Odontogenic Cysts And Tumors

Cyst: its a pathological cavity lined by epithelium & filled with fluid or semi fluid material. In the jaw bones & oral tissue there are remnant of epith. come mostly from odontogenic apparatus & most of these remnants are embryonic in origin .for this reason the jaw bone are affected by a large numbers of cysts in comparism to other bones in the body. The epith. remnants that give rise to these cysts are: 1- rest of malasses (e.g. P.A.C & residual cyst) 2- reduced enamel epithelium (dentigerous cysts & eruption cyst) 3- remnants of the dental lamina (rest of serres) (e.g. odontogenic keratocyst, lateral periodontal cyst, gingival cyst & glandular odontogenic cyst).

What is a cyst?

An abnormal space within tissue lined by epithelium

Classification of the jaw cysts

I-Epithelial cysts A- odontogenic: 1- developmental cyst (odontogenic keratocyst, dentigerous cyst, eruption cyst, lateral periodontal cyst, gingival cyst, glandular cyst) 2- inflammatory cyst (radicular cyst, paradental cyst) B- non-odontogenic: Nasopalatine duct cyst, nasolabial cyst II-Non epith. (primary bone cysts) Solitary bone cyst (traumatic, simple), aneurysmal bone cyst, Stafine cyst III-Soft tissue cysts: Salivary mucoceles (mucus extravasation, mucus retention) Dermoid & epidermoid cyst Lymphoepithelial cyst Thyroglossal tract cyst

Odontogenic cysts

A cyst in which the lining of the lumen is derived from epithelium produced during tooth development, these include: 1- cysts derived from rests of Malassez: Periapical cyst (radicular)

2- cysts derived from reduced enamel epithelium: reduced enamel epithelium refers to the layer of epith. That remains around the tooths crown after enamel formation is complete. This layer of epith. Is derived from the specialized epith. Components of the enamel organ (inner enamel epith., stratum intermedium, stellate reticulum, & outer enamel epith.)That were active during amelogenesis, & collapse into a thinned dormant membrane of two or three cells in thickness.

Odontogenic Cysts Inflammatory Periapical (radicular) cyst Residual periapical (radicular) cyst Buccal bifurcation cyst (usually first molars) Paradental cysts (partially erupted third molars

Radicular (Periapical) Cyst

!Most

common (65%)

!Epithelial

cell rests of Malassez !Response to inflammation !Radiographic findings

!Pulpless, !Small

nonvital tooth

well-defined periapical radiolucency

!Histology !Treatment

- extraction, root canal

Radicular Cyst

Radicular Cyst

Periapical Cyst vs. Periapical Granuloma CANNOT TELL THE DIFFERENCE BY X-RAY CAN ONLY TELL BY HISTOLOGY Periapical Granuloma Granulation Tissue - No epithelial lining

Periapical Cyst True cyst with central lumen and epithelial lining

Why does a periapical cyst form instead of just a granuloma? Simply the chance of Rests of Malassez being in the area of inflammation

Periapical Inflammation

Odontogenic Epithelial Rest

1. Cells in the rest proliferate due to the inflammation

2. The ball of cells gets so big the center gets too far from the blood supply, the cells in the center die

3. The center of the ball of cells then has a higher protein concentration than the surrounding tissue

4. Fluid flows into the center by osmotic pressure, cyst expands, more cells grow around the periphery, more cells in the center die, concentration increases again

5. Osmotic pressure can keep expanding the cyst independent of the inflammation

Result = Periapical Cyst

A radiolucency in the jaws must be investigated If the teeth in the area are vital, you must biopsy the radiolucent area. If non-vital, obviously RCT

Vitality Test! Vitality Test! Vitality Test! Vitality Test! Vitality Test!

Residual Cyst

Paradental Cyst
!Associated

with partially impacted 3rd

molars of inflammation of the gingiva over an erupting molar !0.5 to 4% of cysts

!Radiology !Result

- radiolucency in apical portion of the root !Treatment - enucleation

Paradental Cyst

Odontogenic Cysts
!

Inflammatory
Radicular ! Paradental
!

Developmental
Dentigerous ! Developmental lateral periodontal ! Odontogenic keratocyst
!

Glandular odontogenic
!

Odontogenic Cysts Developmental Dentigerous cyst Odontogenic keratocyst Orthokeratinized odontogenic cyst Gingival (alveolar) cyst of the newborn Gingival cyst of the adult Lateral periodontal cyst Calcifying odontogenic (Gorlin) cyst Glandular odontogenic cyst Eruption cyst

Cyst Lumen

Lining Epithelium

A- Dentigerous cyst : an odontogenic cyst that surrounds the crown of an impacted tooth, caused by fluid accumulation between the reduced enamel epith. & the enamel surface, resulting in a cyst in which the crown is located within the lumen & root outside.

The pathogenesis of this cyst is uncertain but apparently it develops by accumulation of fluid between the REE & the tooth crown. Progressive growth of the cyst leads to dilatation of the dental follicle.

Clinically: These cysts are commonly associated with unerupted mand. or max. third molars or max. canine. The cyst usually remains asymptomatic but may produce some swelling or pain, particularly if it is large or inflamed. Radiographically: Mostly diagnosed by their radiographic appearance. They present as well circumscribed radiolucency surrounding the crown of a tooth.

Dentigerous Cyst

Dentigerous tooth containing cyst Origin reduced enamel epithelium (dental follicle)

If a radiolucency is ASSOCIATED with an unerupted tooth Dentigerous cyst should be your FIRST differential diagnosis

Differential Diagnosis Dentigerous Cyst Odontogenic Keratocyst Ameloblastoma

Like other cysts, uncomplicated DC (small DC) causes no symptoms until swelling becomes noticeable & is discovered on a routine radiographic examination or when the cause is sought for a missing tooth. Large cyst may be associated with a painless expansion of the bone in the involved area, infection of a DC causes the usual symptoms of pain and accelerated swelling, such infection may arise in a DC that is associated with a partially erupted tooth or by extension from a periapical or periodontal lesion that affects an adjacent tooth.

