GITAM DENTAL COLLEGE & HOSPITAL

DEPARTMENT OF

ORAL & MAXILLOFACIAL SURGERY

SEMINAR ON

Odontogenic tumours- current concepts

and its treatment modalities.

Presented By:

Dr. Sambhav K Vora

III MDS

CONTENTS
INTRODUCTION.
CLASSIFICATION.
ODONTOGENESIS.
GENERAL PRINCIPLES OF MANAGEMENT.
PRINCIPLES OF SURGICAL MANAGEMENT OF JAW TUMOURS.
AMELOBLASTOMA.
CALCIFYING EPITHELIAL ODONTOGENIC TUMOUR.
ADENOMATOID ODONTOGENIC TUMOUR.
SQUAMOUS ODONTOGENIC TUMOUR.
ODONTOGENIC FIBROMA.
GRANULAR CELL ODONTOGENIC TUMOUR.
MYXOMA.
AMELOBLASTIC FIBROMA.
AMELOBLASTIC FIBRO-ODONTOMA.
AMELOBLASTIC FIBRO-DENTINOMA.
ODONTOAMELOBLASTOMA.
CALCIFYING ODONTOGENIC CYST.
BENIGN CEMENTOBLASTOMA.
ODONTOGENIC MALIGNANCIES.
CONCLUSION.
REFERENCES.

INTRODUCTION: The embryologic events that initiate control and guide the formation
of human odontogenic structures take place through a complex and finely
regulated series of inductive interactions between epithelium and
mesenchyme, which begin during 5th and 6th weeks of intrauterine life and
are completed about 16th year after birth. During this extended period, there
is ample opportunity for developmental and growth mechanisms to fail in
whole or in part, resulting in the formation of malformations, hamartomas
and neoplasms.
Odontogenic tumors are an imprecise term that encompasses a
wide spectrum of lesions and variants that are derived from the specialized
dental tissues. These tumors do not always meet the criteria for neoplasia
and some appear to represent abortive attempts at odontogenesis. The
distinctions among the above mentioned 3 forms of abnormalities might in
part depend upon the embryologic stage of initiation and histologic and
gross appearance of the lesion at the age of clinical discovery.
Odontogenic tumors are primarily intraosseous lesions, although
extraosseous variants have been described occasionally. These tumors
have a varied clinical and radiographic appearance and can consist of
entirely soft tissue, a mixture of soft and calcified tissues or entirely

calcified tissues. A unique feature of these tumors is the wide range of

biologic behavior that they express. Because of this wide range of
behavior, various methods of treatment have been described for each
tumor ranging from conservative to aggressive therapy. Often the proper
therapy is selected only after careful consideration has been given to
correlate the specific lesion with its clinical behavior pattern. Here let us
discuss the various treatment modalities used to treat the odontogenic
tumors and the present day concepts regarding the management of the
same.

CLASSIFICATION: I.
A.

GORLIN, CHAUDHRY AND

PINDBORGS CLASSIFICATIONEpithelial odontogenic tumors:
1.
Minimal inductive change in the connective tissue(i)
Ameloblastoma.
(ii)
2.

AOT

(iii)
CEOT.
Marked inductive change in the connective tissue(i)
Ameloblastic fibroma.
(ii)

Ameloblastic fibrosarcoma.

(iii)

Odontoma:

Ameloblastic odontoma.

Ameloblastic dentinosarcoma.

Complex odontoma.

 Compound odontoma.
Mesodermal odontogenic tumors:
1.
Myxoma.
2.
Odontogenic fibroma.
3.
Cementoma-

B.

(i)

Periapical cemental dysplasia

(ii)

Benign Cementoblastoma.

(iii)

Cementifying fibroma.

(iv)

Gigantiform cementomas.

II. REICHART AND RIES

CLASSIFICATIONAmeloblastic
Ameloblastic

Ectomesenchy

Mesenchym

Neuroectoderm

mal

al

al

Ameloblasto
ma.
AOT.
CEOT.

Ectomesenchy

SOT.
AF.

Cementoblasto

mal

AFO.

ma.

Complex

Odontogenic

odontoma.

Myxoma.

Compound

Odontogenic

odontoma.

fibroma.

Adamantino

Dentinoma.
Periapical

Mesenchymal

Fibroma.

cemental

Lipoma.

hemangioma.

dysplasia.

Hemangiom

Gigantiform

a.

cementoma.
Cementifying
fibroma.
Neuroectoder

Ameloblasto

Central

Neurofibro

Melanotic and

mal

ma with

pacinian

ma.

Neuroectoderm

neurinoma.

neurofibroma.

al tumor of
infancy.

I.

WHO CLASSIFICATION (1992)I.

Neoplasms and other tumors related to the odontogenic

apparatusA.
Benign odontogenic tumors:
1) Odontogenic epithelium without odontogenic
ectomesenchyme: (i)
Ameloblastoma.

2)

(ii)

SOT.

(iii)

CEOT.

(iv)
Clear cell odontogenic tumor.
Odontogenic epithelium with odontogenic
ectomesenchyme, with or without dental
hard tissue formation: (i)
Ameloblastic fibroma.
(ii)

AFO and AFD.

3)

(iii)

Odontoameloblastoma.

(iv)

AOT.

(v)

Gorlin lesion.

(vi)

Complex odontoma.

(vii) Compound odontoma.

Odontogenic Ectomesenchymal with or
without included odontogenic epithelium: (i)
Odontogenic fibroma.
(ii)

B.

Myxoma.

(iii)
Benign Cementoblastoma.
Malignant odontogenic tumors:
1)
Odontogenic carcinomas: (i)
Malignant Ameloblastoma.
(ii)

Primary intraosseous carcinoma.

(iii)

Malignant variants of other

odontogenic epithelial tumors.

(iv)
2)

Malignant changes in odontogenic

cysts.
Odontogenic sarcomas: (i)
Ameloblastic fibrosarcoma.
(ii)

Ameloblastic fibro-dentinosarcoma
and Ameloblastic fibroodontosarcoma.

II.

(iii)
Odontogenic carcinosarcoma.
Neoplasms and other lesions related to boneA.
Osteogenic neoplasms: 1)
Cementifying fibroma
B.

(OF, Cementifying fibroma).

Non-neoplastic bone lesions: -

1)
2)

Fibrous dysplasia of the jaws.

Cemento-osseous dysplasias(i)

Florid cemento-osseous dysplasia.

(Gigantiform cementoma, familial
multiple cementomas).

(ii)

Other cemento-osseous dysplasias.

3)
4)
5)
6)
C.

Cherubism.
Central giant cell granuloma (CGCG).
Aneurysmal bone cyst.
Solitary bone cyst.
Other tumors: 1)
Melanotic neuroectodermal tumors of
infancy.

III. REVISED VERSION OF WHO

CLASSIFICATION (2002)I.

Neoplasms and tumor-like lesions arising from the

odontogenic apparatusA]
Benign: 1) Odontogenic epithelium with mature, fibrous
stroma; odontogenic ectomesenchyme not
present:
(i)
Ameloblastomas.
Intraosseous (central,
infiltrative).
Extraosseous (peripheral).
Desmoplastic.

 Unicystic.

2)

(ii)

SOT.

(iii)

CEOT.

(iv)
AOT.
Odontogenic epithelium with odontogenic
ectomesenchyme, with or without dental
hard tissue formation:
(i)
AF and AFD (neoplastic).
(ii)

AFD (non-neoplastic).
AFO.
Complex odontoma.

3)

(iii)

Compound odontoma.

(iv)

Odontoameloblastoma.

(v)

Calcifying ghost cell odontogenic

tumor.
Mesenchyme and /or odontogenic
ectomesenchyme with or without included
odontogenic epithelium:
(i)
Odontogenic fibroma (simple).

B]

(ii)

Odontogenic fibroma (WHO-type).

(iii)

Odontogenic Myxoma.

(iv)
Benign Cementoblastoma.
Malignant (odontogenic carcinomas): 1)
Malignant (metastasizing) Ameloblastoma.
2)
Ameloblastic carcinoma(i)
Primary.
(ii)

Carcinoma in intraosseous

Ameloblastoma (dedifferentiated).
(iii)
3)

Carcinoma in peripheral

Ameloblastoma (peripheral).
Primary intraosseous squamous cell
carcinoma
(i)
Solid.
(ii)

Cystogenic
Non-keratinizing cyst.

4)
5)

OKC.
Clear cell odontogenic carcinoma.
Calcifying ghost cell odontogenic
carcinoma.
(Malignant epithelial odontogenic ghost cell

C]

II.

tumor).
Malignant (odontogenic sarcomas): 1)
Ameloblastic fibrosarcoma
2)
Ameloblastic fibro-dentino and fibroodontosarcoma.
3)
Odontogenic carcinosarcoma.
Neoplasms and other lesions occurring in the jaw

bonesA)
Osteogenic neoplasms: 1)
Cemento-ossifying fibroma.
B)
Non-neoplastic lesions: 1)
Fibrous dysplasia of the jaws.
2)
Cemento-osseous dysplasia
(i)
3)

Focal cemento-osseous dysplasia.

(ii)
Florid cemento-osseous dysplasia.
Other cemento-osseous dysplasia
(i)

Cherubism.

(ii)

Central giant cell granuloma (CGCG).

4)

Pseudocysts of the jaws

(i)

Aneurysmal bone cavity.

(ii)

Simple bone cavity.

(iii)

Lingual and buccal mandibular bone

depressions.

C]

(iv)
Focal marrow containing jaw cavity.
Other tumors: 1)
Melanotic neuroectodermal tumor of
infancy.

GENERAL PRINCIPLES OF
MANAGEMENT: IMAGINGI.

Plain radiography.

II.

CT scan and 3-D CT scan.

III.

MRI.

IV.

Angiography.

BIOPSYI.

Exfoliative cytology.

II.

Aspiration biopsy.

III.

FNAC.

IV.

Excisional biopsy.

V.

Incisional biopsy.

PRINCIPLES OF SURGICAL
MANAGEMENT OF JAW
TUMOURSGoals of treatmenta. Complete eradication of a lesion
b. Preservation of normal tissue as permissible
c. Excision with least morbidity
d. Restoration of tissue loss, form and function
e. Long-term follow-up for recurrence.

Factors to be evaluated before

surgery(i)

Nature of the lesionBefore choosing the most appropriate surgical method, it is a

must to identify the lesion histologically with biopsy, whether
benign/malignant.

(ii)

Anatomical location of the lesiona. The location of the lesion within the jaws may severely
complicate the surgical excision and therefore jeopardize the
prognosis. A benign tumour in an inaccessible area presents
an obvious problem surgically and from the standpoint of the

function rehabilitation of the patient. A more aggressive

tumour in an easily reachable and resectable area, as in the
anterior mandible, offers a better prognosis
b. Maxillary tumours exhibit much more aggressive behaviour
than their mandibular counterpart. Proximity to maxillary
sinus,

nasal

cavity,

orbital

cavity,

cranial

fossa,

and

nasopharynx allows these tumours to grow asymptomatically

and more extensively to attain a large size with late symptoms.
c. Proximity of the benign tumours to adjacent vital structures
like neurovascular bundles and teeth is an important
consideration because preservation of these structures should
be attempted.

If the tumour is aggressive, then both these

important structures have to be sacrificed.

d. The amount of involvement with in a particular site, mandible,
has bearing on the type of surgical procedure necessary to
obtain a cure with the more aggressive lesions. If the inferior
border is not involved in the lesion, marginal resection can be
carried out without continuity defect.

When the tumour

extends through the entire thickness of the involved jaw, a

partial resection then becomes mandatory.
e. If the tumour is confined to the inferior of the jaw, without
perforating cortices, offers a better prognosis than those that
have invaded surrounding soft tissues. In the latter case, one

has to carry out wider resection sacrificing the more amount

of normal soft tissue along with the resection of the bone.
(iii)

Aggressiveness of the lesionSurgical therapy of the odontogenic tumours ranges from

enucleation / curettage to composite resection.

Histological

diagnosis positively identifies and therefore directs the treatment

of the lesion.

Locally invasive tumours are treated with wider

resection in order to prevent recurrence.

(iv)

Duration of the lesionMany odontogenic tumours exhibit slow growth and may
become static in size. The slower growing lesions seen to follow
a more benign course and treatment should be individually
tailored to each case.

(v)

Functional rehabilitation of the patientAfter achieving the primary goal of eradicating odontogenic
tumours, next most important consideration is the residual
defects resulting from the extirpative surgery. Reconstruction is
much easier in mandible than in maxilla. When reconstructing
defects in the mandible, treatment goals should be established.

Restoration of mandibular continuity-using reconstruction

plates.

Restoration of alveolar bone heights, restore the adequate

bulk of the mandible-primary/secondary reconstruction
method-to prevent pathological fracture.

Gold, Upton and Marx in 1991 have presented a standardized surgical

terminology for the excision of lesions in the bone
(i)

Enucleation

(ii)

Curettage

(iii)

Marsupialization

(iv)

Recontouring

(v)

Resection without continuity defect

(vi)

Resection with continuity defect

(vii)

Disarticulation.

1. Enucleation with/without curettageEnucleation- removal of an entire structure, without rupture, as one

shells kernel of a nut.
Curettage- a scraping, usually of the interior of a cavity/tract, for the
removal of new growths or other abnormal tissues, or to obtain
material for tissue diagnosis using hand instruments.
Eventhough, this is mainly used for the cystic lesions; nonaggressive small benign tumours can also make use of enucleation
with/without curettage.

