Dent 356-11: Odontomes & Odontogenic Tumors II

Dr. Rima Safadi

Squamous Odontogenic Tumor

Rare tumor presenting with tooth

mobility.

Radiographically presents as a well-circumscribed, semilunar or triangular radiolucency associated with roots of teeth. Histologically consists of islands of benign, well-differentiated squamous epithelium. Thought to b e derived from epithelial rests of Malassez. Although some lesions may be aggressive, curettage is the treatment of choice.

Calcifying Epithelial Odontogenic Tumor (Pindborg Tumor*)

Rare, benign, but locally invasive epithelial neoplasm. Wide age range. Mandible > maxilla.

Most arise in molar and premolar areas.

~50% associated with crown of unerupted tooth. Extraosseous examples reported. Locally invasive but less aggressive than ameloblastoma.

Calcifying Epithelial Odontogenic Tumor (Pindborg Tumor): Radiographic Features

Irregular radiolucent area, may or may not be clearly demarcated. May contain varying amounts of radiopaque bodies due to calcification.

Calcifying Epithelial Odontogenic Tumor (Pindborg Tumor): Histopathologic Features

Sheets and strands of polyhedral epithelial cells with abundant eosinophilic cytoplasm in fibrous stroma. Cells have prominent intercellular bridges and marked nuclear pleomorphism, nevertheless it is not malignant.

Amyloid-like material may be present, it may become calcified.

Adenomatoid Odontogenic Tumor: Clinical & Radiographic Features

2nd and 3rd decades. Majority in anterior maxilla, especially canine area. Slowly enlarging swelling.

Well-defined radiolucency, may have radiopacities. often associated with unerupted tooth, simulating dentigerous cyst. Rare extraosseous variant.
Treatment is conservative enucleation.

Adenomatoid Odontogenic Tumor: Histopathologic Features

Well-encapsulated, solid or cystic. Sheets, strands, masses of epithelium which in some places forms duct-like structures lined by columnar epithelium. Small foci of calcification and occasional dentin and enamel matrix may be seen. Treatment is conservative enucleation.

Ameloblastic Fibroma

Rare benign tumor with both epithelial and mesenchymal components neoplastic. Important to differentiate from ameloblastoma since it is not invasive and does not require aggressive therapy. Presents in young age groups. Slowly enlarging swelling mostly in posterior parts of mandible.

Ameloblastic Fibroma: Radiographic Features

Radiographically, welldefined unilocular radiolucency. May be associated with an unerupted tooth and mimic a dentigerous cyst. Treated conservatively. Recurrence rates of ~18%.

Ameloblastic Fibroma: Histopathologic Features

Proliferating strands of odontogenic epithelium lying in highly cellular fibroblastic tissue resembling dental papilla. Epithelium resembles that of ameloblastoma but stellate cells are much less abundant. If dentin is present, the tumor is designated ameloblastic fibrodentinoma.

If there is also enamel formation, they are designated ameloblastic fibro-odontoma.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst) & Dentinogenic Ghost Cell Tumor

The calcifying cystic odontogenic tumor is a grossly cystic tumor and may be a hamartoma rather than a true neoplasm. The dentinogenic ghost cell tumor is histologically similar except that it is solid. It was originally considered to represent the solid variant of the calcifying cystic odontogenic tumor (calcifying odontogenic cyst or Gorlin cyst).

Increasing evidence that they are distinct entities.

Both present centrally within the jaws, but peripheral gingival lesions also occur.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Clinical Features

The calcifying cystic odontogenic tumor usually occurs below age 40.

75% intraosseous.
Majority arise anterior to 1st molar in either jaw. Slowly enlarging swelling.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Radiographic Features

Well-defined unilocular or multilocular raadiolucency with variable amounts of radiopaque material.

May be associated with crown of an unerupted tooth. Usually responds to conservative treatment.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Histopathologic Features

Cyst lined with epithelium similar to ameloblastoma, but shows ghost cell keratinization. Ghost cells may undergo calcification. There may be dentin-like masses.

Calcifying Cystic Odontogenic Tumor (Calcifying Odontogenic Cyst or Gorlin Cyst)

Histopathologic Features

Dentinogenic Ghost Cell Tumor

The dentinogenic ghost cell tumor tends to occur in an older age group than the calcifying cystic odontogenic tumor. Some cases respond to conservative treatment. Other cases pursue a more aggressive course and are locally invasive like ameloblastoma.

Odontogenic Fibroma and Myxoma

Derived from mesenchymal dental tissues. May arise in relation to root, crown, or replace a tooth. These different presentations reflect the different mesenchymal tissues that may give rise to the tumors-PDL, dental follicle, and dental papilla.

Odontogenic Fibroma
Clinical Features

The central variant is an uncommon, welldemarcated benign fibroblastic neoplasm.