Radiographically:

It appears as well circumscribed radiolucuncies surrounding the crown of an unerupted tooth which may displace from its normal position.
If the width of RL around the crown is less than + 2mm, it is considered fitting a normal tooth follicle, and if the RL space is of least 3 to 4 mm then a DC should be considered. The RL usually has well-defined and often sclerotic borders. A large DC may appear radiographically as a multilocular process because of the persistence of bone trabeculare within the RL. However, a keratocyst or ameloblastoma may occasionally envelop the crown of the tooth and appear radiographically similar to DC. The diagnosis ultimately therefore depends on histological examination thats why radiographical findings are not diagnosis for a DC.

Histopathology:

The gross examination of the wall of DC show nodular thickening, which must examined microscopically to role out early neoplastic changes.
The lining of a non-inflammated DC composed of stratified sequamous epithelium, rarely showing keratinization. Mucous - producing cells are sometime seen in the epithelium. The wall consists of loose fibrous C.T. with inactive odontogenic epithelial rests. Small nests of sebaceous cells rarely may be noticed with the fibrous cyst wall. These mucous and sebaceous elements are believed to represent the multipotentiality of the odontogenic epithelial lining in a DC. Therefore it's advisable to examined DC microscopically and to role out the presence of early neoplastic changes such as mucoepidermoid Ca, ameloblastoma and sequamous cell Ca.

In inflammed DC The fibrous wall is more collagenized with the chronic inflammatory cells, and the lining show hyperplasia and develop rete -ridges.

Histopathology:

The epith. Lining is relatively uniform layer of nonkeratinized, stratified, squamous epith., measuring 2-10 cells in thickness. This lining may alters by inflammation & may become hyperplastic, atrophic, or ulcerated. Variable numbers of mucus cells are occasionally seen in the epith. Lining . Like the periapical cyst, crystalline cholesterol deposits, hemosiderin deposits, hyaline (Rushton ) bodies, & lipid laden macrophage are also seen in dentigerous cyst. Long standing cyst will occasionally exhibit areas of keratinization or premalignant (dysplastic) changes of their epith. Lining. Treatment: either by enucleation or marsupilization, recurrency is uncommon

Dentigerous Cyst

Treatment Surgical enucleation, with removal of associated tooth. A large DC may be treated by marsupialization, which permits decompression of the cyst and reduction in the size of bone defect, especially in canines, and then we do alignment of the tooth properly by orthodontic appliance. Recurrences are very rare.

However, several potential complications must be considered when recurrence occurs. In which it will need more aggressive treatment.

B- Eruption cyst: Is a variant of the dentigerous cyst that develops in

the alveolar soft tissue around the crown of an erupting tooth.

Clinically, appear as fluctuant swelling of the alveolar ridge, the lesion may bleed during mastication giving rise to the term eruption hematoma Histologically, same as those of a dentigerous cyst. Most of these cysts require no treatment because rupture spontaneously during mastication. But sometime surgical exposure of the crown of affected tooth may be needed.

Eruption cyst: (eruption hematoma) An eruption cyst occasionally forms over a tooth about to erupt. Its a soft tissue analogue of the DC, results from separation of the dental follicle from around the crown of eruption tooth.

Presumably, the cysts developed because of collagen deposition in the gingival C.T. that resulted in a thicker per coronal roof.

Clinical features EC effect children and adult chiefly in the deciduous teeth or permanent molars. Its soft, fluctuant, translucent swelling in the gingival mucosa overlying the crown of unerupting tooth and rarely painful. Surface trauma may result in a considerable amount of blood in the cystic fluid which gives its blue to purple-brown color and that why some time referred as eruption hematomas. No X-ray findings appear because it occurs in soft tissue.

Histopathologically Surface oral epithelium covering the underlying lamina propria with inflammatory cells, and the deep of the bottom is the roof of the cyst, thin layer of non-keratinized sq. epithelium.

Treatment Treatment may not be needed because the cyst usually ruptures spontaneously permitting the tooth to erupt. Sometime simple excision of the roof of the cyst is needed to permits the eruption of tooth.

Developmental Lateral Periodontal Cyst

From epithelial rests in periodontal ligament!

Mandibular premolar region ! Middle-aged men ! Radiographic findings

!
!

Interradicular radiolucency, well-defined margins Nonkeratinizing stratified squamous or cuboidal epithelium

Histology
!

Treatment - enucleation, curettage with preservation of adjacent teeth

Lateral periodontal C These are uncommon type of developmental OC that typically occur along the root surface of a tooth, it arise from rests of dental lamina. Its asymptomatic lesion, mainly occur in mandibular caninepremolar region & less between maxillary lateral and canine.

Radio- graphically Appear as a well-circumscribed RL area located laterally to the root(s) of vital tooth.

Occasionally this cyst appears as multilocular (poly cystic) and termed: botryoid OC. The radiographic picture of lateral periodontal cyst is not diagnostic:
An odontogenic KC that develop between the roots of adjacent teeth may show identical radiographic findings

An inflammatory RC that occurs laterally to a root in relation to an accessory foramen A cyst that arises from periodontal inflammation.

Developmental Lateral Periodontal Cyst

Lateral Periodontal Cyst

Derived from remnants of the dental lamina

Histopathology The typical picture is thin lining of nonkeratinized epith. Which only 1-2 cell layers thick, with foci of glycogen-rich clear cells, some cyst exhibit focal epithelial thickening (plaque) of clear cells. Clear cell epithelial rests sometimes are seen within the fibrous wall.

Treatment Conservative enuncleation, no recurrence and avoid damages to adjacent teeth.

3- cysts derived from dental lamina:

A- Odontogenic Keratocyst ( okc): A cyst derived from remnants of the

dental lamina. Its less common than the dentigerous cyst but more serous because epith. Lining show high rate of growth.

Clinically:
Occurs in young patient, however, it can occur at any age. The mand. Is affected more commonly than max. in the mand., the cyst occur mostly in the 3rd molar area, while in the max., the 3rd molar & canine areas are usually affected. The cyst is slowly growing & asymptomatic, but later it may cause bone expansion. Although okc is usually present as a single lesion, it can occasionally occur as multiple cysts that sometimes occupy all four quadrants of the jaws as in nevoid basal cell carcinoma.