Indications1) Surgical excision of the tumours, which tend to grow by

expansion, rather than by infiltration of the surrounding
tissues.
2) Lesions occurring in the bone with a distinct separation
between the lesion and the surrounding bone.
3) Often there is a cortical margin of bone that delineates the
tumour or cyst from the bone.
4) Indicated in following tumours:
(i)

Odontogenic tumoursa. Odontoma

b. AF.
c. AFO.
d. AOT.
e. Cementoblastoma

(ii)

(iii)

Fibro-osseous lesions and nonodontogenic tumours

Ossifying fibroma

Cherubism

CGCG.

Osteoblastoma

Other lesions
i. Hemangioma
ii. Eosinophilic granuloma

iii. Neurofibroma
iv. Neurilemanoma
v. Pigmented neurectodermal tumour.

Marginal resection or resection without continuity

Defect / peripheral osteotomy / en block
resection:
Enblock resection- this is a procedure that allows complete excision of the
tumour, at the same time, a continuity of jawbone is retained. And thus
deformity, disfigurement, and the need for secondary cosmetic surgery and
prosthetic rehabilitation are avoided.
Peripheral osteotomy- a procedure in which scraping usually of the interior
of a cavity/tract, for the removal of new growths or other abnormal tissues
are done using rotary instruments.
Indications1. Benign lesions with a known propensity for recurrence.
2. In those lesions that are incompletely encapsulated or tend to
grow beyond their surgically apparent capsule.
3. Recurrent lesions
4. Indicated in the following tumoursa. Ameloblastoma
b. CEOT
c. Myxoma

d. Ameloblastic odontoma
e. Squamous odontogenic tumour,
f. Benign chondroblastoma
g. Haemangioma

Intraoral marginal resection (surgical procedure)

The circumgingival incision is taken and the releasing incisions can
be extended into the buccal mucosa on either side of the lesion leaving at
least one or two adjacent teeth on either side. Using periosteal elevator
reflects the full thickness mucoperiosteal flap.

Care is taken not to

perforate the lesion. If the lesion is perforated, then overlying mucosa

should be sacrificed along with the excision of the tumour. The tooth next
to the tumour mass should be extracted on either side.

The vertical

osteotomies are performed through the sockets of the extracted teeth on

either side using bur or saw blade.
Both the vertical osteotomy cuts are extended from the buccal to
lingual cortex. These cuts are joined with a horizontal cut, placed well
beyond the tumour mass including margin of the normal bone. Horizontal
cut is also completed through and through the buccal and lingual cortex.
Using osteotome carries out the complete excision of the tumour along
with some amount of normal bone. Teeth involved in the tumour mass are
removed with the block in toto. The remaining bony margins should be
checked for sharp areas and contoured by using round bur or bone file.
The excess mucoperiosteal flap is trimmed off and approximated to suture

the wound. This type of excision can be done under local anaesthesia with
sedation for smaller lesions or it can be planned under general
anaesthesia.

Extraoral peripheral osteotomySince complete resection of large segments involving both the
horizontal and the ascending rami or disarticulation of the segment causes
considerable amount of disability, this method is useful, which will allow
complete removal of the pathology, but is less debilitating than complete
resection.

The procedure is based on the observation that the cortical

inferior border of the horizontal body, the posterior border of the ascending
ramus and the condyle are not generally involved in the benign tumour
process. Bone regeneration will start from such areas even though a thin
rim of bone is preserved, especially in young patients result in
considerable restoration of the jaw anatomy.
IndicationsLarge lesions involving posterior mandible.
Operative procedureIn dentulous patients, intraoral crevicular gingival incision is taken
around dentulous area, both buccally as well as lingually. Buccinator and
mylohyoid muscles are separated after reflecting the mucoperiosteal flap.
In edentulous patients there is no need to take intraoral incision.
Extraoral submandibular incision is taken and the inferior border of
the mandible is exposed, if required posterior border of the ramus is also

exposed. The site of peripheral osteotomy is already decided by studying

CT scans or radiographs. The drill holes are made with the electrical dental
drill in the healthy bone around the periphery of the lesion. These holes
are connected until the tumour mass is separated from the thin span of
bone to be retained. The part to be excised is now separated from the
attached soft tissue on the external surface. The masseter and buccinator
muscles are detached with care, so that not to injure the branches of the
facial nerve. Then the mucoperiosteum of the alveolar process is elevated
and the specimen in turned outward to get access to the inner surface to
detach the mylohyoid and the internal pterygoid muscles. The temporalis
muscle is severed above the coronoid, after which the inferior alveolar
nerve and vessels are identified and grasped with a hemostat, ligated and
cut off. The remaining bone is now carefully inspected. Intraoral wound is
closed with watertight suturing. An immediate bone graft can be inserted
through the extraoral wound and fixed with intraosseous wires or bone
plates. The wound is closed in layers with a small rubber drain inserted to
prevent formation of a hematoma.

An external pressure bandage is

applied. Careful long-term following up is mandatory.

Segmental resection of the jawIt is carried out for more extensive lesions, which include the inferior
border of the mandible.
Partial resection-

A procedure wherein resection of a tumour by removing

the full thickness portion of the jaw is carried out. In mandible, this can

vary, depending on the site of the tumour from a small continuity defect to
hemi-mandibulectomy.
Disarticulation- Whenever condylar head is included in the resection of the
part of the mandible, then a procedure is called as hemi-mandibulectomy
with disarticulation.
Whenever the condylar head is retained for rehabilitation procedure
then the procedure is called hemi-mandibulectomy without disarticulation.
Total resection- Excision of a tumour by removal of the involved bone is
carried out. Maxillectomy or mandibulectomy procedures can be carried
out.
Composite resection- Requires much more radical intervention with wider
margins of excision of uninvolved tissues. Surgery may include along with
resection of the jaw, neck dissection. Radiotherapy or chemotherapy alone
or in combination with surgery may be used.

This is usually used for

malignancies.
Indications1. For treatment of lesions that are infiltrative or
have a tendency to recur.
2. Those lesions, which extend close to the inferior
or posterior border of the mandible, the maxillary
sinus or the nasal cavity.
3. It is also used for malignant lesions with huge
recurrence potential.

4. Recurrent odontogenic tumours with difficulty in

examining and gaining an access surgically.
5. Maxillary ameloblastomas with high recurrence
rate.
6. Lesions close to the borders of the jaw, with the
possibility of postoperative pathologic fracture.

Resection of mandibleIntraoral approachAdvantagesa. Excision of overlying mucosal tissue involved in the lesion is
facilitated.
b. Easy access for application of arch bars, extraction of
involved teeth, ligation of the inferior alveolar neurovascular
bundle, closure of the maxillary sinus and maintaining the
forward stability of the tongue, whenever required.
c. An external scar is avoided.
d. An extraoral tissues are preserved for a later reconstruction
procedure
Disadvantagesa. Contamination of the surgical wound by the oral flora.
b. Lack of dependent drainage of the dead space.
c. Difficult access to the most posterior portions of the mandible.

d. The risk of damage to the branches of the internal maxillary

artery, deep in the wound.
e. Immediate bone graft reconstruction by oral route has a higher
rate of infection and loss of the bone graft (30%).
Surgical procedureFor mandibular lesion extending posterior to the mental foramen,
ligation of the inferior alveolar neurovascular bundle prior to resection may
prevent significant intraoperative hemorrhage. The incision is designed to
expose the lesion on both the facial and lingual aspect with or without
inclusion of overlying tissues in the specimen.

The same incision is

extended posteriorly through the buccal mucosa approximately 7 mm

lateral and parallel to the anterior border of the ascending ramus and
external oblique ridge. Blunt dissection is carried out along the surface of
the buccinator muscle to expose the periosteum and periosteum is
elevated lingually to expose the retromolar area of the mandible, internal
oblique ridge and medial surface of the ramus, till the lingula. The inferior
alveolar neurovascular bundle is located and a curved aneurysmal needle
can be passed around it with a ligature threaded through its eye.

The

neurovascular bundle is ligated at the higher level above the lingula.

After

this,

the

entire

lesion

is

exposed

by

reflecting

the

mucoperiosteal flap on buccal and lingual side till the inferior margin of the
mandible.

The anterior and posterior bony cuts are then outlined as

planned and the cuts are finished by using bur or saw blade from buccal to

lingual cortex. In the tooth bearing area, one or two teeth may be removed
prior to sectioning the jaw, bony cut can be carried out through an empty
socket. Separation of the bony cuts can be completed with an osteotome.
The specimen is separated from its bony and soft tissue attachments and
inferior alveolar neurovascular bundle is sectioned below the ligature level.
Whenever the anterior portion of the mandible is removed, it is mandatory
to hold the genioglossus muscles with the hemostat prior to separation
from the specimen and then these muscles should be secured with a
suture in the forward position to the soft tissues of the chin or to the
reconstruction plate to prevent airway obstruction due to tongue fall
postoperatively.

Primary closure of the wound is done.

Intermaxillary

fixation or reconstruction plate is necessary to preserve the alignment of

the fragments.

Extraoral approach1) Segmental resection with disarticulation:

The surgical approach is through a combined postramal (Hinds) and
submandibular (Risdon) incision placed at least 2cm below and
parallel to the inferior and posterior borders of the mandible. After
the incision through the skin, subcutaneous tissue and platysma is
taken, the dissection plane is made deep to the platysma, along the
investing fascia to the inferior border of the mandible. The
pterygomasseteric sling and periosteal layer is divided at the inferior
border of the mandible. Subperiosteal reflection of the masseter

muscle is done along the lateral surface of the mandible to expose

the coronoid process, sigmoid notch and condylar neck. The anterior
portion of the resection is determined and dissected. Care should be
taken to prevent an intraoral communication, using the bur or saw
blade, the osteotomy is completed. By swinging the specimen
laterally, it is freed from its medial attachments subperiosteally. The
temporalis muscle is detached from the coronoid process. The
condylar

head

is

separated

from

lateral

pterygoid

muscle

attachments. Care should be taken to prevent damage to the

maxillary artery and its branches. The specimen is completely
removed and the surgical wound is irrigated. The proximal portion of
the segment is smoothened to prevent intraoral perforation.
Reconstruction plate with condylar prosthesis is placed and fixed on
to the proximal stump with screws. The wound is closed in layers
after proper hemostasis.
2) Segmental resection involving the mandibular midline:
An incision of at least 2cm is made below the inferior border of the
mandible. Incision is taken through the skin, subcutaneous tissue. At
the level of platysma muscle the tissues are undermined. Platysma is
cut sharply and dissection is followed to the investing layer of the
deep cervical fascia. Incision through this layer is taken till the
periosteum at the inferior border of the mandible. The dissection is
carried out subperiosteally to detach the left and right digastric as

well as the mylohyoid muscle from the medial aspect of the

mandible. The genioglossus and geniohyoid muscle attachments are
located and a suture is passed around them. Then the muscles are
detached from the genial tubercles. The subperiosteal dissection is
carried on the buccal surface of the mandible to expose the planned
resection portion. The mental nerves are identified and protected.
The lateral extent of the resection is defined in the dentulous jaw by
extracting the teeth and placing the bony cuts through the sockets. A
mucoperiosteal flap is developed intraorally to free the specimen
from the soft tissue attachments. Prior to the removal of the
specimen, the remaining segments should be stabilized by carrying
out temporary intermaxillary fixation. The reconstruction plate is
contoured prior to excision of the bone and immediately secured it
following the resection by means of screws. This prevents the
collapse of the segments and allows jaw function during healing and
prior to reconstruction and also prevents scar contraction. The oral
mucosa is closed intraorally, using a horizontal mattress suture to
obtain as watertight seal as possible. The genioglossus and
geniohyoid muscles are pulled forward with suture, which is secured
to the reconstruction plate. Extraoral wound is closed in layers.

MaxillectomyAccess to the maxilla is generally obtained by designing the classic

Weber- Fergusson incision. The eyelids are closed temporarily by taking

tarsorrhaphy sutures. For esthetic good results, it is recommended to

tattoo the vermilion border and other points on both sides of the incision
with methylene blue. These points are then matched during closure. The
typical incision splits the midline of the upper lip. But better cosmetic
results can be obtained by incising the philtral ridges and then offsetting
the incision at vermilion border. The incision is turned laterally at the base
of the columella, then around the alar base and along the side of the nose
to within 2mm of the medial canthus. Intraorally the incision is continued
down through the gingival margin. It is connected with a horizontal incision
at the depth of the labiobuccal vestibule, extending back to the maxillary
tuberosity. From here the incision turns medially across the posterior edge
of the hard palate. It then turns 90 0 anteriorly, several mm to the proximal
side of the midline, if possible to cross the gingival margin once again.
The incision is carried to the bone, except beneath the lower eyelid,
where the orbicularis oculi muscle is preserved. The cheek flap is then
reflected back to the tuberosity. The central incisor on the involved side is
extracted and the gingival and palatal mucosa is elevated back to the
midline. The incision extending around the nose is then deepened into the
nasal cavity. The palatal bone is then divided near the midline with a saw
blade or bur. The basal bone is then separated from the frontal process of
the maxilla with an osteotome. The orbicularis oculi muscle is retracted
superiorly and the bone cut is extended across the maxilla, just below the
infraorbital rim into the zygoma. If the posterior wall of the maxillary sinus

has not been invaded by the tumour, it is separated from the pterygoid
plates with a pterygoid chisel. The entire specimen is removed by severing
the remaining attachments with a large curved scissors placed behind the
maxilla.
After removal of the specimen, some amount of brisk bleeding is
expected, which is controlled with packing and electrocautery. Branches of
the maxillary artery in the pterygomaxillary fissure area may require
ligation. While packing in place, the specimen should be inspected to make
sure that complete tumour has been excised. All sharp bony projections
should be trimmed. The palatal flap is turned up to cover the medial bony
margin. A split- thickness skin graft is then sutured to the wound margins
to cover the entire defect. Graft is maintained in place by placing softened
impression compound ball in the defect. The surgical obturator, which is
prefabricated, is placed to seal the defect and support the packing. The
obturator is fixed to the remaining teeth by means of interdental wiring. The
main cheek flap is then turned back and closed in layers.
Modifications(i)

When the tumour extends upto the roof of the maxillary sinus, but
does not invade, then the orbital floor should be included in the
resection.

