Background
Origin
Theory | Authors, year |
---|---|
1. Fetal crest | Fischer, 1913 [4] |
2. Basal cell lineage, Trauma | Ryrie, 1932 [6] |
3. Implantation | Dockerty and Meyerding, 1942 [7] |
4. Unknown | Baker et al, 1954 [8] |
5. Synovial cell like | Lederer and Sinclair, 1954 [9], Naji et al 1964 [10] |
6. Angioblastic | Changus et al, 1957, Elliot 1962, Llombart bosch and Ortuno-pacheco, 1978, Reed, 1982 [11-14] |
7. Mesenchymal | Vinogradova, 1969 [15] |
8. Dermal inclusion | Lichtenstein, 1977 [16] |
9. Epithelial cell | Jaffe, 1958, Saacebra et al, 1968, Rosai and Pincus,1982, Ishida et al,1992, Hazelbag et al, 1993, Jundt et al, 1995 [17-22] |
Clinical features
Radiology
Pathology
Features | Classic | Differentiated |
---|---|---|
Age | More than 20 years, Adults | Less than 20 years, Children |
Radiology | Soft tissue or intramedullary involvement regularly observed | Intra cortical location indistinguishable from OFD |
Histopathology | Admixture of both epithelial and osteofibrous component, most commonly solid nests of basaloid cells | OFD like pattern lacks conspicuous nests and masses of epithelial cells. Scattered positivity of epithelial elements for cytokeratin |
Behavior | Aggressive clinical course | Relatively benign |
Gross appearance
Microscopic examination
Relationship between adamantinoma and OFD
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] |
Relationship between adamantinoma and Ewing's sarcoma
Immunohistochemistry
Cytogenetics
Author | Type of adamantinoma | Karyotype |
---|---|---|
Mandahl et al [68] | Classic | 46,XX,t(1;13;22), t(15;17) |
Sozzi et al [69] | Classic | 52,XY +7,+12,+13,+19,+der(7), +der(13) |
Hazelbag et al [70] | Classic | 50,XY,+7,+8,+12,+19 |
Classic | 50,XY,+7,+8, +12,+19 | |
Classic | 51,XY,+X,+7,+12,+19,+21/50, | |
Differentiated | 48,XY,+7,+8 | |
Differentiated | 46,XX,t(2;11), inv(11) | |
Kanamori et al [55] | Classic | 54,XX,-1,+5,+der(7),+der(8), +der(9),+19,+20,+21,+mar1,+mar2 |
Classic | 7,8,12,19 extra copies | |
Classic | 7,8,12,19 extra copies | |
Differentiated | 54,XY,+5,+7,+8,+12,+12,+14,+19,+21/53 |
Cytopathology
Author | Age of patient | Site of tumor | Behavior of tumor | Cytologic features |
---|---|---|---|---|
Hales and Ferrell [71] | NA | Tibia | Primary | Peripheral palisading, and prominent extracellular hyaline-like material |
Galera-Davidson, et al [72] | 31 | Tibia | Recurrent | Single-lying cells, small clusters and Indian files, with some nuclear molding. Three cell types (1) large polygonal cells with chromatin clearing, (2) smaller cells with poorly defined cytoplasm and dense chromatin and (3) fusiform cells |
Laucirica, et al [73] | 25/M | Tibia | Primary | Numerous sheets and single cytologically bland spindle cells with "chromatinic membrane folds" |
Perez-Ordonez and Bedard [74] | Two cases | Lung | Metastatic | Small round and spindle cells with indistinct cytoplasm. The nuclei had delicate nuclear membranes, with finely dispersed chromatin and occasional micronucleoli. No pleomorphism. |
Tabei, et al [75] | One case | Lung | Metastatic | Clusters of small cells with either prominent nucleoli or spindle-shaped hyperchromatic nuclei. |
Flowers et al [76] | One case, 32/M | Tibia, Lung | Recurrent and metastatic | Numerous nuclear membrane grooves within the polygonal cells |
Differential diagnosis
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 oft en 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 |