Odontogenic Keratocyst
of jaw cysts !May mimic any of the other cysts !Most often in mandibular ramus and angle !Radiographically
!Well-marginated, !Pericoronal,

!11%

radiolucency

inter-radicular, or pericoronal !Multilocular

Odontogenic keratocyst It has specific histopathological features and clinical behavior. It arises from cell rests of the dental lamina. It shows a different growth mechanisms and biological behavior from the more common dentigerous cyst and radicular cyst, which enlarged as a result of increased osmatic pressure within the lumen of the cyst. While the growth of the keratocyst may be related to unknown factors inherent in the epithelium itself or enzymatic activity in the fibrous wall. Thats why now day several investigators suggested that OKC be regarded as benign cystic neoplasms rather than cysts, and the latest WHO classification of odontogenic tumors, these lesions have given the name (keratocystic odontogenic tumors). It makes up 3% to 11% of all odontogenic cysts.

Clinical features

The peak incidence between ages 20 to 30 years, the mandible is usually affected, usually in the region of the jaw angle and the ascending remaus. KC like other jaw cyst, are symptom less until the bone is expanded or they become infected.
It grows in anterioposterior direction within mandibulary cavity of the bone without causing obvious bone expansion. This is useful to differentiate clinically and radiographically between DC & RC which produce bone expansion.

Multiple OK is seen in Nevoid basal cell carcinoma (Gorlin syndrome).

When multiple KC are found in the jaws, basal cell nervous syndrome appear to be involved in about half of cases. This syndrome is characterized by multiple KC, basal cell nevi of the skin and bifid ribs.

Radiographically Well defined RL area will smooth corticated margin. Large lesion in posterior body and ascending remaus of the mandible may appear multilocular. An unerupted tooth is involved in the lesion in 25% to 40% of cases. Differential diagnosis. DC (radiographic findings an highly suggestive but not diagnostic) Large RC & residual C which are near impacted tooth. Lateral periodontal cyst or globulomaxillary cyst if the KC is small and between teeth.

Odontogenic Keratocyst

Odontogenic Keratocyst

Odontogenic Keratocyst Because of its behavior, many oral pathologists now consider the OKC an odontogenic tumor that has a cystic form

2005 Classification by the W.H.O. Keratinocystic Odontogenic Tumor

Odontogenic Keratocyst (Keratinocystic Odontogenic Tumor) Three important things associated with this diagnosis: 1. High recurrence rate (up to 60%) 2. Highly aggressive (now considered by W.H.O. to be an odontogenic tumor) 3. Relation to Gorlin syndrome

Odontogenic Keratocyst Can be in the location of any odontogenic cyst Can be isolated in the jaws

Histopathology: the microscopic appearance of okc is

pathognomonic, characterized by:

Thin uniform lining of parakeratin sq. epith. 6-10 cells thickness. palisaded layer of columnar or cuboidal basal cells. Corrugated layer of parakeratin. Lack of rete ridges, and focal separation of epithelium C.T. wall. The C.T wall is often loose and fibrillar and free of inflammation. dental lamina rests and microcysts (daughter or satellite) cyst may present in the capsule wall the capsule wall is folded The lumen contains desquamated parakeratin.

Odontogenic Keratocyst Diagnosis entirely depends on the histology of the cyst lining

1. Uniform (5-8 cells) thickness 2. Hyperchromatic, cuboidal or columnar basal cell layer 3. Corrugated parakeratin layer 4. Virtually no inflammation in cyst wall

Odontogenic Keratocyst

Daughter Cyst

Odontogenic Keratocyst
!Histology

epithelial lining with underlying connective tissue (collagen and epithelial nests) !Secondary inflammation may mask features
!High

!Thin

frequency of recurrence (up to 62%) !Complete removal difficult and satellite cysts can be left behind

Nevoid Basal Cell Carcinoma (Gorlin) Syndrome Multiple basal cell carcinomas Multiple jaw cysts (odontogenic keratocysts) Numerous bone abnormalities including bifid ribs, intracranial calcification, vertebral anomalies Mild ocular hypertelorism Epidermal cysts of the skin Etc. Gorlin (Nevoid Basal Cell Carcinoma) Syndrome

Basal cell carcinomas in non-sun exposed areas

Multiple OKCs

Treatment of OKC
Depends on extent of lesion ! Small - simple enucleation, complete removal of cyst wall
! ! !

Larger - enucleation with/without peripheral ostectomy

promote complete resection with 1 cm bony margins (if extension through cortex, overlying soft tissues excised) ! Long term follow-up required (5-10 years)

The causes of recurrence are: Loose attachment between the epith. & C.T. Presence of daughter cysts

Orthokeratinized OC
It's an OC that shows microscopically an orthokeratinized epithelial lining. Although such lesion were originally called the orthokeratinized variant of OK . It represents 7% to 17% of all keratinizing jaw cyst. It appear clinically and radio graphically like dentigerous cyst, they mostly involve unerupted mandibular 3rd molar, occur predominantly in young adult and show 2:1 male to female ratio, more common in the mandible than the maxilla. They have the same clinical and radiographical features of inflammatory or developmental OC it appear as a unilocular RL but occasionally it may appear as multilocular.

Histopathology The cyst lining is composed of st.sq.epith., which shows an orthokeratotic surface of various thicknesses. The epithelial lining may be relatively thin and the prominent palisaded basal cell layers of OKC are not present.

Treatment
Enucleation with curettage, no recurrence but may be associated with greater risk of malignant transformation.

Orthokeratinised keratocyst. Perforation of and pressure on the cyst roof has caused keratin to extrude and has helped to confirm the diagnosis.

B- glandular odontogenic cyst:

an unusual large unilocular or multilocular odontogenic cyst, first described in 1987. it originated from epith. Of the dental lamina & it has greater growth potential

Radiographically: Appear as well defined, unilocular, or multilocular radiolucencies, mostly in the mandible.