(ii)

When the tumour invades the roof of the maxillary sinus, orbit or
the ethmoid sinuses, orbital exenteration is mandatory.

(iii)

The tumours, which are confined to the posterior aspect of the

maxillary sinus, may be managed with amore conservative
resection that spares the premaxilla.

Postoperative management1) Patients are kept in ICU for first one or two days for cardiovascular
monitoring and necessary maintenance of fluid and electrolyte balance.
2) Patients are kept on liquid diet.
3) Instruct to maintain the oral hygiene.
4) Prophylactic antibiotics are advocated for 5-7 days.
5)

The surgical obturator is removed after 15 days and the wound is

irrigated with warm saline.

6) Excess skin graft and other debris are removed.

7) Long-term follow-up is necessary.

RECONSTRUCTIONAfter

surgical

resection

with

continuity

defect,

there

is

disfigurement, deviation of the mandible along with resultant altered

mandibular movements with facial asymmetry.

Need for reconstruction:

For restoration of the movements and equilibrium of the mandible.

For maintenance of normal occlusion, floor of the mouth and tongues

anatomical position.

For restoration of near normal feeding.

For acceptable esthetics and function.

For more favourable social acceptance.

Immediate reconstruction:
Advantages1. Single stage surgery.
2. Early return of the function.
3. Minimal compromise of esthetics.
Disadvantages:
1) Recurrence in the grafted bone.
2) Loss of graft from infection.
Three ways of immediate reconstruction:
A] Performing the surgical excision and grafting, both via intraoral
approach.
B] Surgical excision utilizing both intraoral and extraoral approach, first
obtaining the watertight oral closure and grafting is done through extraoral
approach.
C] Earlier extraction of the involved teeth and waiting for 6-8 weeks for oral
healing and then surgery at second stage, with complete procedure via
extraoral approach to avoid communication of the graft.

Delayed reconstruction (second stage):

Here the consideration should be given to maintain the residual
mandibular fragments with their normal anatomic relationship with
intermaxillary fixation or using reconstruction plate. This is to avoid

cicatricial and muscular deformation and displacement of the segments,

which aids in the secondary reconstructive efforts at a later date. Usually
six months waiting period is observed for recurrence.
I. AMELOBLASTOMA.
Synonym:
Adamantinoma
Introduction:
It is a true neoplasm of odontogenic epithelial origin. It is the second most
common odontogenic neoplasm. Its incidence combined with its clinical
behaviour makes Ameloblastoma the most significant odontogenic
neoplasm of concern to oral and maxillofacial surgeons. Opinions differ
regarding its terminology, etiology, histological pattern, classification,
clinical and biological behaviour, diagnosis, treatment aspect and
malignant potential.
History:
1827 first recognized by Cuzack.
1879 described by Falksson.
1885 Malassez coined the term Adamantinoma.
1934 Ivy and Churchill coined the term Ameloblastoma.
Definition:
Robinson defined Ameloblastoma as usually unicentric, nonfunctional,
intermittent in growth, anatomical benign and clinically persistent.

WHO it is a true neoplasm of enamel organ type tissue which does not
undergo differentiation to a point of enamel formation.
Types:
I. Clinicallya. Central or intraosseous.
b. Peripheral or extraosseous.
c. Desmoplastic.
d. Unicystic
II. Histologicallya. Follicular
b. Plexiform
c. Acanthomatous
d. Granular cell
e. Basal cell
Pathogenesis:
The origin of Ameloblastoma is not known with certainty. According to
Thoma, Williams in 1993, the tumour may be derived from various origins.

Late developmental sources cell rests of enamel organ, either

remnants of dental lamina or epithelial cell rests of Malassez or
remnants of Hertwigs sheath, follicular sacs.

Early embryonic sources disturbances of developing enamel

organ, dental lamina, tooth buds.

Basal cells of the surface epithelium of the oral mucosa.

Secondary developmental sources epithelium of odontogenic

cysts, particularly primordial, lateral periodontal cyst, dentigerous
cyst and odontomas.

Heterotopic epithelium in other parts of body, especially from the

pituitary gland.

With concepts of neoplasia in general, it is likely the result of

alterations or mutations in the genetic material of cells involved in
odontogenic cysts.

Environmental factors

Patient variables general health status, nutritional status.

Clinical features:
Frequency it accounts for approximately 1% of all oral tumours.
18% of all odontogenic tumours.
Age the average age at diagnosis is around 33 39years. And most
cases cluster between 1st decades to 7th decade.
Sex men = female.
Race more common in blacks and some studies identifies Asians as most
commonly affected population.
Site - mandible is most commonly affected area.

(5:1).

There is a

predilection for posterior maxilla and posterior molar-ramus of the

mandible.
Clinical presentation(i)

Asymptomatic

(ii)

C/o swelling and facial asymmetry.

(iii)

Swelling is usually slow-growing, hard, non-tender, and ovoid

which is often large in size.

(iv)

Pain occasional sign, if secondarily infected.

(v)

Ulceration of the overlying mucosa.

(vi)

Tooth mobility.

(vii)

Occlusal alterations

(viii) Failure of eruption of teeth.

(ix)

Exfoliation of teeth

(x)

Ill fitting dentures

(xi)

Nasal obstruction

(xii)

Later stage with nerve involvement sensory changes of the

lower lip.

(xiii) Large persistent lesions may exhibit fluctuation and egg-shell

crackling.
If not treated,
It invades into the neighboring tissues by replacing them. Invasion into the
medullary space is the first feature.

When the tumour attains larger size

with bone erosion, there is escape into the periosteum mucosa and
muscles of adjoining region.
Root resorption will occur.

Locally aggressive invasion in

maxillofacial region may compress vital structures, obstruct airway,

impairs swallowing, erode major arteries, or invade middle cranial fossa.

The extensive tumours can cause gross facial deformity.

Size ranges from 1 cm upto 16 cm.
In maxilla, it may enlarge to involve maxillary sinus, and nasal cavity
leading to nasal obstruction and even proptosis of eye.
Radiographic findings:
(i)

It presents as a unilocular / multilocular radiolucent lesions.

(ii)

50 % of ameloblastomas appear as multilocular radiolucent

lesions with sharp borders.

2 % of the Ameloblastomas are

peripheral. 6 % appear as Unicystic lesions.

(iii)

The apparent lesional edge may be distinct or indistinct.

(iv)

Multilocular cyst like radiolucency with compartmentalized

appearance due to bony septa ( honey comb or soap bubble
appearance ) resembles fibromyxoma, giant cell lesions.

(v)

Small or large unilocular or multilocular lesion may contain

unerupted deciduous or permanent teeth and may resemble a
dentigerous cyst.

(vi)

Lesions in dentulous area cause root resorption (30 %) and tooth

displacement.

(vii)

Buccolilngual cortical expansion (80%), this tendency is stronger

than a cyst.

(viii) The displacement of neurovascular bundle at the inferior border

is often seen.

(ix)

Maxillary lesions often involve the maxillary sinus and change the
normal radiolucency of the sinus to a more opacified appearance.

(x)

The radiographic image of Ameloblastoma is at the most

suggestive and not pathognomonic. CT scan with 3-D reconstruction will

show the exact extent of the lesion.
(xi)

One of the variants of the Ameloblastoma the desmoplastic

Ameloblastoma most often found in the anterior maxilla or mandible

appears as a relatively radiopaque lesion because of its dense connective
tissue content (solid type lesion).
Histopathology:
The Ameloblastoma is composed of nests, strands and cords of
Ameloblastic epithelium, all separated by relatively small amounts of
fibrous connective tissue stroma. The characteristic histological features
of the Ameloblastoma are the orientation of the outer epithelial cell nuclei,
which are positioned (polarized) away from the basement membrane.
Two main patterns are seen:
1. Follicular

type

(resembles

tooth

follicle)

consists of small to large odontogenic epithelial

nests (the follicles) and variously shaped and
sized ameloblastomatous islands (more common
pattern). Cyst formation is commonly seen.
2. Plexiform type consists of interlacing strands of
narrow or wide odontogenic epithelial trabeculae

resembling the dental lamina.

Both these

patterns may be observed in the same tumour.

Both these types have a columnar or cuboidal outer epithelial cell layer
bordering the connective tissue stroma and enclosing the inner epithelial
cells.
Subtypes
(i)

Acanthomatous type compression of stellate reticulum into a

squamoid mass with squamous metaplasia is seen. Sometimes
keratin formation in the central portion of the tumour islands is
noticed. Occasionally epithelial or keratin pearls may be seen.
Usually seen in follicular type of Ameloblastoma or peripheral
Ameloblastoma.

(ii)

Basal cell type bears resemblance to the basal cell carcinoma of

the skin.

The inner follicular and outer follicular cells may

assume a basal cell appearance throughout the lesion.

(iii)

Desmoplastic type variant of solid Ameloblastoma. Abundant

fibrous tissue stroma can be seen in this variety. Some areas of
calcification also can be seen. Marked stromal desmoplasia is
noticed. High recurrence rate.

(iv)

Granular cell type stellate reticulum like cells, get transformed

into cells with coarse granular, eosinophilic cytoplasm. This type
is found to be more aggressive type with high chances of
recurrence and metastasis (malignant transformation)

(v)

Mural Ameloblastoma another name for Unicystic lesion, which

is found in children and young adults.

The Ameloblastic

epithelium lines the luminal surface and may proliferate into the
cyst (mural growth). Often budding aggregates of basal cells,
follicular cells, or plexiform strands extend from the luminal lining
into the cystic wall. Further growth of the lesion takes place by
creation of microcystic and macrocystic formations in the
budding Ameloblastic elements that extend and enlarge the
primary cystic lumen or the cyst wall by resorption of host bone.
Differential diagnosisII.Radiologically

Dentigerous cyst,

Odontogenic keratocyst,

Cherubism,

Giant cell granuloma,

Odontogenic myxoma,

ABC.

II. CALCIFYING EPITHELIAL ODONTOGENIC TUMOUR.

Synonym: Pindborgs tumour.
Acronym: CEOT.
Introduction:

CEOT is an uncommon odontogenic tumour that appears to occupy

a position between hamartoma and aggressive neoplasm.
CEOT has been categorized by 1992 WHO classification as a benign,
locally invasive odontogenic neoplasm that is exclusively epithelial in its
tissue origin.
History:
Described by J J Pindborg in 1955.
Thoma and Goldman (1946) and Ivy (1948) have erroneously reported
the case of CEOT as Ameloblastoma.
1976-Franklin and Pindborg have reported that 113 cases of CEOT have
been described in literature since 1955.
2002-Neville, Damn, Allen and Bouguot have reported that around 200
cases of CEOT have been described in the literature.
Types:
I. Based on site of occurrence Intraosseous-within the bone.
Extraosseous-outside the bone.
II. Based on histopathology

Classical type.

Clear cell variant.

CEOT

associated

odontomas.
Pathogenesis: -

with

AOT,

dentigerous

cysts

and

confirmed

etiology

still

remains

elusive.

The

probable

mechanisms arei. Reduced enamel epithelium.

ii. Stratum intermedium c ells of enamel organ.
iii. Immunologic response to stratum intermedium cells.
iv. Remnants of the primitive dental lamina.
v. Basal layer of oral epithelium.
Clinical featuresFrequency- uncommon to rare odontogenic neoplasm.

Represents less

than 1% of all odontogenic neoplasms.

Extraosseous-5 to 6% of all CEOTS.
Age ranges from 8-92 years.
Most commonly reported in patient during 4th, 5th and 6th decades of life.
Average patient age between late 30s to early 40s. 37-43 years.
Extraosseous 12-48years.
Sex no sex predilection.
Race occurs more frequently in whites.
Site - mandible is preferred site than maxilla (3:1) may occur in any area of
the jaw, but premolar/molar region appears to be favoured. (65%)
Extraosseous anterior region. Predilection for incisor/premolar region.
Clinical presentationIntraosseous lesion-

2. Usually

asymptomatic,

discovered

through

routine radiographic examinations.

3. Present as a slow-growing, painless, expansile,
hard, bony swelling cortical bone thinning
egg-shell crackling perforation soft tissue
infiltration.
4. May cause associated tooth tipping, rotation,
migration and/or mobility secondary to root
resorption.
5. In maxilla, the lesion may invade maxillary sinus,
pterygoid space, ethmoid and sphenoid sinuses
by extension.

