Odontgenic tumors vii / dental implant courses by Indian dental academy

Odontogenic Tumors
INDIAN DENTAL
ACADEMY
Leader in continuing
Dental Education
www.indiandentalacademy.com

LEARNING OBJECTIVES
At the end of the lecture student should be able to
• Describe etiology,
• Enlist clinical features,
• Enlist radiological features,
• Enlist Histopathological features of Metastasizing
ameloblastoma, Ameloblastic carcinoma, Primary
intraosseous carcinoma, Ghost cell odontogenic
carcinoma, Clear cell odontogenic carcinoma,&
Ameloblastic fibrosarcoma

Metastasizing
Ameloblastoma
• Ameloblastoma can be locally aggressive &
occasionally can metastasize & kill the patient.
• It shares many histologic features and clinical
behavioral attributes with cutaneous basal cell
carcinoma
• Metastasizing ameloblastoma, ambiguously termed
‘‘malignant ameloblastoma’’, clearly demonstrates the
biologic behavior of a well-differentiated low-grade
carcinoma

• Arises in the mandible of a young adult.
• The average age at presentation is 30 years, but 33% of
patients are younger than 20 years of age.
• Metastatic nodules develop in the lung (80%), cervical lymph
nodes (15%), or extragnathic bones.
• Pulmonary metastases are multifocal and involve both lungs
• Median survival after metastasis is 2 years
Clinical features

• Single to multiple recurrences of the primary ameloblastoma
• The multiple recurrences could result from
– Aggressive tumor
– Surgery associated tumor ‘‘spillage’’ into adjacent tissue
– Tumor embolization into lymphatic or blood vessels.
• Pulmonary metastases result from aspiration of tumor
fragments during multiple surgical procedures for recurrent
ameloblastoma is highly questionable;
• Intravascular spread
• Lymphatic spread

• Treated surgically
• Cervical metastases are managed by neck dissection
• If adequate pulmonary function can be preserved, lung
metastases (often multiple, small nodules that involve lower
lung lobes bilaterally) can be excised by lobectomy
• At surgery, more numerous pulmonary metastatic deposits
often are identified
• Chemotherapy ineffective but a short-term partial response is
possible

Ameloblastic
carcinoma
• Malignant epithelial proliferation that is associated
with an ameloblastoma (carcinoma ex
ameloblastoma) or histologically resembles an
ameloblastoma (de novo ameloblastic carcinoma)
• An aggressive neoplasm that is locally invasive & can
spread to regional lymph nodes or distant sites, such
as lung and bones
• The prognosis is poor.

Primary intraosseous
carcinoma
• A squamous cell carcinoma that occurs in the jaw
bone.
• If the intraosseous carcinoma demonstrates mucous
cells, then a diagnosis of central mucoepidermoid
carcinoma is made
• PIOC presents clinically as a diffuse enlargement of
the jaw.
• PIOC epithelium can extend upward to become
confluent with gingival surface stratified squamous
epithelium.

Solid primary
intraosseous carcinoma
• Typical solid PIOC presents as a painful mass in the
posterior mandible
• Mean age at presentation is 52 years, 20% of patients
are younger than 34 years & 39% are older than 65
years
• Clinically, patients present with pain, swelling,&
paresthesia.
• Radiographically, a cup-shaped radiolucent lesion,
poorly-circumscribed ‘‘moth eaten’’ radiolucency,
• .

• Evidence of cervical metastases.
• The overall 5-year survival rate is 38%.
• Most often, the tumor is treated by surgical excision and
postoperative radiation therapy.
• Most deaths occur within 2 years of therapy

Cystic primary
intraosseous carcinoma
PIOC arising in an odontogenic cyst, PIOC ex odontogenic cyst
• Squamous cell carcinoma that demonstrates a cystic
component with a lumen that contains fluid or keratin & a lining
of stratified squamous epithelium that exhibits cytologic atypia
• The cystic carcinoma can be lined by stratified squamous
epithelium that shows minimal to marked epithelial dysplasia.