Readily enucleated with no tendency for recurrence. Peripheral odontogenic fibroma arises on the gingiva.

Odontogenic Fibroma
Histopathological Features

Cellular fibrocollagenous tissue containing inactive odontogenic epithelial islands.

Foci of cementum-like and dentin-like material may be present.

Odontogenic Fibroma
Histopathological Features

Odontogenic Myxoma
Clinical Features

Myxoma is benign but locally invasive.

More common than odontogenic fibroma.

Odontogenic Myxoma
Radiographic Features

Multilocualr radiolucency, soap-bubble, or tennis-racket appearance, often with well-defined margins. Root resorption.

Odontogenic Myxoma
Histopathological Features

Nonencapsulated, infiltrative pattern of growth. Stellate, fibroblast-like cells with long anastomosing processes. Abundant connective tissue ground substances, predominnaltly glycosaminoglycans.

Odontogenic Myxoma
Histopathological Features

Odontogenic Myxoma
Histopathological Features
Some cases contain a few strands of odontogenic epithelium. Variable amounts of collagen, and if prominent it may be designated as myxofibroma or fibromyxoma.

Odontogenic Myxoma: Behavior

Unlike fibroma, the locally invasive growth of myxoma and friable myxoid tissue predispose to local recurrence. Recurrence rates of 10-30%.

Cementoblastoma

Although cementum is considered to be a modified form of bone, cementoblastoma is still classified as an odontogenic tumor because of its unique association with the root of a tooth. Identical to osteoblastoma except for association with tooth roots.

Cementoblastoma
Clinical Features

A rare, benign neoplasm. Mostly seen < 25 years of age. Usually mandibular molarpremolar area attached to a root of a tooth. Most cases involve mandibular 1st permanent molar.

Slowly enlarging swelling which sometimes gives rise to pain. Involved tooth is vital. May recur if incompletely removed.

Cementoblastoma
Radiographic Features

well-circumscribed, mottled or dense radiopaque mass with a radiolucent margin attached to the root of a tooth which usually shows resorption.

Cementoblastoma
Histopathologic Features

Mass of calcified cementum-like tissue containing scattered cells lying in lacunae growing in continuity with the apical cemental layer of the root.

Cementoblastoma
Histopathologic Features

Around the periphery and in actively grwoing parts, extensive sheets of uncalcified matrix formed by plump, deeply staining cementoblasts.

Malignant Odontogenic Tumors

Malignant Ameloblastoma & Ameloblastic Carcinoma

Very rarely, an ameloblastoma shows true malignant behavior with metastasis. The primary and metastatic neoplasms show histopathologic features of typical ameloblastoma, i.e. no atypia is seen. If only lung metastasis is seen the possibility of seeding due to aspiration during surgery should be considered.

If the tumor shows features of atypia, it is given the diagnosis of ameloblastic carcinoma.

Malignant Odontogenic Tumors

Primary Intraosseous Carcinoma

Rarely, carcinomas arise within the jaws without a pre-existing cyst. They are presumed to arise from residues of odontogenic epithelium.

They may also be referred to as odontogenic carcinomas.

Malignant Odontogenic Tumors

Malignant Change in Odontogenic Cysts

A squmaous cell carcinma may present with clinical and radiographic or histological evidence consistent with an origin from an odontogenic cysts. The cysts include radicular, residual, dentigerous and keratocysts. Possibility of cystic degeneration within an existing carcinoma or carcinoma involving an adjacent cyst. Dysplasia is seen sometimes in long standing odontogenic cysts.

Malignant Odontogenic Tumors

Odontogenic Sarcomas
Malignant

counterpart of ameloblastic fibroma and related lesions.

Fibrosarcomas

containing nonneoplastic odontogenic epithelium and occasionally dental hard tissues, e.g. ameloblastic fibrosarcoma, fibrodentinosarcoma.

Tumors of Debatable Origin

Melanotic Neuroectodermal Tumor of Infancy

Rare tumor which occurs in infants usually in the first year of life. Most common in maxilla tha mandible or elsewhere.
Symptomless mass expanding the bone.

Tumors of Debatable Origin

Melanotic Neuroectodermal Tumor of Infancy

Evidence of neural crest origin. Most are benign and recurrence is uncommon following conservative excision.

Tumors of Debatable Origin

Congenital Epulis

Also known as: congenital gingival granular cell tumor, congenital granular cell epulis, congenital epulis of the newborn. A unique and rare congenital tumor of the alveolar mucosa of the jaws that occurs only in newborns. 10 times more common in females.

Predilection for anterior maxilla.

Tumors of Debatable Origin

Congenital Epulis

Large, closely packed granular cells.

Resembles granular cell tumor, but histogenesis unknown.