Extraosseous lesiona. Painless mucosal-coloured to red swelling

of

about

6-month

duration

that

may

produce saucerization of the underlying

bone.
b. Sometimes may produce pain.
Radiographic featuresNot pathognomonic.
Radiographic features ranges with regard to

i. Lesion size and

ii. Bone pattern.
Routine dental radiographic images show65% - mixed radiolucent and radiopaque pattern.
32% - completely radiolucent.
3% - totally radiopaque.
Wind - driven snow appearance scattered flecks of calcification
throughout radiolucency.
May be unilocular and cystic, often associated with an unerupted teeth but
cyst like hydraulic pattern of cortical expansion is absent.
May be multilocular
i. Honeycomb pattern locules appearing small, round and somewhat
corticated.
ii . Soap bubble pattern large spaces.
Radiographic border between tumour and surrounding bone appears to be
diffuse / well defined and circumscribed.
3 features associated with teeth that assist in the diagnosis of CEOT
I.

Embedded tooth that is rarely impacted e.g. I/II molar.

II.

Inferior displacement of mandibular unerupted tooth causing

protuberance in this region because of penetrating apex of the
tooth into the inferior cortex.

III.

Apical extension of the occlusal c luster that may obscure the

embedded tooth.

Erupted teeth may be displaced and root resorption may be seen in

adjacent teeth.
CT scanExpansion and thinning of buccal and lingual cortical plates by a
well-defined mass containing scattered radiopaque areas of varying size
and signal intensity.
MRI
Predominantly a hypointense lesion on T1-weighted images.
A mixed hyperintense lesion on T2-weilghted images.
Hilstopathological features: Has characteristic histopathologic features1. Trabeculae and/or sheets of large, eosionophilic staining,
squamoid shaped epithelial cells frequently with prominent
intercellular bridges.
2. Tumour cells vary from polygonal to round to oval in shape. It
may be irregular, pleomorphic and ominous in morphology.
3. Scattered cells with clear glycogen rich cytoplasmic contents
may be seen.
4. Nuclear morphology and staining is highly variable. Single to
multinucleated cells that has normal to very large nuclei,
abnormal/pleomorphic in shape and have hyperchromaticity with
deep basophilic staining characteristics.

5. Increased

number

and/or

abnormal

mitotic

figures

are

uncommon.
6. Connective tissue stroma is usually inconspicuous between the
islands and sheets of epithelium.
7. Presence of a homogeneous, eosionophilic substance which has
been identified as amyloid-characteristic
8. Ultrastructural studies show 2 forms of amyloidi. Immunamyloid-arise from the light chain fragments
of immunoglobulins.
ii. Apudamyloid-arise

from

the

cells

of

certain

endocrine tumours, which may be derived from the

endocrine polypeptide cells of neural crest origin of
amine precursor uptake and decarboxylation.
9. Immunohistochemical and electron microscopy reveals that the
amyloid is derived froma. Degenerative

process

involving

the

cytokeratin

intermediate filaments in tumour cells.

Or
b. Degeneration of type IV collagen associated with basement
membrane.
10. Special staining for amyloidi. Crystal violet stains metachromatically.

ii. Congo red display bright orange to red hue when

exposed to polarized light apple green
iii. Thioflavin T fluoresces to a blue colour decreases
an UV light.
11. Prominent or scattered calcifications, in concentric crenate like
shapes liesegang rings are seen maturing within the amyloid
stroma.
12. Chen et al, reported that CEOT expresses bone sialoprotein.
Liesegangs rings and epithelial cells near them demonstrated
positive histochemical staining to bone sialoprotein antibodies
evidence of a n in situ hybridization bone sialoprotein mRNA
signal respectively.
Differential diagnosisII.

Clinically1. Ameloblastoma
2. Odontogenic Myxoma
3. Fibro Myxoma
4. Fibroma
5. Ameloblastic fibroma
6. Ameloblastic fibro odontoma
7. Large dentigerous cysts
8. OKC.
9. OF.

10. CGCG.
11. Central giant cell lesion.
12. Osteoma.
13. Osteoblastoma.
14. Cementoblastoma.
15. Primary odontogenic carcinoma.
16. Metastatic carcinoma of jaws.
17. Osteosarcoma.
18. Chondrasarcoma.
III.

RadiologicallyA. Radiolucent1. Dentigerous cyst

2. OKC.
3. Gorlin cyst.
4. AOT
5. Central giant cell lesion
6. Early stage of ossifying fibroma
7. Early stage osteoblastoma
B. Mixed1. AOT
2. Gorlin cyst
3. Ameloblastic fibroodontoma
4. Maturing / advanced stage of ossifying fibroma

5. Osteoma
6. Osteoblastoma
C. Completely radiopaque1. Odontoma
2. Gorlin cyst associated with odontoma
3. AOT associated with odontoma
IV.

Histologically1. Squamous cell carcinoma.

2. Metastatic carcinoma of jaws.
3. Unknown primary metastatic adenocarcinoma.
4. Primary

intraosseous

mucoepidermoid

carcinoma.
5. Osteosarcoma.
ManagementThe surgical treatment of CEOT should be case specific and is
dependent on:
(i)

Size and location of the neoplasm

(ii)

Patients overall medical condition or tolerance to withstand the

surgical procedure.

(iii)

Clinical activity (perforation of the cortical bone, periosteal/nerve

involvement etc.)

(iv)

Recognized behavioural characteristics

(v)

Skill / experience level of the operator

Small, intrabony mandibular lesions with well-defined borders simple

complete but conservative tumour enucleation / curettage followed by
indicious removal of a thin layer of bone adjacent to the tumour.
Small maxillary lesions surgically excised by either through intrabony
curettage or conservative enblock resection.
Maxillary tumours can be treated more aggressively than a similar sized
lesion in the mandible.
Resection without continuity defect or resection with continuity
defect can also be used.
Resection with continuity defect segmental resections-significant
bone discontinuity requiring either distraction osteogenesis/graft and
reconstruction procedures.
Treatment of atypical/frankly malignant CEOTEarly intervention essential.
Radical resection of the affected jaw portion and associated soft tissue
with no less than 1cm in every dissection.
Adjunctive external beam radiotherapy-local spread to cervical
lymph nodes
Adjunctive chemotherapy-controls distant organ metastasis.
Radiotherapy in lieu of surgical resection is contraindicated for CEOT.
Treatment of peripheral CEOT
According to Houston and Fowler, simple local excision.
Prognosis-

Local recurrence rate 10%-20%

Incidence of malignant transformation to odontogenic calcifying
epithelial pindborg tumour is extremely low.
No reported recurrences in patient treated for peripheral CEOT.
II.

ADENOMATOID ODONTOGENIC TUMOUR

Synonymsa. Adamantoma
b. Epithelial odontome
c. Tooth germ cyst of the jaw
d. Adenoameloblastoma
e. Glandular Ameloblastoma
f. Adenomatoid Ameloblastoma
g. Ameloblastic adenomatoid tumour
Acronym- AOT

IntroductionAOT

is

benign,

nonaggressive

odontogenic

tumour

characterized by the formation of duct like structures by the

epithelial component of the lesion.
AOT is considered as an odontogenic tumour becausea. Occurs only in the tooth bearing area of
the jaws.

b. Its

histomorphologic

resemblance

to

components of tooth germ.

AOT is the fourth most frequent odontogenic tumour.
Some investigators consider AOT as an hamartomas becausei. Limited size of most cases
ii. Lack of recurrence
Others consider AOT to be a non-aggressive, non-invasive benign
neoplasm because
i. The limited size of the most cases
stems from the fact that most are
detected early and removed before
slow-growing

tumour

reaches

clinically noticeable size.

ii. The

microscopic

lesional

tissues

features

of the

show

greater

departure from the arrangement of

the normal odontogenic apparatus,
than

should

be

expected

in

developmental anomaly.
Gardner describes AOT as benign embryonal neoplasm
History1909-James and Forbes reported case as epithelial odontome almost
certainly an AOT (England).

1915- earliest irreputable case reported by HARBITZ, Norway, as

Adamantoma.
1916-Ist

acceptable

American

case

reported,

Tooth

germ

(chorioblastomatous) cyst of the jaw by Wohl.

1948-Stafne reported first series of AOT as epithelial tumours associated
with developmental cysts of the maxilla.
1950-BERNIER AND TIECKE, coined the term adenoameloblastoma
1951-V annual American Academy of Oral Pathology meeting simple
Ameloblastoma and adenoameloblastoma as separate entities was
presented for further consideration but was not adopted.
1961-GORLIN et al introduced the term Ameloblastic adenomatoid tumour.
(AAT)
1963- SHAFER et al adopted the term AAT in his 2 nd edition of Textbook of
Oral Pathology.
1968-ABRAMS et al suggested the term odontogenic adenomatoid
tumour
1969-PHLIPSEN AND BIRN, proposed the name AOT.
1971-the term AOT was adopted in the initial edition of WHO Histological
typing of Odontogenic tumours, jaw cysts and allied lesions.
1992- retained in the 2nd edition of WHO Histological Typing of
Odontogenic Tumours, jaw cysts and allied lesions
Types

Based on the location-

1. Intraosseous/central/intragnathic
2. Extraosseous/peripheral/extragnathic

Radiographically1. Pericoronal
2. Extracoronal

PathogenesisA variety of ultrastructural studies attempting to clarify the origin of

AOT have been carried but in vain. The specific stimulus that triggers the
proliferation of the progenitor cells of AOT is unknown.

Nearly all

investigations have agreed upon the cytologic resemblance of the

structures of AOT to the dental lamina and components of enamel organ.
Thoma suggested a dual origin for AOT(i)

Salivary

(ii)

Odontogenic

This was ruled out by Smith et al in 1979 who showed the presence of
hemidesmosomes and basal lamina at the luminal pole of the cells that
form the duct like structures.
This was supported by negative reactivity of these cells to lactoferrin and
L1 antichymotrypsin antibodies. (Takahashi et al).
The duct forming cells and non-duct forming cells exhibit secretory
granules and coated vesicles near the luminal pole resembling inner
enamel epithelium cells as they develop through preameloblast stage.

(Smith et al, Schlosnagle and Sameren, Yamamoto et al and Poulson and

Greer).
Spindle cells that are between cell-rich nodules resemble ultrastructurally
the stellate reticulum and those that are immediately adjacent to the
nodules resembles stratum intermedium of enamel organ.
The cell present in the tumour resembles the outer enamel epithelium cells
(Hatakeyama and Sujuki).
The cells present in the tumour have been described to resemble
odontoblasts. (Khan et al).
Philipsen et al, 1992 argued that AOT arises from remnants of the
successional lamina/accessional dental lamina.
Clinical featuresFrequency-3%-7% of all odontogenic tumours.
Intraosseous-97%
Extraosseous-3%
Age ranges from 3-82 years of age. Its predilection for young patients is
well established.
88% diagnosed in the 2nd and 3rd decades of life. (Intraosseous)
Extraosseous 12-14years
Sex females are commonly affected (2:1) intraosseous extraosseous F;
M= 14:1
Race more common in b lacks. (Just harvesting phenomenon).

Site maxilla more commonly affected than mandible (2:1).

predilection for anterior jaw.

Marked

85 % of lesions appear anterior to the II

premolar (central and peripheral)

Clinical presentation
Intraosseous
(i)

Usually asymptomatic, but patient may be aware of an area of jaw

enlargement.

(ii)

Discovered on routine dental radiographic examination.

(iii)

Delayed eruption of permanent tooth or slow-growing bony

expansion discovery of the central AOTs

(iv)

Mobility of teeth, swelling of the cheek, and asymmetrical facial

swelling has been reported less frequently.

(v)

Infection of the tumour or fracture of the mandible rarely led to

discovery of the lesion.

(vi)

Nasal obstruction rarely encountered.

Extraosseous(i)

Gingival coloured mass that ranges from 1 1.5 mm in diameter.

(ii)

Epulis like fibrous swelling that mimics periodontal disease if

bone erosion occurs.

(iii)

10 times more prevalent in maxillary gingival.

(iv)

Painless

(v)

Peripheral lesions represent central lesions that erupted with

tooth, to complete their development in the gingival.

Radiographic featuresA. Pericoronal70 % of AOTs are associated with the crown of an

unerupted tooth.
6% associated with two or more unerupted teeth.
60% of AOTs associated with cuspids.
40% with maxillary cuspids.
3% associated with permanent molars.
B. Extracoronal 30% of AOTs
The relationship to the roots of adjacent / nearby teeth that
ranges from that lateral / interproximal to Periapical to no relationship at
all.
89% of extracoronal AOTs occurred adjacent to permanent cuspids.
Radiographic presentation1. Not pathognomonic.
2. As well-circumscribed, pear-shaped radiolucency lying between the
diverging roots of the canine and I premolar teeth or
3. As unilocular, radiolucent lesion that is well-circumscribed exhibiting
smooth corticated border.
4. Most lesions are pericoronal or juxta coronal, but the radiolucency may
extend apically beyond the CEJ.

5.Rare multilocular lesions have been reported and a scalloped border is

observed occasionally.
6. Lesion may range from 1.5 7 cm and more.
7. May be associated with one or more impacted teeth.
8. Maxilla slight buccal expansion of cortex.
9. Mandible significant expansion present.
10. Maxillary lesions may grow into nasal fossa and antrum and may
obliterate the antrum and expand the orbital floor.
11. Evidence of calcification within the lesion (65%) faint/quite dense
radiopaque foci.
Patterns of calcifications
Snowflakes
Animal prints
Hand / foot prints
Doughnuts
Semicircles
Groups of dots.
Predominant pattern-even distribution throughout the lesion.
12. Occasionally, intralesional radiopacity may be identified usually
eccentrically positioned within the lesion.
13. Unique feature presence of capsular space 0.3 0.8 cm wide well
defined, radiolucent band, that partly / most often completely surrounds
the periphery of the lesion.