Histopathology:
Its composed of a thin squamous epith. lining that may show areas of focal epith. thickening. The epith. Contain glandular structures or microcysts lined by columnar or cuboidal cells.

Treatment:
By surgical enucleation.

Glandular odontogenic cyst It is a rare type of developmental OC that show aggressive behavior, and it closely resemble polycystic variant of lateral periodontal cyst but has much greater growth potential and tendency to recur.

Clinical feature It occurs most commonly in middle aged adult, more in mandible and in anterior region of the jaws. Their size varies from small lesion less than 1cm to large destructive lesions that involve most of the jaw Radiographically: May appear as well defined unilocualr or multilocuallr RL.

Glandular Odontogenic Cyst

Histopathology Lining by sq.epi of varying thickness. The interface between the epith and the fibrous C.T. wall in generally flat. The fibrous wall is usually devoid of any inflammatory cell infiltrate. Small glandular structures of microcysts within epith. glandular structure lined by cuboidal cells, the lumen contain secretory products

Glandular Odontogenic Cyst

!

Histology
Stratified epithelium ! Cuboidal, ciliated surface lining cells ! Polycystic with secretory and epithelial elements
!

Glandular Odontogenic Cyst

Treatment of GOC
!Considerable

recurrence potential !25% after enucleation or curettage !Marginal resection suggested for larger lesions or involvement of posteriormaxilla ! close follow-up

Gingival cyst of newborn (alveolar) They are small, superficial, keratin-filled cysts that are found on the alveolar mucosa of infants. These cysts are arises from remnants of the dental lamina. It is appear as small, usually multiple whitish papules on the mucosa overlying the alveolar processes of neonates. Histopathology: show thin, flattened epith, lining with parakeratin. The lumen contain keratinaeous debris. Treatment: not indicated, lesion rupture spontaneously.

2 Week female brought to pediatric dentistry clinic parents were very upset

Gingival cysts of the newborn No treatment needed - theyll regress in a short time

Gingival cyst of the adult It considered the extra osseous form of the lateral periodontal cyst. Its uncommon lesion, it occurs mostly in mandibular canine and premolar area 60-75% and is most commonly found in patients in the 5th and sixth decades of life. They are almost located on the facial gingival or alveolar mucosa. It appears as small painless swelling bluish-gray in color. Histopathologic: resemble lateral periodontal cyst. Treatment: conservative surgical enucleation.

Gingival Cyst of the Adult

Lining derived from remnants of the dental lamina

Gingival cyst

Malignant lymphoma

Calcifying OC: (calcifying chost cell OC) Gorlin cyst Its uncommon lesion, that show divers histopathological feature and variable clinical behavior. Although it is widely considered to regards a cyst, some investigators prefer to classify it as a neoplasm. Recent WHO classification of OT groups, consider the calcifying OC with all its variants as an OT rather than an OC.

Clinical features COC Is predominantly an intraosscous and 1330% of cases reported as per- peripheral (extra osseous) lesion. Usually occur in maxilla region, the mean age is 33years. Those which associated with odontoma tend to occur in younger patients.

Radiographically: RL and multilocualr RL RO structures are present in about one third to one half of cases.

Calcifying Odontogenic (Gorlin) Cyst

Histopathology

The cyst lining consist of epithelium often with palisading of the basal cells which may resemble ameloblasts , the overlying layer of loosely arranged epithelium may resemble stellate reticulum.
The most striking features is areas of abnormal keratinization producing swollen cells whose outlines and nuclei become progressively polar (ghost cell) The nature of the ghost cell is controversial, some believe that this change represent 1-coagulative necrosis or accumulation of enamel protein. It is a form of normal or aberrant keratinization of Oral epithelium. Masses of ghost cells may fuss to form large sheets of amorphous a cellular materials. Calcification within the ghost cells is common. Treatment: surgical enucleation and prognosis good.

Ghost cells calcify

Squamous cell carcinoma arising in the epithelial lining of a cyst A rare, but definite possibility in any cyst

Odontogenic tumors: According to the origin of these tumors, they are classified as follows: Tumor of odontogenic epithelium (ectodermal origin).
Ameloblastoma

odontogenic ectomesenchymal with hard tooth (mixed OT)

Ameloblastic fibroma Ameloblastic fibro-odontoma Ameloblastic fibrosarcoma Odonto ameloblastoma Compound odontoma Complex odontoma

Clear cell odontogenic carcinoma Adenomatoid OT Calcifying epithelial OT Squamous OT

Malignant ameloblastoma Ameloblastic carcinoma

tumors of odontogenic ectomesenchymal alone

Odontogenic fibroma Myxoma Cementoblastoma Granular cell odontogenic fibroma

Biologic Classification of Odontogenic Tumors*

BENIGN, NO RECURRENCE POTENTIAL Adenomatoid odontogenic tumor Squamous odontogenic tumor Cementoblastoma Periapical cementoosseous dysplasia Odontoma BENIGN, AGGRESSIVE Ameloblastoma Clear cell odontogenic tumor (some consider this a carcinoma) Odontogenic ghost cell tumor (COC, solid type) Odontogenic myxoma Odontoameloblastoma

MALIGNANT Malignant ameloblastoma Ameloblastic carcinoma Primary intraosseous carcinoma Odontogenic ghost cell carcinoma Ameloblastic fibrosarcoma
BENIGN, SOME RECURRENCE POTENTIAL Cystic ameloblastoma (unicystic) Calcifying epithelial odontogenic tumor Central odontogenic fibroma Florid cementoosseous dysplasia Ameloblastic fibroma and fibroodontoma *From Regezi, et al.

