AMELOBLASTOM

A
Name :- Pandey Arpit
content
 Introduction
 Definition
 Etiology
 Classification
 Pathogenesis
 Clinical features
 Radiographic features
 Diagnosis
 Types
 Differential diagnosis (Radiological)
 INTRODUCTION
 First detailed description of ameloblastoma was given by
'Falkson' in 1879. It is an aggressive tumor that appears to
be arising from remnants of dental lamina or dental organs.
It represents 1% of all oral tumors and 11% of odontogenic
tumors. It is the most common epithelial tumor producing
minimal inductive changes.
 There are different names given to this tumor. Term
'adamantine epithelioma' was given by Malassez in 1885.
It was replaced by ameloblastoma in 1934 by Churchill. It
is also called as 'adamantinoma, 'adamantinoblastoma,
'epithelial odontome' and 'multilocular cyst.
 The ameloblastoma is the best known of the epithelial
odontogenic tumors. It is a slow-growing and locally
aggressive epithelial odontogenic tumor. The microscopic
appearance of this tumor includes tumor cells resembling
ameloblasts, with no formation of calcified material.
DEFINITION
 It is a benign, but locally invasive polymorphic neoplasm
consisting of proliferating odontogenic epithelium which is
usually in a follicular or plexiform pattern, lying
in fibrous stroma.
ETIOLOGY
 Irritation: It might be considered as one of the etiological factors, as it often occurs in the
posterior region of the mandible which is most susceptible to irritation
 Infection: Robinson found that 1/3rd of the cases have history of oral infection, extraction of
teeth and injuries to teeth
 Trauma: trauma can be a causative factor for ameloblastoma
 Dietary deficiency: dietary deficiency has been considered to be a possible factor, e.g.
pronounced defect in development of tooth germ as seen in rickets may lead to irregularity in
the ameloblastic layer
 Virus: injection of polyoma viruses and nitrosoureas have been shown to produce
ameloblastoma-like lesion in animals.
CLASSIFICATION

 On pathological basis
 Conventional ameloblastoma
 Unicystic ameloblastoma
 Peripheral ameloblastoma
 Pituitary ameloblastoma
 Adamantinoma of long bones
 ON HISTOLOGICAL TYPE
 Follicular ameloblastoma
 Plexiform ameloblastoma
 Acanthomatous ameloblastoma
 Basal cell ameloblastoma
 Unicystic ameloblastoma
 Plexiform unicystic ameloblastoma
 Granular cell ameloblastoma
 Papilliferous ameloblastoma
 Hemangioameloblastoma
 Desmoplastic ameloblastoma
 Clear cell ameloblastoma
 Dentinoameloblastoma
 Melanoameloblastoma
 Keratoameloblastoma.
 PATHOGENESIS
 The resemblance of the tumor epithelium to the normal enamel organ indicates that
ameloblastoma arises from dental epithelium. The possibilities for its development are as
follows:
 Enamel organ: Due to histological similarities such as its origin, it has been thought that the
tumor growth starts at an early age; i.e. during the period of existence of enamel organ but
most of the patients are middle aged i.e. in a period which is long after regression of the
enamel organ. However, the occasional occurrence of the tumor as a unilocular cystic lesion
surrounding the crown of an unerupted tooth also suggests that in some cases, the enamel
organ may give rise to it
 Cell rests: Cell rests of enamel organ either remnants of dental lamina or remnants of
Hertwig's sheath, i.e. epithelial cell rests of Malassez have the potential of transforming into
ameloblastoma
 Epithelium of odontogenic cysts, i.e. from dentigerous cyst and odontoma: This may be
possible as the epithelium in the wall of the cyst and that of ameloblastoma are derived from
the same embryonic source
 Oral mucosa: Basal cells layer of the oral epithelium of jaws can be the origin. The reason
behind this is that there is communication between the tumors and overlying mucosal
epithelium as seen in peripheral ameloblastoma
 Heterotrophic epithelium: Heterotrophic epithelium in other parts of the body, especially of
pituitary gland can serve as source of origin.
 Clinical Features
 incidence: Ameloblastoma accounts for approximately 1% of all oral tumors and 11% of all
odontogenic tumors
 Sex and race: It has slight predilection for males and often seen in blacks
 Age: Most patients are between 20 and 50 years of age with mean age of discovery being 40
years. The tumor can occur in young children. Unicystic type of ameloblastoma is more
common in the 2nd and 3rd decade and the extraosseous form is more common in the older
age group
 Site: It develops in the molar ramus area (approximately 3/4th of cases) in the mandible and
also occurs in maxilla in third molar area, followed by the maxillary sinus and floor of the
nose. The right side of the mandible is affected slightlymore as compared to the left side
 Preceding factors: Neoplasm is frequently preceded by extraction of teeth, cystectomy and
some other traumatic episodes
CLINICAL FEATURES
 Age and sex distribution: It is common in males and more commonly found in middle age
group
 Site: It is seen on the mandibular gingiva in the molar- premolar region. Some cases also seen
in buccal mucosa and maxillary tuberosity area
 Symptoms: The patient complains of an intraoral mass which is growing in size and
interfering with speech, mastication and esthetics
 Sign: Mass is mildly tender on palpation when ulcerated and bleeding may occur on slight
touch
 Appearance: The lesion appears as nodular swelling on gingiva varying in size from 3 mm to
2 cm in diameter.
 Onset: It begins as a central lesion of the bone which is slowly destructive but tends to expand
bone rather than perforating it
 Symptoms: Patient notices a gradually increasing facial asymmetry. Teeth in involved region
are displaced and become mobile . Pain and paresthesia may occur, if the lesion is pressing
upon a nerve or is secondarily infected