14. Divergence of the roots and displacement of teeth occurs frequently.

15. Root resorption is rare.
16. Gingival lesions rarely are detectable radiographically but those may be
slight erosion of the underlying alveolar bone cortex.
Histologic featuresA. Macroscopic features
Soft, roughly spherical mass with a discernible fibrous capsule.
White to tan solid to crumbly tissue or one/more cystic spaces of
varying size.
Minimal yellow brown fluid to semisolid material.
Fine, hard gritty granular material.
One to several larger calcified masses.
B. Microscopic featuresTumour

is

made

up

of

cellular

multinodular

proliferation of spindle, cuboidal, and columnar cells in

a variety of patterns; usually scattered duct like
structures, eosinophilic material, and calcifications in
several forms; and a fibrous capsule of variable
thickness.
6. Cell-rich epithelial nodules (hyaline droplets /
tumour droplets)
7. Microcysts
8. Internodular epithelial cells

9. Basaloid epithelial cells

10. Calcifying epithelial odontogenic tumour-like foci
11. Cystic space
12. Reduplicated basement membrane
13. Dysplastic dentin/dentinoid
14. Calcified bodies
15. Stroma
16. Fibrous capsule
17. Cytologic atypia
Differential diagnosisI.

Clinically
Central odontogenic tumours and cysts
Benign fibroosseous lesions
Benign mesenchymal neoplasms

Gingival lesions
Gingival fibroma
Peripheral cemento ossifying fibroma
Peripheral giant cell lesions
Other peripheral odontogenic tumours
II.

Radiographically
1. Pericoronal
1. Dentigerous cyst
2. OKC

2. COC
3. Unicystic Ameloblastoma
4. Ameloblastic fibroma
5. Early Ameloblastic fibro-odontoma
6. Odontogenic fibroma
7. CEOT.
2. Extracoronal
a. Aforementioned odontogenic tumours and
cysts
b. Central giant cell lesion
c. Benign fibroosseous lesions (early C-OF).
d. Lateral periodontal cyst
e. Lateral radicular cyst
f. Apical radicular cyst
g. Central benign mesenchymal neoplasms
(neurilemoma).
III.

Histologically

1. Vascular Ameloblastoma
2. Plexiform type of Ameloblastoma
Treatment
Because of uniformly benign biologic behaviour of AOT and
consistent presence of a well-developed fibrous capsule conservative

complete surgical excision by means of enucleation and curettage

treatment of choice.
Impacted teeth incorporated within the lesion should be removed.
Teeth whose radicular bone cover has been resorbed by the lesion
endodontically treated or removed.
Several reported cases have resolved or failed to progress following
incomplete removal of varying extent not a preferred treatment
incisional biopsy above or subtotal excision following confirmatory
incisional biopsy or marsupialization followed by cystectomy.
Prognosis1. Growth rate slow / very slow growing tumour, but no
report of measurements of growth rate over a course
of time could be located
2. Recurrence rate till date only 5 cases of recurrence
have been reported. Unequivocal case of recurrence
has not been reported.
Association of AOT with other odontogenic cysts and tumours

Dentigerous cyst

Combined epithelial odontogenic tumour

Odontoma

Ameloblastoma

COC

Unusual cases

(i)

Melanotic AOT

(ii)

Multilfocal AOT.

Future researchModern research methods have facilitated the advancement of our

understanding of the histogenesis of this lesion but unanswered questions
remain.

We await the results of in situ hybridization, DNA microassay

analysis, gene rearrangement studies and other developing molecular

biology techniques to solve the remaining mysteries.
IV.

SQUAMOUS ODONTOGENIC TUMOUR

Synonyms
Benign epithelial odontogenic tumour
Acanthomatous Ameloblastoma
Acanthomatous Ameloblastic fibroma
Hyperplasia and squamous metaplasia of residual odontogenic
epithelium
Benign odontogenic tumour, unclassified.
Acronym SOT.

Introduction
SOT is a relatively rare, benign, but locally infiltrative and
occasionally aggressive odontogenic epithelial lesion that appears to have
hamartomatous and neoplastic characteristics.
History-

1975 first described by PULLON et al.

Since then a number of papers have amplified details of the clinical
presentations and range of biologic activity of SOT.
Types
I. Based on the site of occurrence
Intraosseous
Extraosseous
II. Based on histopathology Solid type
Cystic type
PathogenesisMay be multifactorial.
(i)

Cell rests of Malassez} intraosseous

(ii)

Dental lamina

(iii)

Surface stratified squamous epithelium} extraosseous

(iv)

Cell rests of Serrae

Clinical features
Frequency rare odontogenic epithelium. 1 case of extraosseous lesion
described.
Age SOT occurs over a wide range of age ranging from 2 nd 7th decade
and reported incidence peaks in the third decade of life.

Sex no sex predilection.

But some studies show slight male

preponderance and some others show slight female preponderance.

Race no racial predilection. But SOT was diagnosed in whites, blacks
and Asians.
Site maxilla = mandible. Some studies show that mandible is commonly
affected.
Maxilla lesions centered around the incisor cuspid region
Mandible lesions had a predilection for the bicuspid molar area.
Lesions may be found singly or multicentrically in all areas of both jaws.
Clinical presentation(i)

Often

asymptomatic.

Casually

discovered

by

routine

radiographs.
(ii)

Lesions involving dentate areas alveolar resorption around the

teeth tooth mobility and pain.

(iii)

Tender on percussion positive

(iv)

Occasionally abnormal sensations present.

(v)

If cortical bone is penetrated by the lesion, tumour infiltrates

gingival, occasionally unattached alveolar mucosal, palatal soft
tissue and rarely the buccal mucosal.

(vi)

Aggressive maxillary tumours may infiltrate the maxillary sinus

and nasal floor and nasal spine.

(vii)

Familial SOTs have been reported.

Radiologic features-

Not pathognomonic of SOT.

Presents as a triangular / semicircular

radiolucency within the alveolar bone between the roots of several teeth.
Displacement of one / both the adjacent roots. Destruction of crestal bone.
Presence of smooth sclerotic border at the margin of the lesion. Some
lesions are cystic / are associated with cysts.

Thus they may appear

radiographically as dentigerous, periodontal / Periapical in form.

Extraosseous saucerization of underlying bone.
Histologic featuresI. Solid type(i)

Consists of numerous islands of squamous epithelium dispersed

in a connective tissue stroma.

(ii)

Islands are distributed in a uniform fashion and are well

demarcated from the surrounding connective tissue stroma.

(iii)

The basal cell layer consists of flat to cuboidal cells and the
internal cells exhibit squamous differentiation.

(iv)

The central areas of islands show foci of parakeratin or keratin

calcification or cellular degeneration.

(v)

The epithelial cells are uniform and without pleomorphism or

nuclear hyperchromatism or mitotic activity.

II. Cyst type Contains many islands of squamous epithelium in the connective
tissue wall, either completely separated from the odontogenic

epithelium lining the lumen or appearing to arise directly from the

luminal epithelial lining.
These

islands

often

exhibit

vacuolization

and

microcyst

formation.
Chronic inflammation may be seen.
Differential diagnosisI. Clinically
1. Periodontal disease
II. Radiographically
1. Dentigerous cyst
2. Apical periodontal cyst
III. Histologically
Acanthomatous and Desmoplastic Ameloblastoma
SCC

Treatment
Complete excision is required depending on the clinical and
radiographic extent of the lesion.
Intraosseous
1. Enucleation
2. Curettage
3. Local excision

Clinically aggressive lesions enbloc excision

Intrabony lesion involving the soft tissue due to the penetration of cortical
bone soft tissue should be included in the excision.
Teeth within the lesional area extracted.
Cystic lesion treated conservatively.
Complete excision by enucleation and / or curettage.
PrognosisSOT exhibits a biologic behaviour pattern of aggression that appears
unaccountable in strictly histologic terms.
Recurrences of incompletely excised lesions have been reported.
V.

ODONTOGENIC FIBROMA

IntroductionA benign, relatively rare connective tissue tumour that contains a variable
amount of inactive odontogenic epithelium.
It has a benign behavioural course.

Types(i)

Based on site of occurrencea. Intraosseous / central.

b. Extraosseous /peripheral.

CENTRAL ODONTOGENIC FIBROMA

Introduction-

The odontogenic fibroma is a central tumour of the jaws which is

seen so infrequently that little is known about this neoplasm. There is lack
of unanimity of definition of the lesion. Hence lots of uncertainty regarding
this tumour.
WHO classified COdF under Odontogenic ectomesenchyme with / without
included odontogenic epithelium
Slater et al (2001) considers this to be a mixed epithelial Ectomesenchymal
neoplasm.
Philipsen and Reichart (2002) , believes that the fibrous connective tissue
component is of a mature mesenchymal type and not that of an
Ectomesenchymal type.
DefinitionWHO defines COdF as a fibroblastic neoplasm containing variable
amounts of apparently inactive odontogenic epithelium and in some cases,
calcified material resembling dysplastic dentin or cementum like
material.

Types1. Simple type composed of delicate fibrous and myxoid tissue with
scant numbers of inactive appearing islands and strands of
odontogenic epithelium
2. WHO type / complex type composed of cellular, mature, fibrous
tissue containing numerous strands and islands of odontogenic

epithelium without palisading, reverse polarization or Stellate

reticulum.
Handlers et al (1991) concluded that COdF need not be separated into
simple and WHO types because their histologic patterns did not
correlate with clinical behaviour.
The tumour formerly classified as the simple type was subclassified
as a myxofibroma under Myxoma section in WHO histological typing of
odontogenic tumours.
Basic concepts concerning COdF1. It is a lesion around the crown of an unerupted tooth resembling a
small dentigerous cyst.
2. It is a lesion of fibrous connective tissue, with scattered islands of
odontogenic epithelium, bearing some resemblance to the dental
follicle because of size it may attain appearing to constitute a
neoplasm.
3. It is a lesion as described by WHO, as previously mentioned.

Pathogenesis1. Cells in dental follicle gives origin to COdF associated with

unerupted teeth.
2. Periodontal ligament gives rise to COdF not associated with
unerupted teeth.
Clinical features-

Frequency 4% -5% of all odontogenic tumours. Ranges from 0 14%.

Age 5 80 years. Mean 37 years.
Sex female predilection (2.8: 1)
Race occurs more frequently in whites. (95.5%)
Site occurs more frequently in mandible (52%).

Fonseca found a

predilection for maxilla. 77% of cases were located anterior to the I molar.
Clinical presentation1. Asymptomatic
2. Mild tenderness / sensitivity discomfort.
3. Paresthesia
4. Size 0.3 6.0 cm (Avg. 2.2 cm)
5. Duration few weeks to months to years.
6. Slow growing progressive enlargement
7. Unusual finding presence of a cleft / depression or sinus tract like
defect involving palatal gingival and palatal mucosal.

39% of

maxillary COdF anterior to I molar had an associated palatal cleft.

Mechanism of formation of such palatal clefts is poorly understood attributed to the presence of myofibroblasts. COdF extends through
a perforation in the palatal bone adhering to the periosteum and
infiltrating the palatal mucosal. The palatal mucosa clinically seems
to collapse into the bony defect.
Radiographic features1. Expansile, multilocular radiolucency majority.

2. May have mixed density or completely radiopaque mass.

3. May also be manifested as unilocular lesion
4. May have loculated / scalloped periphery.
5. Most lesions are well defined, with a sclerotic border. But in some
lesions the borders were ill-defined or irregular.
6. May also show cortical expansion
External resorption of the tooth roots
Displacement of teeth
Tooth mobility
Erosion / perforation of cortical bone
Displacement of the inferior alveolar canal.
7. May be associated with residual odontogenic cyst / roots of erupted
tooth / unerupted tooth.
Histological featuresThe microscopic components of COdF are
1. Fibrous tissue of variable cellularity and density.
2. Variable amounts of inactive appearing odontogenic epithelium.
3. Variable presence of calcifications resembling dysplastic dentin,
cementum like tissue or bone
1. Fibrous tissue Mesenchymal component varied from loosely collagenized to
well collagenized, with/without myxoid areas.

 Myxoid areas, when present, were focal to prominent in

distribution.
Cellularity varied from sparse to moderate to cellular.
Mitotic activity also was observed.
Eosinophilic, amorphous globules, which stain weakly for
amyloid but may represent enamel matrix protein infrequent.
Pleomorphic

plump,

stellate

shaped

and

binucleated

fibroblasts Gunhan et al.

2. Odontogenic epitheliuma. Inactive.
b. Present as islands / cords ranging from few to numerous.
c. Epithelial cells exhibited cytoplasmic vacuolization.
d. A zone of hyalinization may surround islands of epithelium.
e. Presence of microcystic spaces in epithelial strands Dunlap.
3. Calcificationsa. Ranged from focal to florid in distribution.
b. These were interpreted asNonspecific calcifications.
Cementum /cementum like
Cementum /dentin
Cementum and osteoid
Woven bone
None of the COdFs were encapsulated.

Differential diagnosis1. Giant cell granuloma

2. Odontogenic fibromyxoma
3. Desmoplastic fibroma
4. Neurofibroma
5. Ameloblastic fibroma
6. Follicular sac surrounding the crown of an unerupted tooth.
7. Metastatic carcinoma
8. CEOT
9. Ameloblastoma
10. CEOC
11. CGCG
12. Cementifying fibroma
TreatmentThis tumour is treated by surgical excision.