Ameloblastoma
!Most common odontogenic tumor !Benign, but locally invasive

and histologically similar to BCCa !4th and 5th decades !Occasionally arise from dentigerous cysts !Subtypes - multicystic (86%), unicystic (13%), and peripheral (extraosseous - 1%)

!Clinically

Are the most common neoplasms of the jaws. They may arise from Rest of dental lamina Developing enamel organ Epithelial lining of OC The basal cell of the oral mucosa ameloblastoma are slow-growing, locally invasive, produce bone expansion and facial deformity, had high recurrence rate, no metastasis, it present in 3 type. (They occur in 3 different clinico-radiographic situations which deserve separate consideration because of different therapeutic considerations and prognosis)

Ameloblastoma
!Radiographic
!Classic

findings

- multilocular radiolucency of posterior mandible

!Well-circumscribed,

soap-bubble !Unilocular - often confused with odontogenic cysts

!Root

resorption - associated with malignancy

Ameloblastoma

1-common polycystic ameloblastoma (Conventional solid or multicystic interoasseous ameloblastoma) It's most common type, occur in patients over 25y (20-40y) it is rare in children, there is no sex predilection. About 80%-85% of conventional ameloblastoma occur in the mandible and ascending rams. The tumor is often asymptomatic, a small lesion are detected during a radiographic examination. If untreated the lesion grow slowly to massive producing facial deformity when palpated produce easily bone cracks due to thin outer shell of expand bone (egg shell cracking, diagnostic sign).

Histopathology: several microscopic subtypes of conventional ameloblastoma are recognized: The classical feature is the ameloblast- like cells which are palisaded columnar basal cells, with reverse polarization (nucleous away from basement membrane). It may be arranged in the following forms: Follicular: It is the most common and resembles early stage of tooth development. It consisted of island of odontogenic epithelium resemble enamel organ in a mature fibrous C.T. stroma. These islauds consist of outer border of palisaded ameloblast-like cell and a central loosely arranged angular cells resembling the stellate reticulum. The central area may undergo degeneration and form microcystic changes or some tissue they transformed to sq. cells and produce keratin and referred as Acanthomatous pattern. If these cells are swollen and densely packed with eonsinophilic granules, it referred as granular cell, variant.

Large epithelial areas of loosely structured spindle epithelium enclosing liquefacting stromal areas are typical of a plexiform ameloblastoma. The epithelial cells facing the stroma show palisading

Ameloblastoma

In cases of follicular ameloblastoma, the tumour consists of epithelial islands with a loose oedematous centre and a peripheral rim of palisading cells. Liquefaction of their centre results in cyst formation

2- Plexiform pattern: It consist of anastomosing cords or large sheets of odontogenic epithelium in a lossely arranged and vascular supporting stroma. 3- Acanthamatous pattern 4- Granular cell pattern: Represent aging or degenerative change in long standing lesion , seen in young patient and in clinically aggressive tumors. 5- Desmoplastic pattern Contain small islands and cord of odontogenic epithelium in a densely collagenized stroma. IHC studies have shown increased production of the cytokeratin which know as transforming growth factor-B (TGF-B) in association with this lesion which may be responsible for desmoplasia. 6- Basal cell pattern It is the least common type , composed of nests of uniform basaloid cells which histologically are very similar to basal cell carcinoma of the skin , no stellate reticulum is present.

Desmoplastic ameloblastoma consists of densely packed spindle cells lying in a fibrous stroma. Palisading of peripheral cells is not a conspicuous feature in this type of ameloblastoma

Treatment of Ameloblastoma
!

According to growth characteristics and type ! Unicystic

!

Complete removal ! Peripheral ostectomies if extension through cyst wall

!

Classic infiltrative (aggressive)

Mandibular - adequate normal bone around margins of resection ! Maxillary - more aggressive surgery, 1.5 cm margins

! Ameloblastic
!

carcinoma
!

Radical surgical resection (like SCCa)

Treatment: It never depends on histopathological pattern, all types are locally invasive therefore resistant.extensive surgical removal ( block resection ) is recommended . The lesion is radio resistant.

Unicystic amelblastoma It is a new entity: it account for 10% to 46% of all intraosseous ameloblastoma . It occurs at young patients 16-20 y as a symptomatic painless swelling of the jaw , more than 90% are found in the mandible usually in the posterior regions . X-Ray : typically appears as unilocular RL,well demarcated, associated with unerupted 3rd molar.

Histopathology:

Three histopathological variant of unicystic ameloblastoma, have been described:

Luminal unicystic ameloblastoma. The tumor is confined to the luminal surface of the cyst, it consist of fibrous cyst wall with a lining that consist of ameloblastic epithelium, the over lying epithelial cell are loosely cohesive and resemble stellate reticulum. Intra luminal unicystic Ameloblastoma. When one or more nodules of ameloblastoma projects from the cystic lining in to the lumen of the cyst. Mural unicystic ameloblastoma. There will be infiltration of the fibrous wall by typical follicular or plexiform ameloblastoma.

unicystic ameloblastoma, the tumour consists of cyst-lining epithelium that still shows the typical features of ameloblastoma: loose epithelium and a rim of palisading cells facing the stroma

Treatment: enucleation for intraluminal plexiform pattern however mural compound beneath bone required marginal resection and periodic follow up to ensure adequate removal.

Peripheral Ameloblastoma: Its uncommon, it accounts for about 1%-10% appear as painless firm sessile, nodule on the gingiva which is small in size but continuously growing and has normal color. Most lesions are clinically considered to represent a fibroma or pyogenic granuloma.

X-Ray: Seldom present, but occasionally superficial saucerization of cortical plate. Sometimes tooth separation will show if located in the interdental papilla. Histopathology: Resemble follicular pattern or plexiform pattern. Treatment: Local surgical excision with small normal bone margin.

Malignant ameloblastoma and ameloblastic carcinoma It is a rare type, account 1% of all ameloblastoma. It produce metastasis that not appear until 10y after treatment of primary tumor. X-ray: it had ill defined margines with cortical bone destruction due to aggressive behavior. Histopathology: The malignant ameloblastoma at primary and metastatic site are similar to benign local course ameloblastoma, while ameloblastoma carcinoma has anaplasia and dysplasia of malignancy and necrosis. Treatment: surgical removal , has poor prognosis.

Ameloblastic carcinoma combines the presence of epithelial nests with peripheral palisading and cytonuclear atypia

Calcifying odontogenic tumor (Pindborg tumour) Its rare, account 1% of all odontogenic tumor, its locally invasive, not capsulated arise from remnants of dental lamina. Its most often present in patients between 30-50 years of age, there is no sex predilection mostly occur in mandible in posterior areas a painless, slow growing swelling is the most common presenting features.