Facial asymmetry in patient

with ameloblastoma
 Extraoral swelling present in patient with ameloblastom
Disfigurement of face due to huge swelling of ameloblastoma
 intraoral picture showing swelling in
Intraoral view of ameloblastoma showing displacement of tooth retromolar area
 Signs: In later stages, the lesion may show ovoid and fusiform enlargement that is hard but
non-tender . If it is left untreated for many years, the expansion may be extremely disfiguring,
fungating and ulcerative type of growth characteristic of carcinoma can be seen
 Eggshell cracking: As tumor enlarges, palpation may elicit a hard sensation or crepitus.
Surrounding bone may become thin, so that fluctuation and 'eggshell cracking' may be elicited
 Maxillary ameloblastoma: Maxillary lesions are more dangerous than mandibular lesions
due to tendency for the former lesion to spread more extensively in the more porous maxillary
bone and possibility of the involvement of the cranial base. It may extend into the paranasal
air sinuses, orbit or the nasopharynx
 Mural ameloblastoma: Ameloblastoma may form from the epithelial lining of dentigerous
cyst and in such cases, it is called as mural ameloblastoma'

Mural ameloblastoma showing huge

expansion
 Spread: This tumor causes expansion of the bone rather than destruction. Ameloblastoma
infiltrates cancellous bone but it is largely confined by the compact bone. It never infiltrates
haversian canal. It infiltrates between the trabeculae of the cancellous bone and hence leaves
them intact for some duration. Hence, the actual margins of the lesion are much greater than
radiological margin which accounts for recurrence rate, if neglected. As the cortical plates of
maxilla are thin, it does not possess an effective barrier to the spread of ameloblastoma into
adjacent vital structures.
Radiographic Features
 Radiodensity: In early stages, there is area of bone destruction which is well-defined and is
indicative of slow growth with hyperostotic borders
 Margins: Outline is smooth, scalloped, well-defined and well corticated. The walls of the
cavity are coarse. In some cases, the margins of the tumor are lobulated
 Internal structure: Usually it is multilocular but may be unilocular . Coarse or fine trabeculae
may be present within the tumor and it is common for the free margins of the tumor to be
devoid of bony covering
 Appearance: There is presence of septa in the lesion. In some cases, number and arrangement
of septa may give the area a 'honeycomb appearance' (numerous small compartments) or a
'soap bubble appearance' (larger compartments) . In advanced stages, perforated cortical plate
may contribute to a multilocular appearance
 Progress: In early stage, ameloblastoma presents as bubble like appearance with fairly large,
round and distinct compartments. As the tumor grows and expands, the compartments may
coalesce and fuse. As the lesion again increases in size, the cortex is expanded and destroyed
 Subclinical lesion: A small subclinical lesion usually presents in radiographs as many small
rounded cavities in the bone having sharply defined and sometimes corticated borders. In
some cases, there are two rounded and well-defined small cavities having good bony cortex; in
the center of which there is a small white dot
 Unicystic variety: In case of mural ameloblastoma, the radiological appearance is like
unicystic variety
 Effect on surrounding structures: The jaws are likely to be enlarged, depending on the
overall size of the tumor. Extensive root resorption may occur. Thickening of membrane,
cloudiness and destruction of walls are the findings when the sinus is involved. Soft tissue
swelling may appear as haziness on the radiograph
 Eggshell of bone: Expansion and thinning of cortical plate occurs leaving thin 'eggshell of
bone. Perforation of bone is a late feature
 CT features: Computed tomography can explain exact nature and dimension of the lesion.
Honeycomb appearance seen in ameloblastoma Ameloblastoma also showing soft tissue swelling in
the radiograph