But the excision is

dependent on clinical extent and anatomic involvement of the individual

lesion. Most lesions can be managed by enucleation and curettage. The
lesions tended to shell out easily and completely from the surrounding
bone. The lesions associated with palatal defect dissection of the defect
at their junction with palatal mucosa and periosteum.

If the tumour

incorporates the mandibular neurovascular bundle, an attempt should be

made to preserve it. Teeth in the lesional area extracted. The recurrent
lesions can be treated conservatively. Rare is a resection required.

PrognosisRecurrence rate 26%.

Length of follow up ranges from 1 10 years. The time interval between
first surgical procedure and the first recurrence ranged from 14 48
months. The spectrum of histologic patterns bears no correlation with the
biologic behaviour.
PERIPHERAL ODONTOGENIC FIBROMA
Synonyms

Odontogenic gingival epithelial hamartomas

Peripheral Ameloblastic fibrodentinoma

Peripheral hamartomas of dental lamina rest

Calcifying fibrous epulis.

Acronym- POdF
IntroductionPOdF is considered to be the gingival counterpart of the COdF and is
rare in occurrence.

POdF can be confused with peripheral ossifying

fibroma, a reactive lesion.

Clinical featuresFrequency 1.2% of all odontogenic cysts and tumours.

Age ranges from I to VIII decades.
Sex M=F
Site mandible > maxilla. Found anterior to the II premolar.

Clinical presentation(i)

Measures around 1 3 cm in diameter.

(ii)

The lesions may be on the attached gingival or alveolar ridge.

(iii)

May be pedunculated / sessile.

(iv)

May have a normal gingival colour.

(v)

Large lesions may displace teeth but do not involve the

underlying bone usually.

Radiographic features
Radiographs reveals calcification in POdF more often than in the
COdF.
Histologic featuresPOdF

mirrors

central

lesion

in

histologic

appearance.

Unencapsulated proliferation of cellular fibrous or fibromyxomatous

connective tissue that exhibits variable amounts of odontogenic epithelium
and sometimes foci of calcification in the form of dentinoid, cementicles or
bone.
Differential diagnosis
1. Peripheral giant cell granuloma
2. Pyogenic granuloma
3. Giant cell fibroma
4. Simple fibroma
5. Peripheral ossifying fibroma
6. Neurofibroma

7. Fibroepithelial polyp
Treatment
The lesion should be excised to the interface with bone and a
modest perimeter of the uninvolved tissue.

Teeth incorporated in the

tumour should be extracted.

PrognosisUnknown potential for recurrence. 39% recurrence rate in a 3 4
year interval. (Daley et al, 1994 and Gardner, 1982).
VI.

GRANULAR CELL ODONTOGENIC TUMOR.

Synonyms

Granular cell odontogenic fibroma

Granular cell Ameloblastic fibroma

Granular cell tumour of the jaws.

Acronym GCOT
IntroductionRare, benign odontogenic neoplasm that contains variable amounts
of large eosinophilic granular cells and apparently inactive odontogenic
epithelium.
Types1. Central GCOT
2. Peripheral GCOT
Pathogenesis

Uncertain. Microscopic studies and immunohistochemical studies

support a mesenchymal origin for the granular cells.
Clinical featuresFrequency rare. Only 30 cases have been reported.
Age ranges from 16 77 years. Average 45.5 years.
Sex F > M (73.3%)
Site mandible > maxilla (3:1). Premolar / molar region most common site.
Clinical presentation
Asymptomatic.
Cortical bone expansion initial examination
Duration ranges from months to years
Displacement of the teeth
Facial swelling
Intraoral ulceration
Cortical perforation
Maxillary sinus involvement
Displacement of mandibular canal
Radiographic features(i)

May be radiolucent / mixed density / radiopaque.

(ii)

Patterns ranged from unilocular to multilocular often with a

sclerotic border.

Histologic features Specific.

 Consists of sheets / lobules of round to polygonal cells with

abundant, eosinophilic granular cytoplasm with round to ovoid
nuclei,
Scattered among the granular cells are cords and nests of
odontogenic epithelium. Epithelial cells often possess clear
cytoplasm.
Thin septae of fibrous connective tissue separate the lobules
of granular cells.
The islands of epithelium do not form stellate reticulum.
Scattered small cementum like or dystrophic calcifications
seen (50%). Florid distribution of cementum like calcifications
has been reported.
Ultrastructural studies supported the presence of lysosomes
within the cytoplasm of granular cells.
Immunohistochemical studies have shown that the granular
cells express
Vimentin
1 antiltrypsin
Antichymotrypsin
Lysozyme
CD68
HLA DR
But stains negative for cytokeratins and S-100 protein.

Differential diagnosis1. Ameloblastic fibroma

2. COdF
TreatmentTreated using conservative approach enucleation / curettage.
PrognosisDoes not show an aggressive biologic behaviour but can recur.
VI MYXOMA
Synonyms(i)

Odontogenic Myxoma

(ii)

Myxofibroma

(iii)

Odontogenic fibromyxoma

IntroductionBenign, neoplasm of uncertain histogenesis with a characteristic

histologic appearance, often behaves in a locally aggressive, infiltrating
fashion.
This

is

grouped

ectomesenchyme

with

under
without

the

subheading

included

Odontogenic

odontogenic

epithelium

according to WHO classification.

Traditionally,

myxomas

are

considered

to

be

neoplasm

of

odontogenic origin.
The odontogenic derivation of Myxoma is believed to origi9nate from
the primitive mesenchymal portion of the developing tooth germ as an

inductive effect of nests of odontogenic epithelium on mesenchymal tissue

or as a direct myxomatous change of fibrous tissue in an odontogenic
fibroma.
DefinitionWHO defines myxoma as a locally invasive neoplasm consisting of
rounded and angular cells that lie in an abundant mucoid stroma.
Types2 forms of myxomas are recognized in head and neck region.
1. Those derived from the soft tissue.
2. Those derived from facial skeleton nearly nonexistent.
a. Odontogenic myxomas
b. True Osteogenic myxomas.
Clinical featuresFrequency 3% - 6% of all odontogenic tumours. Occurs 2 4 times less
frequently than Ameloblastoma.
Age occurs from 11 months to 7 th decade. 75% occurred between 2 nd and
4th decades. 7% occurred in the I decade.
Sex slight female preponderance. F: M = 1.5: 1
Site 66% occurs in mandible. 34 % occurs in maxilla. Mandible: maxilla =
2:1.
Has a definite predilection for molar and premolar regions in both jaws.
May also occur in sinonasal tract, facial bones, extracranial skeleton.
Clinical presentation-

1. Usually asymptomatic.
2. Painless
3. Slow growing lesion and unilateral, some may cross the midline.
4. Mandible buccal and lingual swelling.
5. Maxilla swelling if sinus not involved.
6. Exophthalmos and nasal obstruction.
Radiographic features1. Periapical radiographs and panoramic radiographs I indicators of
Myxoma of jaws.
2. Not pathognomonic.
3. Ranges from small unilocular lesions to large multilocular lesions.
4. Often displaces the teeth.
5. Rarely resorb the roots of the teeth.
6. The multilocular pattern honeycomb, soap bubble, tennis racket,
wispy and spider web in appearance.
7. Multilocular myxomas > 4.0 cm. Unilocular smaller.
8. 5 % of myxomas associated with unerupted teeth.
9. May appear as radiolucent common.
Mixed density 12.5%
Radiopaque 7.5%
10. 1/3rd exhibited diffuse / poorly defined borders.
11. CT & MRI should be used to clearly define tumour margins and to
define the true extent of the Myxoma before surgery is performed.

12. Imaging help to determine the true extent and involvement of the
neoplasm.
13. Significant cortical expansion and perforation of the cortex with
invasion into the soft tissues.
14. In maxilla, extension into the antrum sunburst / hairbrush
appearance.
15. Extension into nasal fossa and zygoma and thinning the orbital floor
and hard palate.
Histological featuresI.

Macroscopic features1. Well-delineated, unencapsulated, gray-white to tan-yellow mass

that can be rubbery, soft or gelatinous in texture.
2. Margins are ill defined in gelatinous specimens
3. On cut surface, it is glistening, translucent and homogenous.

II.

Microscopic featuresBland in appearance.

1. Composed of loosely arranged, evenly dispersed spindle
shaped, rounded and Stellate cells with long protoplasmic
processes with a lightly eosinophilic cytoplasm in a mucoid
rich, intercellular matrix.
2. Mild nuclear pleomorphism / hyperchromatism exist. Mitosis /
binucleate cells occasionally be present.

3. Myxoid matrix is rich in hyaluronic acid and chondroitin

sulphate.
4. Myxomas have a fine network of reticulin fibres and some have
low collagen content.
5. Inconspicuous vascularity supports the diagnosis.
6. Occasional island / rest of inactive appearing odontogenic
epithelium may be found.
7. Non encapsulated and may pervade surrounding bone by
expansion.
Differential diagnosis1. Enlarged dental follicle with myxoid change
2. Odontogenic fibroma
3. Dental papilla of a developing tooth
4. Malignant tumours of the jaws
5. Myxoid neurofibroma
6. Myxoid liposarcoma
7. Myxoid chondrosarcoma
8. Desmoplastic fibroma
9. Ameloblastoma
10. Hemangioma
11. CGCG
12. ABC.
13. Hyperparathyroidism

14. Central neurilemmoma

TreatmentThe standard treatment for Myxoma is surgical excision. Radiation
therapy and chemotherapy are ineffective treatments.

A number of

surgical methods have been advocated

1. Excision
2. Enucleation and curettage
3. Curettage with / without electrical or chemical cautery.
4. Enbloc resection
5. Wide resection with and without immediate grafting.
GOLDS MANAGEMENT PROTOCOL1. Biopsy performed in a central area of the lesion and at the
radiographic lesion host bone interface histologically identifies the
lesion.
2. Excision of the lesion by enucleation and curettage is restricted to
unilocular lesions of no more than 1-2cm in diameter.

Chemical

cauterization (e.g. Carnoys solution) of the tumour bed may be of

value.

Teeth and alveolar process at the edge or within the

radiographic and clinical limits of the lesion should be removed.

3. Extensive lesions should be excised by resection without continuity
defect or resection with continuity defect including a perimeter
margin of tumour free bone of 1 cm depending upon the anatomic
extent of the lesion.

4. The neurovascular bundle of the mandible should not be sacrificed

routinely, even in lesions that require resection with continuity
defect.
5. The decision for immediate or delayed reconstruction is dependent
upon the
a. Clinical and / or histologic certainity of the complete excision
of the tumour.
b. Tissue requirements for reconstruction.
PrognosisBenign but aggressive tumour that has a propensity to infiltrate vital
structures.

Recurrence rate ranges from 10% - 33%.

Recurrences of

myxomas are related to incomplete removal than to the intrinsic biologic

behaviour of the tumour.
VII.

AMELOBLASTIC FIBROMA

Synonyms

Soft mixed odontogenic tumour

Soft mixed odontoma

Fibroademantoblastoma

Acronym- AF
IntroductionRelatively rare, true benign mixed tumour in which epithelial and the
Ectomesenchymal elements are neoplastic.

It is characterized by the

simultaneous neoplastic proliferation of the mesenchymal and epithelial

components without formation of dental hard tissues, namely dentin and

enamel.
DefinitionWHO defines AF and related lesions as neoplasms composed of
proliferating

odontogenic

epithelium

embedded

in

cellular

Ectomesenchymal tissue that resembles the dental papilla, and with

varying degrees of inductive change and dental hard tissue formation.
HistoryFirst reported by Kruse in 1891.
Types1. Intraosseous
2. Extraosseous
PathogenesisArises denovo during odontogenesis result of overproduction of the
basal lamina without odontogenic differentiation.
Clinical featuresFrequency 1% - 2% of all odontogenic tumours.
Age predominantly in children and young adults with an age range of 6
months to 42 years (average 14. 6 15.5 years).
Sex no sex predilection (Tradahl).
preponderance
dominance.

(Slootweg).

Some

Some authors have reported male

authors

have

reported

female

Site mandible most commonly affected.

Most frequently in the I

permanent molar and II primary molar region. (80%). Rarely in the posterior
maxilla and anterior regions of the jaws.
Clinical presentation
Asymptomatic.

20 % of the lesions discovered on routine

radiography.
Painless, slow-growing and expansile neoplasm.
Exhibits slower clinical growth than Ameloblastoma and does not
tend to infiltrate bone.
Enlarges by gradual expansion periphery of the lesion often
remains smooth.
Initial presentation pain, tenderness or mild swelling of the jaw,
discharge, and failure of teeth to erupt.
Associated with an impacted tooth 75%).
Radiographic features no pathognomonic radiographic features.
a. Appears as a large, expansile pericoronal radiolucency and is
well defined, unilocular / multilocular with a smooth, welldefined outline and often with a sclerotic opaque border.
b. Small

lesions

are

typically

unilocular,

whereas

large

mandibular lesions are multilocular. They may range in size

from 1-8cm in diameter.
c. Associated with unerupted teeth / impacted tooth.
d. May be completely with in the bone or perforate the cortex.

e. Root resorption and inferior displacement of the mandibular

canal may be seen.
Histologic featuresI.