X-Ray: unilocualr or multilocular RL with ill defined borders. The lesion usually contains calcified structures of varying size and density and frequently associated with an impacted tooth, (mandibular molar).

Calcifying Epithelial Odontogenic Tumor

Differential Diagnosis : DC, Adenomatoied OT, amelofibroodontoma,

a few cases of peripheral (extraosseous) calcifying epithelial OT have been reported which appear as sessile gingival mass most often on the anterior gingiva and show superficial cortical erosion radiographically.

Calcifying Epithelial Odontogenic Tumor

Histopathology:
It consists of sheets of polyhedral cell with prominent intracellular bridges, pleomorphism, multinucleated prominent nucleoli accosionally hyperchromatism, rare mitosis. There is a large areas of apple-green birefringenic amorphous, eosinphilic hyalinized (amyloid-like) extracellular material scattered through out the epithelium and C.T. calcification within the amyloid-like material and form concentric rings. (liesengang ring calcification). The peripheral type has less tendency for calcification and prominent clear cell cytoplasm variant.

Nuclear atypia, ample cytoplasm and pronounced intercellular bridging are typical of a calcifying epithelial odontogenic tumor

Extracellular material staining for amyloid with congo red is another characteristic feature of a calcifying epithelial odontogenic tumour

Treatment: It is less aggressive than ameloblastoma so local resection including soft tissue and normal bone surrounding margins.

Adenomatoid Odontogenic Tumor

! !

Associated with the crown of an impacted anterior tooth

Painless expansion ! Radiographic findings

Well-defined expansile radiolucency ! Root divergence, calcified flecks (target) !
!

Histology
!

Thick fibrous capsule, clusters of spindle cells, columnar cells (rosettes, ductal) throughout

Treatment - enucleation, recurrence is rare

Adenomatoid Odontogenic Tumor

Calcifying odontogenic cyst (COC)

It is uncommon lesion may appears as cyst, solid pattern and associated with odontoma. Clinical :
Occurs at any part of teeth bearing area, commonly in anterior region to molars, at any age predilection 2nd decade , may be intraosseous causing generalized expantion of buccal and lingual cortices, or extra osseous appears as localized swelling. There is no pain. X-ray:- Well circumscribed unilocular RL containing RO and tooth like structure . Differential diagnosis: odontoma, ameloblastic fibro-odontoma. Histopathology: It has varying pictures

Cystic and solid , containing epithelial cells like ameloblastoma , outer layer of palisaded columnar basal cells an dinner layer like stellate reticulum , in between there is enlarged eosinophilic epithelial cells lack nuclei ( ghost cells) and calcification are scattered within the epithelial and C.T.
Treatment: enucleation , recurrence are uncommon.

Calcifying odontogenic cyst closely resembles ameloblastoma, but the presence of large intraepithelial aggregates of ghost cells rules out the latter diagnosis

Histopathology: It has varying pictures Cystic and solid , containing epithelial cells like ameloblastoma , outer layer of palisaded columnar basal cells and inner layer like stellate reticulum , in between there is enlarged eosinophilic epithelial cells lack nuclei ( ghost cells) and calcification are scattered within the epithelial and C.T.

Squamous OT
Its rare, benign tumor with aggressive potential. It arises from remnant of dental lamina, rest of malasses and overlying epithelium. Clinical: Sq. OT has been found in patient with average age 38y. Occur in anterior region to molar, in max and mandible, as painless gingival swelling associated with mobility of teeth and its slowly growing. X-Ray: Unilocular RL lateral to roots which result in teeth separation but not root resorption.

Squamous Odontogenic Tumor

!

Hamartomatous proliferation ! Maxillary incisor-canine and mandibular molar ! Tooth mobility common complaint ! Radiology - triangular, localized radiolucency between contiguous teeth ! Histology - oval nest of squamous epithelium in mature collagen stroma ! Treatment - extraction of involved tooth and thorough curettage; maxillary - more extensive resection; recurrences treat with aggressive resection

Histopathology: Microscopically the tumor consist of varying shaped (round and elongated) islands of stratified sq. epith. in mature fibrous C.T. stroma, no polarization in epith. Microcystic vaculation and individual cell keratinized is common. Treatment: local excision or curettage.

Squamous Odontogenic Tumor

Clear cell odontogenic tumor or carcinoma,

it is a rare jaw tumor that was first described in 1985. It is a malignant neoplasm, clear cytoplasm indicate glycogen rich presecretory ameloblast cells. Clinically: It occurs in patient older than age 50y. 80% develop in mandible as painful swelling or sometime symptom free. Radiographically: uniloccular or multiloccular RL with irregular margins.

Histopathology: Three patterns have been described: The biphasic pattern appears as nests of varying size of clear cell or faint eosinophilic cytoplasm admixed with more eosinophilic polygonal epithelium cell. monophasic type: characterized by appearance of cord or nests of clear cell in a fibrous C.T. stroma. The third pattern has a resemblance to amelobastoma in that the peripherial cells of the clear cell islands may infrequently demonstrate palisading and the lesional cells dont show nuclear or cytoplasmic pleomorphism, mitosis are rare and necrosis is not a prominent feature.

Clear cell odontogenic carcinoma is characterised by clear cells forming epithelial nests

At higher magnification, clear cell odontogenic carcinoma is seen to contain clear cells as well as eosinophilic cells

Differential diagnosis Intraosseous mucoepidemoid carcinoma (+ve mucin stain) with a prominent clear cell component. COT with clear cell (amyloid stain +ve in COT and ve in Odontogenic carcinoma) Metastatic clear cell neoplasm (renal cell carcinoma, clear cell breast carcinoma). Treatment: it has aggressive clinical course need surgical radical resection, metastasis to lung and L.N.