Unicystic ameloblastoma Mural ameloblastoma showing unilocular radiolucency

Soap bubble appearance seen in ameloblastoma CT scan of ameloblastoma

CT scan of ameloblastoma Unilocular ameloblastoma seen in posterior region of mandible

DIAGNOSIS
 Clinical diagnosis: Swelling in posterior mandible with expansion and eggshell
cracking will give clue to diagnosis
 Radiological diagnosis: Honeycomb appearance, soap bubble appearance seen in
posterior mandible with bony expansion
 Laboratory diagnosis: In biopsy, various types of ameloblastoma can be seen. It can
be follicular (small discrete islands of tumor cells with peripheral layer of cuboidal and
columnar ameloblast-like cells), plexiform type (ameloblasts-like tumor cells arranged in
irregular masses or more frequently, as network of interconnecting strands of cells, each of
which is bounded by layer of columnar cell and between these layers found stellate reticulum-
like cells), acanthomatous type (cells occupying the position of stellate reticulum undergo
squamous metaplasia, with keratin formation in the central portion of tumor islands), granular
(stellate reticulum cells takes coarse granular eosinophilic appearance), basal type (show
baseloid pattern), unicystic (same like dentigerous cyst with ameloblastic changes).
TYPES OF AMELOBLASTOMA
 Peripheral ameloblastoma
 It is also called as 'extraosseous ameloblastoma. It is a tumor
which occurs in the soft tissue outside and overlying the
alveolar bone. It originates from either the surface epithelium
or the remnants of dental lamina.
RADIOLOGICAL FEATURES

 Bone erosion: Sometimes underlying bone may exhibit evidence of

pressure resorption in the form of saucer-shaped depression
beneath the tumor.
Diagnosis
 Clinical diagnosis: Not so specific
 Radiological diagnosis: Saucer-shaped bone depression beneath the tumor mass may
suspect the lesion
 Laboratory diagnosis: Biopsy shows same pattern as seen in intraosseous
ameloblastoma..
 MANAGEMENT
a) Radical & conservative surgical excision.
b) En bloc resection
c) Segmental resection
d) Curettage
e) Chemical & electrocautery
f) chemotherapy
g) radiation.