Macroscopic featuresAppears as a solid, soft tissue mass with a smooth

surface and often exhibits lobulated configuration.

well-defined capsule may not be present.

II.

Microscopic features
A. Ectodermal portion

Proliferating islands, cords and strands of

odontogenic epithelium with a peripheral layer
of cuboidal / columnar cells and central area
resembles the Stellate reticulum of embryonic
enamel organ.

(Tumour islands are found

opening).

Mitotic activity uncomm9on.

Epithelial cells may be rounded / cuboidal.

arranged in slender strands.

Amount and density of epithelial component

may vary from area to area.

Cystic degeneration usually not seen.

B. Ectomesenchymal portion-

1. Embryonic, cell-rich mesenchyme that mimics the

dental papilla.
2. The cells are rounded / angular and are fibroblast
like, with little collagen.
3. Degree of cellularity varies.
4. A cell free zone / zone of hyalinization may be
formed around the epithelial connective tissue
interface ultrastructural examination.
5. Degrees of thickening of lamina densa by
granulofilamentous material.
III. Immunohistochemistry Odontogenic epithelial cells positive for cytokeratins
Immature dental-papilla-like mesenchymal tissue positive for
tenascin.
Some areas of dental papilla-like cells and in the basement
membrane positive for vimentin of epithelium.
Collagen IV predominantly found, collagen I and protocollagen III
were found less.
Unduliln not detected.
Proliferating cell nuclear antigen (PCNA). + Rarely encountered
slow growing nature.
MIB

expression

fibrosarcoma.

recurrent

AF

and

Ameloblastic

Growth pattern of AF occurs usually by smooth expansion within the jaws

cortical bone thinning and occasional resorption.
Surgical description finger like projections of the tumour extend into the
bone
TreatmentAF has been managed by a conservative surgical approach unless the size
of the lesion has dictated the anatomic need for resection procedures.
(i)

Most, if not all, recurrences probably result from incomplete

excision.

A conservative first approach to management is

acceptable if the lesion is encapsulated.

(ii)

Presence / absence of the encapsulation should be established

during the first stages of the surgical procedure.

Despite

encapsulation, some lesions may have loculi of the tumour that

escape detection and excision.
(iii)

Enucleation and curettage result in successful excision of

unilocular, encapsulated lesions.

Careful histologic study of

multiple sections of different areas detect dysplastic changes.

(iv)

Extensive / multilocular lesions are more definitely managed by

resection with continuity defect or resection with continuity
defect from the outset.

(v)

Huge lesions require resection without continuity defect or

resection with continuity defect and bone graft.
anatomic integrity of the jaws.

To maintain

(vi)

Long term clinical and radiological follow up evaluation is

required.

PrognosisRecurrence rate 43.5 % (Trodahls 1972). 18 % (Zallen et al). Recurrences

are probably regrowth of residual tumour.

45% of Ameloblastic

fibrosarcomas evolve from untreated / incompletely treated AF.

VIII.

AMELOBLASTIC FIBRO ODONTOMA

Acronym- AFO.
IntroductionBenign, mixed odontogenic tumour that appears to be a combination of AF
and forming complex odontoma. The lesion has expansile growth potential
of AF and inductive ability of complex odontoma. AF represented the least
histologically differentiated lesion that evolves from a moderately
differentiated form of AFO to odontoma.

(Cahn and Blum).

Some

investigators believe that AF and AFO are variations of the same process.
This represented a hamartomatous lesion.

DefinitionWHO defines AFO as a lesion similar to AF, but showing inductive

changes that lead to formation of dentin and enamel.
HistoryFirst delineated by Hooker in 1972 and was clearly separated from
Ameloblastic odontoma.

PathogenesisSimilar to AF.
Clinical featuresFrequency 1% -3% of odontogenic tumours.
Age found in I and II decades of life. 98% of tumours occur before the
age of 20 years.
Sex M: F = 3:1
Site mandible = maxilla. Some studies show mandibular preponderance.
Premolar molar region.

Most commonly in the mandibular posterior

region followed by maxillary posterior region.

Clinical presentationUsually asymptomatic.
Exclusively central / intraosseous lesion.
Painless, slow growing, expansile lesion.
Presents with swelling and failure of tooth eruption.
Most AFOs associated with unerupted tooth.

Radiographic features(i)

The tumour is unilocular / multilocular, with well-defined sclerotic

borders and well-defined pericoronal radiolucency.

(ii)

May be seen as mixed radiopaque radiolucent larger extensive

lesions.

(iii)

Associated with unerupted teeth / anodontia / absent teeth.

(iv)

Maxillary lesions may involve entire maxillary sinus and displace

the involved tooth proportionately forts size. Cause more tooth
displacement.

(v)

These lesions are generally not more than 1 2 cm in size.

Histological features(i)

Composed of strands, cords, islands, fingers, and rosettes of

primitive odontogenic columnar or cuboidal epithelial cells,
resembling dental lamina.

(ii)

These epithelial components are distributed in a cell-rich, dental

papilla

like

Ectomesenchymal

stroma

with

delicate

fibrils

interspersed by large primitive fibroblasts.

(iii)

Varying amounts of osteodentin / dentin like material and

occasional enamel matrix present.

(iv)

Odontogenic epithelium adjacent to the enamel matrix seems to

be preameloblast like.

(v)

More calcified lesions exhibits mature dental hard tissues that

resemble rudimentary teeth or a conglomerate of enamel and
dentin.

(vi)

May / may not be well encapsulated.

Differential diagnosisTreatment-

(i)

Noninvasive.

Expansile characteristics encapsulation / well

circumscribed. Conservative excision by enucleation.

(ii)

Concurrent removal of associated unerupted tooth.

(iii)

Bosselated projections / lobular areas of the tumour carefully

removed.

(iv)

Huge lesions resection (resection without continuity defect /

resection with continuity defect).
a. Site
b. Extent
c. Anatomic involvement.

PrognosisRecurrence is probable if residual lesion remains.

Ameloblastic

sarcomatous transformation has been reported.

IX.

AMELOBLASTIC FIBRO-DENTINOMA.

Synonym Dentinoma
Acronym AFD
IntroductionExtremely rare, benign odontogenic mixed tumour and a variant of AF, in
which odontogenic mesenchyme is induced to form dentin or a dentine-like
product by the odontogenic epithelium.

AFD is also considered as an

hamartomas rather than a neoplasm. Some authors consider that AFD is a

variant of AFO.
Clinical features-

Frequency about 35 cases have been reported 1936 1994.

Age ranges from 4 60 years of age. Usual age below 30 years.
Sex slight male predilection noted. (2:1).
Site mandible: maxilla (3:1).
Lesions in children are associated with unerupted / missing deciduous
teeth and are usually found in the anterior jaw. Lesions in adults, involving
permanent teeth, show a preference for the posterior region of the jaw.
Clinical presentation
(i)

Asymptomatic

(ii)

Painless facial swelling / enlargement of the jaws

(iii)

Failure to deciduous / permanent teeth to erupt. brings the

patient to dentist.

Radiographic features(i)

Small / extensive.

(ii)

Unilocular / multilocular.

(iii)

Often with well-defined borders with a thin rim of sclerotic bone.

(iv)

Present as a pericoronal radiolucency with radiopaque flecks

consisting of calcified dentinoid.

(v)

Associated with unerupted deciduous / permanent teeth.

(vi)

Root resorption of the adjacent teeth.

Histological featuresI. Macroscopic features(i)

Can be encapsulated.

(ii)

Described

as

mushroom

like

and

has

white,

rubbery

consistency.
II. Microscopic features1. Epithelial component Strands and islands of odontogenic epithelium proliferation in a
mesenchymal, cellular, dental papilla like connective tissue.
Peripheral epithelial cells induce a dentinoid matrix at interface.
2. Mesenchymal component Mesenchymal tissue may appear hypocellular, eosinophilic, and
hyalinized.
Various stages of inductio9n of dentine may be demonstrated
dentinoid, osteodentin and rarely, tubular dentine.
The dentine may be infrequently and poorly mineralized.
Treatment1. Complete excision of AFD.
2. The form of excision is case dependent enucleation if possible.
Resection without continuity defect / resection with continuity defect if
surgical circumstance requires.
PrognosisRecurrence is not expected. But residual lesion has the capacity to grow.
A long-term follow up is a must.
X. ODONTOAMELOBLASTOMA
Synonyms-

(i)

Ameloblastic odontoma

(ii)

Adamanto odontoma

(iii)

Soft and calcified odontoma

Acronym- OA.
IntroductionRare, controversial epithelial odontogenic neoplasm that is composed of
distinct

areas

of

Ameloblastoma

and

odontoma.

(Composite).

Characterized by peculiar proliferation of tissue of the odontogenic

apparatus

in

an

unrestrained

pattern,

including

complete

morphodifferentiation, as well as apposition and even calcification.

Unusuality of this lesion a relatively undifferentiated neoplastic tissue is
associated with a highly differentiated tissue both may show recurrence.
Definitions1. WHO a very rare neoplasm, which includes odontogenic
ectomesenchyme, in addition to odontogenic epithelium that
resembles an Ameloblastoma both in structure and in behaviour.
Because of the presence of the odontogenic ectomesenchyme,
inductive changes take place leading to formation of dentine and
enamel in parts of tumour.
2. Gorlin and Torsell and Shafer tumour in which there is
simultaneous occurrence of Ameloblastoma and a complex or
compound odontoma with in the same tumour.

3. Regezi and Scuiba rare variant that is essentially an

Ameloblastoma in which there is focal differentiation into an
odontoma.
History
1944 Thoma et al reported 1st case of Odontoameloblastoma.
1953 Frissell and Shafer reported a case, which showed Ameloblastoma
like areas and dental hard tissue but ghost cells were also present.
1980 Labnola et al reported a case with definitive Ameloblastoma with a
hard tissue component.
1986 Gupta and Gupta reported a case with Ameloblastoma like tissue
but hard tissue component.
1991 Kaugars and Zussman
2002 Masqueda Taylor et al reported most recent series of OA.
Pathogenesis
OA and AFO are one entity (Wachter et al).
Clinical features
Frequency very few cases are reported.
Age common in children I and II decades.
Sex male predilection.
Site posterior regions of both the jaws. Mandible > maxilla.
Clinical presentation
1. Slowly expanding lesion of the bone facial asymmetry /
deformity.

2. Central lesion considerable destruction of bone.

3. Mild pain
4. Delayed eruption of teeth.
Radiological features1. Small, large / extensive lesion.
2. Well-circumscribed lesion with central radiopaque mass surrounded
by a radiolucent zone or may contain irregular small / large
radiopaque masses interspersed with radiolucent areas and usually
surrounded by a sclerotic border.
3. No radiographic impression of infiltration.
4. Central destruction of bone with expansion of cortical plates is
prominent.
Histologic features1. Unusual and characteristic
2. Consists of a great variety of cells and tissues in a complex
distribution columnar, squamous and undifferentiated epithelial
cells as well as ameloblasts, enamel and enamel matrix, dentine,
osteodentin, dentinoid and osteoid material, Stellate reticulum like
cells, dental papilla, bone and cementum as well as stromal
connective tissue.
3. Many structures resembling normal / atypical tooth germs may be
found, with / without the presence of calcified dental tissues.

4. An outstanding characteristic presence of sheets of typical

Ameloblastoma, usually basal cell, follicular / plexiform type.
5. Few mitotic figures are present.
Differential diagnosis1. AFO
2. Ameloblastoma
3. Developing odontoma
4. Dentinogenic ghost cell tumour.
TreatmentOA initially treated conservatively, including recurrences extensive
maxillary lesion hemimaxillectomy (LaBriola et al)
Twice recurrent lesion resection without continuity defect (Frissell and
Shafer)
1. Lesions those are primarily radiopaque and circumscribed by
sclerotic bone treated enucleation and careful curettage.
2. Lesions that contain significant areas of radiolucency in addition to
calcified odontomatous masses should be biopsied in several areas.
Histologic assessment determines the presence / absence of a
capsule and the quantity and distribution of Ameloblastic
elements.
If capsule present lesion excised by enucleation and curettage.
If capsule not present lesion excised by enucleation and careful
curettage / at most resection without continuity defect.

 Histologic analysis of the specimen determines whether a further

surgical procedure is indicated. Rarely resection with continuity
defect is justified.
Management depends on clinical features, radiological features and
anatomic involvement.
PrognosisRecurrence rare. Due to growth of residual lesion.
X.

ODONTOMA

IntroductionMost common abnormalities of the jaws. Believed to be a hamartomas.

Odontoma is the end product of the anomalous completion / incompletion
of

tooth

formation

ectomesenchyme.
tumours

by

odontogenic

epithelium

and

odontogenic

WHO classifies odontoma under the category of

containing

odontogenic

epithelium

with

odontogenic

ectomesenchyme, with / without dental hard tissue formation.

History
1866 Broca first coined the term odontome.

Thoma and Goldman

narrowed the term odontoma to include tumours that were composed of

well-differentiated tooth structure.
Definition
Brocas tumour formed by an overgrowth of complete dental tissue.

Shafer and Gorlin tumour that has developed and differentiated enough
to produce enamel and dentin.
Types
Complex odontoma a malformation in which all of the dental
tissues are represented, and individual tissues mainly are well
formed but occur in a disorderly pattern.
Compound odontoma a malformation in which all of the dental
tissues are represented in a more orderly pattern than in the
complex odontoma so that the lesion consists of many tooth
like structures.
PathogenesisComplex odontoma is formed from other mixed odontogenic tumours.
AF / AFD.