Ghost cell Odontogenic Carcinoma
• Ghost cell odontogenic carcinoma (odontogenic ghost cell
carcinoma, malignant epithelial odontogenic ghost cell tumor,
aggressive [malignant?] epithelial odontogenic ghost cell
tumor, dentinogenic ghost cell tumor) is an ameloblastic
carcinoma that shows evidence of ghost cell keratinization
• Can be considered as a variant of ameloblastic carcinoma.
• Ghost cells are seen.

• Mean age - 38 years, but the age range is from 13 to 72
years;
75% arose in men. About 66% of cases occur in the
maxilla.
• More frequent in Asians
• Radiographically, presents as an expansile
multiloculated to
• poorly-delineated radiolucent lesion

• Demonstrate sheets of small basaloid cells that show
intraepithelial islands stratified squamous epithelium
that exhibit ghost cell keratinization
• The tumor can be locally aggressive, maxillary tumors
can invade the orbit & the cranial base but little
metastatic potential
• Treated by surgical excision, postoperative radiation
therapy, & sometimes chemotherapy.

Clear cell odontogenic
carcinoma
• Composed of cells that show uniform nuclei & clear
cytoplasm
• It presents in the mandible with an age range of 17 to
89 years in females
• Presents as a unilocular expansile radiolucent lesion
with an indistinct periphery; however, some cases are
multiloculated & well-circumscribed
• Approximately 20% of tumors show cervical lymph
node metastases; 17% display lung metastases; and
about 20% of patients die of disease

• Lesional clear cells exhibit
central to eccentric small dark
uniform nuclei; little evidence of
nuclear pleomorphism or
mitotic activity; abundant pale
cytoplasm, distinct cell borders
• Tumor islands can display
peripheral ameloblastomatous
palisaded columnar cells;
however, no evidence of
central stellate reticulum,
squamous differentiation, or
cystic change is observed

• The histologic differential diagnosis includes
– Clear cell calcifying epithelial odontogenic tumor
– Metastatic renal cell carcinoma
– Clear cell variant of mucoepidermoid carcinoma
– Clear cell squamous cell carcinoma

• A malignant proliferation of connective tissue cells that contains
benign odontogenic epithelium that is similar to that seen in
ameloblastic fibroma
• Posterior mandible
• Age range is from 3 to 83 years.
• An expansile radiolucency with indistinct margins, evidence of
extraosseous soft tissue extension
• About 35% of cases arise in an ameloblastic fibroma

• Approximately 37% of patients have one or more recurrence
and 19% die of disease.
• Patients do not develop metastases; they die of a locally
aggressive neoplasm.
• Treated most often by a wide surgical excision and
postoperative radiation therapy, without elective neck
dissection.
• If the tumor recurs, it tends to display greater stromal cell
cellularity, increased mitotic activity, more pronounced nuclear
atypia, and less evidence of odontogenic epithelium with each
recurrence

• Has the histologic architecture of
an ameloblastic fibroma.
• Slender, budding and branching
epithelial cords of bland cuboidal
to columnar cells with uniform
nuclei or epithelial islands
• Separated widely by hypercellular
connective tissue that exhibits
plump polygonal to fusiform
stromal cells

• Mild to moderate cytologic
atypia
• Numerous mitotic figures in a
pale hypocollagenous myxoid
extracellular matrix.
• Focal evidence of dentin
formation or dentin & enamel
formation & is termed as
‘‘ameloblastic dentinosarcoma’’
& ‘‘ameloblastic
odontosarcoma,’’ respectively.

Summary
• Etiology, Clinical features, Radiological features,&
Histopathological features of Metastasizing
ameloblastoma, Ameloblastic carcinoma, Primary
intraosseous carcinoma, Ghost cell odontogenic
carcinoma, Clear cell odontogenic carcinoma,&

BIBLIOGRAPHY
• Text book of oral pathology Shafer's, 5 & 6th edition
• Odontogenic Tumors & Allied Lesions Reichart/
Philipsen Ist edition
• Color Atlas of Oral Diseases Cawson, R. 2nd edition
• Oral and Maxillofacial Pathology Neville, Brad W. 2nd
• Lucas’s Pathology Of Tumor’s of the Oral Tissues
• Cawson, R. A., Bennie, W. H 5th edition

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