Surgery: Simple surgical excision of the lesion is treatment of choice. Prognosis of this
tumor is very good
PITUITARY AMELOBLASTOMA
 It is also called as 'craniopharyngioma' or 'Rathke's pouch tumor!.
 Origin: It occurs in anterior lobe, which is of ectodermal origin. The lobe is derived from
Rathke's pouch, an outgrowth of oral ectoderm. The pouch gives rise to craniopharyngeal
duct, which in due course degenerates itself leaving residues of squamous epithelial cells.
From these squamous epithelial cells, ameloblastoma-like tumor develops
 Age: It is most common in childhood and adolescents before the age of 25 years
 Location: It may be located within the sella tursica or commonly in suprasellar space. It grows
as a pseudo- encapsulated mass causing pressure effect and often destroys the pituitary gland
 Symptoms: They are related to destruction of pituitary gland (diabetes insipidus) or to
compression of nearby cranial nerve.
ADAMANTINOMA OF LONG
BONES
 Origin: It is derived from epithelial rests misplaced during the course of development. Trauma
causing implantation of epithelium with subsequent tumor formation
 Site: It occurs in tibia, ulna, femur and occasionally in others bone
 Clinical course: Some lesions behave aggressively and sometimes are metastasizing.
DIFFERENTIAL DIAGNOSIS
(RADIOLOGICAL)
 Small and unilocular ameloblastoma
 Residual cyst: There is history of extraction of the teeth
 Lateral periodontal cyst: Found in incisor, canine and premolar area in maxilla and
ameloblastoma occur in mandibular molar area
 Giant cell granuloma: Anterior to the molar and ameloblastoma occur in molar area
 Traumatic bone cyst: Occurs in mid-twenties and ameloblastoma common in 3rd and 4th
decade
 Primordial cyst: Same as traumatic bone cyst.
 Multilocular ameloblastoma.
 Odontogenic myxoma: History of missing tooth and has a presence of septa that divide the
image into much finer course than those in ameloblastoma.
DESMOPLASTIC
AMELOBLASTOMA
 Eversole et al (1984) were the first to describe the desmoplastic ameloblastoma. This tumor
exhibits differences in the anatomical distribution, histologic appearance and radiographic
findings compared with the classic type of ameloblastoma.
 Desmoplastic ameloblastoma are mainly located in the anterior region of the maxilla ,
maxillary lesion are more insidious than mandibular tumours owing to its proximity to the
vital structure and maxillary sinus
 Radiographically, desmoplastic ameloblastoma may show either a multilocular mixed
radiolucent/radiopaque appearance or multifocal appearance of minute flecks of bone similar
to that seen in benign fibro-osseous lesions.
 Histologically, it shows pronounced desmoplasia containing epithelial islands,
nests and cords.
MANAGEMENT
 Curettage: It is least efficient as it makes recurrence inevitable. Curettage may leave bone that
is invaded by tumor cells
 Intraoral block excision: Excision of a block of bone may be recommended if the
ameloblastoma is small. The segment in which the tumor is contained is removed with a safe
margin of normal bone.
 Extraoral En-block resection: If the lesion is large and horizontal ramus is involved then this
is carried out
 Peripheral osteotomy: It is a procedure which allows complete excision of the tumor, but at the
same time, a part of bone is retained which preserves the continuity of the jaw. The procedure
is based on the observation that cortical inferior border of the horizontal body, the posterior
border of the ascending ramus and condyle are generally not involved by tumor process. These
areas are resistant and strong because of the dense cortical bone. Bone regeneration will
proceed from such areas with considerable restoration of the jaw architecture.
CHEMOTHERAPY
 May be sensitive to platinum agents
 May have role in improvement in non surgical patients
 Eg;
 cyclophosphamide, cisplatin, vinblastine, methotrexate etc
ELECTROCAUTERY
 Desiccation/electro coagulation of the lesion including various amount of surrounding normal
tissues.
 Not been commonly used as primary mode of therapy.
 Much more effective therapy than curettage.
 50% recurrence rate.
 Because of poor results -not been used as a sole method of treatment.
RADIATION THERAPY
 Ameloblastoma is generally radioresistant
 Post operatively in pts with gross/microscopic residual disease
 Recurrent disease
 Pts who are poor candidates of surgery
 Points to remember for types of ameloblastoma

• Falkson in 1879, remnants of dental lamina, multilocular cyst, cell rest of

Malassez, unicystic is common, egg shell crackling, mural ameloblastoma,
honeycomb appearance, soap bubble appearance

• Peripheral ameloblastoma-extraosseous ameloblastoma, males, mandibular

gingiva in premolar molar region, saucer- shaped pressure resorption

• Pituitary ameloblastoma-craniopharyngioma or Rathke'spouch tumor,

anterior lobe, pseudoencapsulated mass,pressure effect
REFERENCES
 Greenberg MS, Glick M. Burkett's Oral Medicine& Diagnoses, 11th ed., Philadelphia P.C
Decker. Inc.2008
 Text book of oral medicine 3rd edition.,ghom
 Daniel .M.Laskin – oral &maxillofacial surgery Volume 2