I step.

AFO.

II step.

Complex odontoma.

final step.

Compound odontoma is a malformation, which is more differentiated, and

its pathogenesis more closely is related to the creation of supernumerary
teeth, especially mesiodens. This hypothesis is supported strongly by the
preponderance of compound odontomas in the anterior maxilla.
OriginUnknown. Several theories have been proposed.

1. Trauma
2. Infection
3. Inherited / develop as a result of gene mutations.
Clinical featuresFrequency- relatively common in North America.
65% - Regezi.
52 % - Daily et al
Compound

odontomas

move

common

than

complex

odontomas.

Compound odontoma 37 % of odontogenic tumours.

Complex odontoma 30 % - Regezi.
Age occur in II decade of life.
51 % - Regezi mean age 19 years
54 % - Kaugars - mean age 16 years
57 % - Owens mean age 19 years.
Sex slight male preponderance (Budnick). Male = female
Site compound odontoma anterior maxilla.
Complex odontoma posterior mandible. Anterior maxilla.
Associated mostly with permanent teeth.

In deciduous dentition, more

commonly occurs in anterior (incisor canine) region; only 5 cases have

been associated with deciduous molars. More odontomas are located on
the right side.
Clinical presentation
I.

Asymptomatic.

II.

Hard, painless masses.

III.

Small, rarely exceeds the diameter of the associated

impacted tooth.

IV.

Most lesions discovered as an incidental radiographic

finding.

V.

Impacted permanent tooth or a retained deciduous tooth

most common symptom. (61 %).
Swelling 2nd most common symptom common in patient

VI.

with odontomas associated with dentigerous cyst (27.6%).

VII.

Complex odontomas may become large and produce

expansion of bone and facial asymmetry.

VIII.

Persistence of deciduous tooth, non eruption of permanent.

Radiographic featuresa. Almost always diagnostic.

b. Lesion consists of densely opaque masses of varying size,
usually associated with unerupted / impacted teeth.
c. A radiolucent line almost invariably surrounds these opaque
masses.
d. Compound odontoma

Collection of tooth like structures of varying size

and shape but the teeth are diminutive in size.

Encased by a rim of sclerotic bone often.

e. Complex odontoma-

Appear more often as calcified masses that have the

same consistency as tooth structure.

f. Small odontomas often seen between the roots of erupted

teeth.
g. Developing odontomas may show little calcification and
appear only as well circumscribed radiolucencies.
h. Odontomas have a tendency to cause only mild expansion.
Histologic features
a. Contain dentin and at least enamel matrix.
b. Varying amounts of enamel, pulp tissue, enamel organ and
cementum also are found.
c. Connective tissue capsule of an odontoma similar to dental
follicle.
d. Dental tissues have normal histomorphology but are arranged
abnormally.
e. Spherical dystrophic calcifications, enamel concretions and
sheets of dysplastic dentin and cementum are also found.
f. Immature

odontomas

may

lack

all

but

rudimentary

calcifications.
g. Compound odontomaMiniaturized single rooted tooth that are embedded in a
fibrous connective tissue stroma.

The crown composed of

enamel is decalcified completely and appear as a cystic space,

containing only fragments of enamel matrix. Pulp tissue is

often seen in the normal location.
h. Complex odontoma consists of sheets of immature tubular
dentine with encased hallow tooth like structures. Consists
of calcified dental tissues in bizarre combinations.
i. Ghost cells seen often in odontomas, complex odontoma.
Differential diagnosis1. CEOT
2. AFD
3. AFO
4. OA
5. Focal sclerosing osteomyelitis.

Treatment
a. Completely calcified complex / compound odontoma is
biologically inert and need not be removed if the clinical
diagnosis is secure.
b. Conservative surgical excision is the treatment of choice.
c. Several reasons for excision of a lesion1. Patients concern about diagnosis, once informed of lesions
presence.

2. Accommodation of possible eruption of a tooth blocked by

odontoma, in a favourable position.
3. Establishment of a diagnosis between complex odontoma and
other radiopaque lesions.
d. Both compound and complex odontomas approached through
intraoral mucosal incisions and adequate removal of overlying
bone to expose the lesion.
e. Compound odontoma enucleated if capsule is intact /
individual tooth forms are carefully removed if the capsule is
disrupted.
f. Small complex odontoma enucleated
g. Large / huge complex odontomas cut into segments for
removal conserve normal bone and to prevent jaw fracture.
h. Large mandibular odontomas lingual approach to the
removal (Blinder et al).
i. Final choice of treatment 1. Surgeon
2. Numerous factors.
j. Teeth are unerupted combination of surgical and orthodontic
therapy bring the teeth to normal occlusion.
k. Impacted teeth associated with odontoma should be preserved
rather than extraction.
Prognosis
Little to no chance of recurrence.

XII. CALCIFYING ODONTOGENIC CYST AND DENTINOGENIC GHOST CELL

TUMOUR.
Synonym
1. Gorlin cyst
2. Keratinizing and / or calcifying epithelial odontogenic cyst
3. Cystic keratinizing tumour.
4. Calcifying ghost cell odontogenic tumour
5. Cystic calcifying odontogenic tumour.
Acronym CEOC
IntroductionA unique odontogenic lesion that possess characteristic of both cyst and
neoplasm. The issue of cyst v/s tumour or cystic v/s solid is not yet
resolved completely.

History
1932 Rywkind gave the first actual description of the COC and believed
that the lesion is a variant of cholesteatoma.
1940 Thoma and Goldman reported 3 cases with odontogenic tumours of
ectodermal and Mesodermal origin.
1962 COC was first described as a distinct clinicopathogenic entity by
Gorlin.

1963 Gold reported and cases with similar lesion.

He named it as

keratinizing and / or calcifying odontogenic cyst.

1972 Fejerskov and Krogh suggested the term Calcifying ghost cell
odontogenic tumour
1975 Freedman et al suggested the name cystic calcifying odontogenic
tumour.
ClassificationI.

Praetorius et al (1981)-

Type I cystic lesion

Type IA simple cystic type.
Type IB odontome producing type.
Type IC ameloblastomatous proliferating type
Type II neoplasm like lesion.
II.

Hong et al 1991.

Type I cystic lesion.

Type IA nonproliferative COC
Type IB proliferative COC
Type IC - Ameloblastic COC
Type ID combination of nonproliferative COC and an odontome.
Type II neoplastic lesion
Type IIA Ameloblastoma ex COC
Type IIB peripheral epithelial odontogenic ghost cell tumour.
Type IIC central epithelial odontogenic ghost cell tumour.

III.

Li and Yu 2000

PathogenesisArises from the dental lamina and its remnants.

Types
1. Cystic.
2. Neoplastic.
1. Central.
2. Peripheral.
Clinical features
Frequency COC is not a common lesion. Dentinogenic ghost cell tumour
is rare. 34 cases of COC in 43,500 accessions from 1950 1973. From
1974 1984 17 more cases reported.
Age seen in any decades of life. In II and III decades most commonly
seen.
Sex male = female.
Site 70 % mandible > maxilla. Asians shows predilection for the maxilla.
Whites show 62 % disposition in mandible.
Clinical presentation
1. Painless expansile lesion.
2. Routinely discovered on radiographic examination.
3. Peripheral lesions present on gingival as painless swellings /
nodules.
4. No pathognomonic clinical features.

Radiographic featuresa. Present as radiolucencies or radiolucencies with foci of

ossification.
b. Unilocular / multilocular.
c. Lesion may have a regular outline, with well-demarcated
margins or the outline may be irregular, with poorly defined
margins.
d. There may be varying admixtures of focally thickened, thinned
and absent sclerotic margins.
e. Calcifications characteristic resemble tooth like
structures or nonspecific calcified bodies.
f. A cystic / hydraulic effect seen.
g. Can cause resorption of an adjacent root apex.
h. Displacement of erupted and unerupted teeth has been
mentioned.
i. CT and MRI have proven to be useful in diagnosis and
description of radiological features of this lesion. (Erasmus et
al).
Histologic features1. Proliferative cystic lining containing 2 3 cells thick low cuboidal /
squamous cells.
2. Focal areas of stellate reticulum positive.
3. Ghost cells within the proliferative epithelium.

4. Sparse

amounts

of

dentinoids.This

material

if

formed

in

abundance and the lesion is solid.

5. Dystrophic calcifications of the ghost cells seen.
6. Ghost cells are large, pale, eosinophilic, swollen epithelial cells that
contained nuclear remnants, remnants of cytoplasmic organelles
and numerous tonofilaments with often vacuolations.
7. Takata et al demonstrated that ghost cells express enamel related
proteins.
8. Badger

and

Gardner

demonstrated

that

COC

and

craniopharyngiomas showed similar immunoreactivity to low and

high molecular weight cytokeratins and involucrin.
Differential diagnosis1. Odontomas
2. Ameloblastoma
3. AFO
4. AO
TreatmentNot well established due to relative lack of knowledge regarding biologic
behaviour of each variants. COC with / without an associated odontoma
treated by enucleation or through curettage.

Peripheral lesions

conservative excision. Central dentinogenic ghost cell tumour surgical

excision. May require block excision or segmental resection.
Prognosis-

Recurrence following conservative treatment has been reported.

May

transform into odontogenic ghost cell carcinoma.

I.

CEMENTOBLASTOMA

Synonym

Benign cementoblastoma

True cementoma.

IntroductionCementoblastoma is an uncommon, perhaps rare, true neoplasm of

functional cementoblasts, which form a large mass of cementum or
cementum like calcification, fused to a tooth root. Cementoma is a time
honoured designation for connective tissue lesions of the jaws that
produce cementum like or osteocementum like calcifications. The term
cementoma encompasses several pathologic entities that overlap and bear
histologic similarities to each other but should be separated on
clinicopathologic

bases.

Such lesions, presently, fall within the

classification of fibroosseous lesions under the term cemento osseous

dysplasia.

Here only the cementum forming odontogenic tumour is

discussed.
PathogenesisCells of origin are cementoblasts.
Clinical features-

Frequency rare to uncommon lesion. 55 cases have been reported upto

1979.
Age commonly occur in 2nd and 3rd decades of life.
Sex male = female. Some cases with female preponderance have been
reported.
Site mandible > maxilla (3:1).

but cases with mandibular = maxillary

distribution have been reported. Occurs predominantly in premolar and

molar region. I permanent molar is most frequently affected.
Clinical presentation asymptomatic.
1. Slow- growing lesion and growth by expansion.
2. Produces a clinical enlargement of the jaws that
expands and sometimes resorbs, the lateral and
medial cortical bone.
3. Discomfort / pain not uncommon symptom. Pulpitis
like.
4. Palpation of the tooth involved with the lesion or
palpation of the hard lesion underlying the mucosa is
painful.
5. Paresthesia is not present.
6. Affected tooth is vital.
Radiographic featuresa. A single radiodensity in intimate associated with the
root of a tooth of a sound 1-3cm in diameter.

b. Well-circumscribed.
c. Often perilesional radiolucency present.
d. The outline of the affected root generally obliterated
because of resorption of the root and fusion of the
mass to the tooth.
e. May cause expansion of the cortical plates.
f. Central area of the lesion may be uniformly dense /
slightly mottled.
g. Peripheral calcified areas exhibit a radial distribution
of the density.
Histologic featuresa. Composed of sheets of cementum like tissue, sometimes
resembling secondary cellular cementum but other times
resembling giant cementicles, being deposited in a globular
pattern.
b. The dense mass forms a calcified mosaic pattern and
numerous reversal lines present throughout this calcified
tissue and occupies the central areas of the lesion.
c. Entrapped cells are sparsely scattered within the cementum.
d. At the edges of the denser calcified masses, an active vascular
fibroblastic

connective

tissue

stroma

form

large

hyperchromatic cementoblasts that line the osteoid streams of

the calcifying trabeculae.

e. Multinucleated giant cells lie in trabeculae lacunae and appear

to be simultaneously remodeling the trabeculae.
f. The very peripheral trabeculae are not well mineralized and
adjoin a narrow border of fibrous connective tissue that
separates the lesion from the adjacent medullary bone.
g. The lesional areas that fuse directly with the resorbing dentin
of the root replace the periodontal ligament at the junction and
may even invade the pulpal tissue.
Differential diagnosis

Cementoma

Florid osseous dysplasia

Multiple cemento-ossifying fibroma

Gigantiform cementoma.

Cementifying fibroma

Chronic sclerosing osteomyelitis

Osteoblastoma

Osteoid osteoma

Osteosarcoma

TreatmentSmall lesions enucleation.

Larger lesions-segmentation and enucleation.
Extraction of the associated tooth because of the tendency for expansion
of the jaws.

Reports of endodontic preservation of the associated tooth have been

mentioned in the literature.
Complete enucleation and curettage of the surrounding bone sufficient
treatment.
Failure to complete enucleation of the mass postoperative pain.
Prognosis- recurrence not expected.

References

1.

Text book of surgical pathology R. J. Fonseca.

2.

Text book of maxillofacial surgery- peter ward

booth, volume 2

3.

Text book of otolaryngology cummings

4.

Text book of oral and maxillofacial surgery- Daniel

laskin, vol 2

5.

Text book of oral pathology shafers

6. Effectiveness of a New Decisional Algorithm in Managing Mandibular

Ameloblastomas: A 10 Years Experience. British Journal Of Oral &

Maxillofacial Surgery 45(2007): 306-310.