever, malaise, local pain and swelling

Multiple aggressive lytic lesions, serpiginous lytic pattern is more specific sequestrum and involucrum are often seen

Bone destruction, cavities containing pus with sequestrum

Inflammatory cells around areas of acellular bone or microscopic sequestra, prominent periosteal bone proliferation

NA

Removal of sequestrum antibiotic, excision of sinuses

Variable prognosis

Chondrosarcoma

30–60

3:1

Metaphysis

Pelvis, rib, humerus, femur, vertebrae

Dull ache or gradullay enlarging lump

Radiolucent area with central flecks of calcification

Lobulated with gelatinous shiny areas

Lobules of highly atypical cells, including binucleate cells.

S-100, Vimentin

Wide excision

Malignant

Epithelial metastasis

Any age

Variable

Pattern of these lesions is more diffuse than regional

Vertebrae, pelvis, rib, femur, skull, humerus(rare below elbow and knees)

Pain

Bone destruction, osteolytic; osteoblastic response with Ca prostate

Osteolytic, rarely sclerotic

Malignant cells with vascular invasion

Depend on the site of primary

Osteoclast inhibiting agents, radiation therapy

Most common primaries breast, prostate, lung, kidney, and thyroid

Hemangioendothelioma

20–30

2:1

Metaphyseal, diaphyseal, or, less commonly, epiphyseal.

Calvarium, femur, tibia and feet

Pain and swelling

Expansive, osteolytic and poorly demarcated lesions. "soap-bubble" matrix with a sclerotic margin

Well-circumscribed, irregular borders soft, bright red hemorrhagic appearance

Solid nests and anastomosing cords of round, polygonal, or spindle-shaped cells with eosinophilic cytoplasm. Intracytoplasmic vacuolization

Factor VIII, CD31, CD34

Depending on the grade of the lesion currettage, or wide resection

Multifocal in up to 50% of cases and may be mono-ostotic or polyostotic locally aggressive, metastasize to bone and lung

Angiosarcoma

Any age

Older male

Metaphyseal and diaphyseal

Any bone, multifocal

Pain and swelling

Eccentric, lytic, metaphyseal and diaphyseal, well circumscribed areas of rarefaction

Variable

Anastomosing vascular channels lined by highly atypical endothelial cells

Factor V11I, CD31, CD34

Wide resection and adjuvant therapy

Malignant

Nonossifying fibromas

10–20

1:1

Tibia, femur

Metaphysis

Pain

Eccentric, sharply delimited lesion

Solid, Granular, brown, dark red

Fibrous tissue arranged in storiform pattern, foamy and hemosiderin laden macrophages

Little or no application

Not necessary

Benign


ABBREVIATION: IGF: Insulin like growth factor, IHC: Immunohistochemistry, f/b: followed by, NA: Not applicable

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

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