Mesenchymal Odontogenic Tumors

!Odontogenic Myxoma !Cementoblastoma

Odontogenic Myxoma
!Originates

from dental papilla or follicular mesenchyme !Slow growing, aggressively invasive !Multilocular, expansile; impactedteeth? !Radiology - radiolucency with septae !Histology - spindle/stellate fibroblasts with basophilic ground substance !Treatment - en bloc resection, curettage may be attempted if fibrotic

Odontogenic myxoma is composed of poorly cellular myxoid material that surrounds pre-existing jaw bone

Cementoblastoma
True neoplasm of cementoblasts ! First mandibular molars ! Cortex expanded without pain ! Involved tooth ankylosed, percussion ! Radiology apical mass; lucent or solid, radiolucent halo with dense lesions ! Histology - radially oriented trabeculaefrom cementum, rim of osteoblasts ! Treatment - complete excision and tooth sacrifice
!

Cementoblastoma

Mixed Odontogenic Tumors

!Ameloblastic

fibroma, ameloblastic fibrodentinoma, ameloblastic fibroodontoma, odontoma !Both epithelial and mesenchymal cells !Mimic differentiation of developing tooth !Treatment - enucleation, thorough curettage with extraction of impacted tooth !Ameloblastic fibrosarcomas - malignant, treat with aggressive en bloc resection

Ameloblastic Fibro-odontoma
This lesion is defined as a tumor with general features of an ameloblastic fibroma but containing enamel and dentin. Some investigators believe that this entity is but a stage in the development of an odontoma; however, most agree that progressive destructive tumors are true neoplasms.

Ameloblastic Fibro-odontoma: Clinical and Radiographic Features

Patient Age: Most common in the 5-12 year age range with a mean of 10 years. Gender Predilection: None. Location: It is more common in the premolar/molar regions of both jaws. Radiographic Features: Usually appears as a well-defined unilocular or rarely multilocular radiolucency with variable amounts of calcified material which is radiopaque. Therefore, it may appear as a mixed, radiolucent-radiopaque lesion.

Ameloblastic Fibro-odontoma: Histologic Features

The soft tissue component is identical to the ameloblastic fibroma. The calcified portion consists of foci of enamel and dentin matrix formation in close relationship to the epithelial structures.

Ameloblastic Fibro-odontoma: Treatment and Prognosis

The ameloblastic fibro-odontoma is usually treated by conservative curettage with the lesion separating easily from the surrounding bone. Prognosis is excellent and recurrence is unusual.

Ameloblastic Fibrosarcoma
This lesion is considered the malignant counterpart of the ameloblastic fibroma in which the mesenchymal portion shows features of malignancy. The ameloblastic fibrosarcoma may arise de novo or there may be a malignant transformation of an ameloblastic fibroma.

Ameloblastic Fibrosarcoma: Clinical and Radiographic Features

Patient Age: The mean age for patients with ameloblastic fibrosarcoma is 26 years. Gender Predilection: Males 2:1. Location: 75 % have occurred in the mandible. Radiographic Features: Appear as an ill-defined, destructive, radiolucent lesion.

Ameloblastic Fibrosarcoma: Histologic Features

The epithelial component of this tumor appears histologically benign. The mesenchymal portion is highly cellular. The cells are hyperchromatic and quite pleomorphic. Mitoses are usually prominent.

Pain and swelling are typically associated with this tumor. Rapid clinical growth is another common feature. Radical surgical excision is the treatment of choice. The long-term prognosis is difficult to ascertain because of the small number of reported cases.

Ameloblastic Fibrosarcoma: Additional Features, Treatment & Prognosis

Odontoma
The odontoma is the most common odontogenic tumor. It is not a true neoplasm but rather is considered to be a developmental anomaly (hamartoma). Two types of odontomas are recognized:
Compound: this type of odontoma is composed of multiple small tooth-like structures. Complex: this lesion is composed of a conglomerate mass of enamel and dentin, which bears no anatomic resemblance to a

Odontoma: Clinical Features

Patient Age: Most cases are recognized during the second decade of life with a mean of 14 years. Gender Predilection: Approximately equal. Location: Somewhat more common in the maxilla. The compound type is more often in the anterior maxilla while the complex type occurs more often in the posterior regions of either jaw.

Odontoma: Radiographic Features

Early lesions are radiolucent with smooth, well-defined contours. Later a well-defined radiopaque appearance develops. The compound type shows apparent tooth shapes while the complex type appears as a uniform opaque mass with no apparent tooth shapes present.

Odontoma: Additional Features

Most odontomas are small and do not exceed the size of a normal tooth in the region. However, large ones do occur and these may cause expansion of the jaw. Most odontomas are asymptomatic and as a result are discovered upon routine radiographic examination. Odontomas may block the eruption of a permanent tooth and in these cases are often discovered when searching for the

Odontoma: Histologic Features

The compound odontoma is composed of enamel, dentin and cementum arrange in recognizable tooth forms; some enamel matrix may be retained in immature and hypomineralized specimens. The complex odontoma is composed of enamel, dentin and cementum but these tissues are arranged in a random manner that bears no morphological resemblance to a

Odontoma: Treatment and Prognosis

Odontomas are treated by simple local excision and the prognosis is excellent.

Tumors of Odontogenic Ectomesenchyme

(Central) Odontogenic Fibroma: Clinical Features

Fewer than 70 cases have been reported in the English literature. (VS hyperplastic follicle when associated with unerupted tooth) Patient Age: Patients have ranged in age from 9-80 years old with a mean of 40 years. Gender Predilection: Females, 7.4:1 in one study. Location: Sixty percent occur in the maxilla where most are located anterior to the first molar. When in the mandible, approximately 50 % occur in the posterior

Odontogenic Fibroma: Radiographic Appearance

The odontogenic fibroma usually appears as a well-defined, unilocular radiolucency. It is often associated with the apical area of an erupted tooth. Larger lesions are often multilocular. Many odontogenic fibromas have sclerotic borders. Root resorption is common.

Odontogenic Fibroma: Additional Features

Small odontogenic fibromas are usually asymptomatic. The larger lesions may be associated with localized bony expansion of the jaw or with the loosening of adjacent teeth.

Odontogenic Fibroma: Histologic Features

Some authors have described two separate types of odontogenic fibromas. The simple odontogenic fibroma is composed of stellate fibroblasts arranged in a whorled pattern with fine collagen fibrils and a lot of ground substance. Foci of odontogenic epithelium may or may not be present. Occasionally, foci of dystrophic calcification may be present.

Odontogenic Fibroma: Histologic Features

The WHO type odontogenic fibroma appears as a fairly cellular fibrous connective tissue with collagen fibers arranged in interlacing bundles. Odontogenic epithelium in the form of long strands or isolated nests is present throughout the lesion. Calcifications composed of cementoid and/or dentinoid may be present.

Odontogenic Fibroma: Treatment and Prognosis

The odontogenic fibroma is usually treated by enucleation and curettage. There have been few recurrences, the prognosis is good.

Developmental non-odontogenic cysts The remaining 5% of cysts within the jaws are made up of non-odontogenic cysts. Developmental non-odontogenic cysts within the jaws must arise from epithelium not associated with tooth development. In the past, several entities were described as fissural cysts because they were suspected to have arisen from tissue entrapped during fusion across various fissures during facial development. It has now been clearly shown that the only true fissural cyst is the midpalatal cyst of infants. Other so-called fissural cysts such as the globulomaxillary cyst, the median alveolar cyst, and the median mandibular cyst are most likely to be developmental or inflammatory odontogenic cysts. The other two lesions within this category are the nasopalatine duct cyst, which arises from the cystic degeneration of the vestigule bilateral oro-nasal ducts, and the nasolabial cyst, which is a soft-tissue cyst arising from dystopic rests of the naso-lacrimal ducts.

Nonodontogenic Cysts
!Incisive

Canal Cyst

!Stafne Bone Cyst !Traumatic Bone Cyst

!Surgical

Ciliated Cyst (of Maxilla)

Nasopalatine duct cyst Midpalatal cyst of infants Nasolabial cyst Globulomaxillary cyst dermoid cyst

Nonepithelial. Idiopathic bone cavity (traumatic, solitary, hemorrhagic bone cyst) b. Aneurysmal bone cyst c. Stafne's mandibular lingual cortical defect

Sublingual dermoid cyst. This is an unusually large specimen but appears even larger because the patient is raising and protruding her tongue. This cyst, unlike a ranula, can be seen to have a thick wall because it has arisen in the deeper tissues of the floor of the mouth

Nasopalatine duct cyst The nasopalatine duct cyst, also known as the incisive canal cyst is formed from cystic degeneration of the oronasal ducts that connect the nasal cavity to the oral cavity during development.

Incisive Canal Cyst

from epithelial remnants of the nasopalatine duct (incisive canal) !4th to 6th decades !Palatal swelling common, asymptomatic !Radiographic findings
!Well-delineated

!Derived

oval radiolucency between maxillary incisors, root resorption occasional lined by stratified squamous or respiratory epithelium or both

!Histology
!Cyst

Incisive Canal Cyst

Nasopalatine cyst. The usual appearance is a rounded or pearshaped area of radiolucency, mainly in the midline.

Incisive Canal Cyst

!Treatment

consists of surgical enucleation or periodic radiographs !Progressive enlargement requires surgical intervention

Nasopalatine cyst. Typical presentation with a dome-shaped bluish enlargement overlying the incisive canal

Nasopalatine cyst. The lining, in part at least, may consist of respiratory (ciliated columnar) epithelium,

Globulomaxillary Cyst Cyst of the globulomaxillary area

Stafne Bone Cyst

!Submandibular

salivary gland depression !Incidental finding, not a true cyst !Radiographs - small, circular, corticated radiolucency below mandibularcanal !Histology - normal salivary tissue !Treatment - routine follow up

Stafne's mandibular lingual cortical defect This entity is also known by several other terms including lingual mandibula bone cavity, static bone cavity, and lingual salivary gland defect. This is an asymptomatic lesion. It usually occurs in adults over the age of 25. Radiographically, the lesion is a solitary radiolucency below the inferior alveolar canal near the angle region of the mandible. The lesion is usually oval and exhibits no growth over long periods of time. The periphery is smooth and symmetric. The lesion is benign, has not growth potential, and a classic radiographic appearance that makes diagnosis without biopsy possible and observation without active intervention the treatment of choice.

Stafne Bone Cyst

Traumatic Bone Cyst

or fluid filled cavity associated with jaw trauma (50%) !Radiographic findings
!Radiolucency,

!Empty

most commonly in body or anterior portion of mandible

!Histology

- thin membrane of fibrous granulation !Treatment - exploratory surgery may expedite healing

Traumatic Bone Cyst

Surgical Ciliated Cyst

!May

occur following Caldwell-Luc !Trapped fragments of sinus epithelium that undergo benign proliferation !Radiographic findings
!Unilocular

radiolucency in maxilla
!Histology

!Lining

of pseudostratified columnar ciliated !Treatment - enucleation

Surgical Ciliated Cyst

Aneurysmal Bone Cyst !Large vascular sinusoids (no bruit) !Not a true cyst; aggressive, reactive !Great potential for growth, deformity !Multilocular radiolucency with cortical expansion !Mandible body !Simple enucleation, rare recurrence

Aneurysmal bone cyst The aneurysmal bone cyst is not unique to the maxillofacial complex. It is a lesion that has a predilection for females and occurs most commonly in the vertebral column and the long bones. In the craniofacial skeleton, the mandible is affected most often. The patient often complains of swelling over a localized area and pain. It is felt that this lesion may develop from some other primary lesion such as the central giant-cell granuloma that undergoes alteration in local hemodynamics. Radiographically, the lesion is a radiolucency with a multilocular or honeycombed appearance. The sclerotic bone margins seen in most cysts may be absent. Histologically, the lesion is made up of a fibrous connective tissue stroma with multiple cavernous and sinusoidal spaces with multinucleated giant cells within the stroma.

Enucleation is the treatment of choice with hemorrhage during the removal being risk because of the large number of vascular spaces within the lesion. Hemorrhage is usually controlled easily once the entire lesion is removed; when treating these lesions, the goal should be rapid enucleation to prevent blood loss with control of bony bleeding with bone wax or packing. The lesion does not recur after simple enucleation.