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Atlas of Genetics and Cytogenetics

in Oncology and Haematology


OPEN ACCESS JOURNAL AT INIST-CNRS

Solid Tumour Section


Review

Head and Neck: Odontogenic tumor:


Ameloblastoma
Punnya V Angadi
Department of Oral and Maxillofacial Pathology, KLE VK Institute of Dental Sciences and Hospital,
Nehrunagar, Belguam-590010, Karnataka state, India (PVA)

Published in Atlas Database: May 2010


Online updated version : http://AtlasGeneticsOncology.org/Tumors/AmeloblastomID5945.html
DOI: 10.4267/2042/44972
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence.
2011 Atlas of Genetics and Cytogenetics in Oncology and Haematology

Identity
Alias: Adamantinoma
Note
It is considered to be the most common odontogenic
tumor. It is a tumor of the enamel organ without
formation of enamel. Robinson has defined it as:
Unicentric, nonfunctional, intermittent in growth,
anatomically benign and clinically persistent. The
importance of this tumor lies in its common
occurrence, locally invasive behavior which causes
marked deformity and serious debilitation. They also
demonstrate increased recurrence rate after surgery.

Clinics and pathology


Note
History: Gorlin identifies Cusack as the first person to
identify ameloblastoma in 1827. Falkson gave a
detailed description in 1879. The first histopathologic
description was given by Wedl (1853) who called the
tumor cystosarcoma or cystosarcoma adenoids and
thought that it could have arisen from tooth bud/dental
lamina. Wagstaffe (1871) gave the first histological
drawing. Malassez (1885) introduced the term
"adamantine epithelioma" while Derjinsky (1890)
introduced the term "adamantinoma". However, this
term has become obsolete and has to be avoided. Ivy Figure 1. Mandibular ameloblastoma presenting as swelling
and Churchill in 1930 encouraged the use of the term causing facial asymmetry.
"Ameloblastoma" which is the preferred terminology Embryonic origin
till date.
Ameloblastoma arise from remnants of odontogenic

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Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV

epithelium i.e. from rests of dental lamina. If these rests rarely the ameloblastoma can ulcerate through the
are situated outside the bone in the soft tissues of mucosa.
gingiva or alveolar mucosa may give rise to peripheral Peripheral ameloblastoma usually presents as normal
ameloblastoma. Other possible sources of origin colored, smooth surfaced nodules or enlargements but
include gingival surface epithelium and lining of occasional tumors may present with erythematous or
odontogenic cysts. papillary surface. They are slow growing and cause
little or no bone erosion. Any saucerisation of the
Epidemiology
underlying bone is due to pressure rather than invasion.
Ameloblastoma although rare, is the most common Radiology: Ameloblastoma usually present as a well
odontogenic tumor accounting for 1% of all tumors in defined, multilocular radiolucency with scalloped
head and neck region and around 11% of all border typically described as 'honeycomb' or soap
odontogenic tumors. They usually occur in middle age bubble appearance. However, unicystic ameloblastoma
group i.e. 20-40 years with highest incidence noted in typically presents as a unilocular radiolucency
33 years. They are rare in children (8.7% - 15%). containing an impacted tooth. They typically infiltrate
Maxillary ameloblastoma and extraosseous through the medullary bone, therefore the radiographic
ameloblastoma occur in a slightly older age group margins are not accurate indicators of the extent of
while unicystic ameloblastoma (Avg 10.8 years) and involvement. Expansion of buccal and lingual cortical
granular cell ameloblastoma occur in a younger age plates, displacement and root resorption may also be
group. It shows equal sex predilection with no specific seen.
racial predominance. There is conflicting evidence on Desmoplastic ameloblastoma present with a ill-defined
the incidence rates in different races. Although some radiolucent radiopaque lesion reminiscent of a fibro-
reports claim an increased incidence of ameloblastoma osseous lesion.
in black individuals, a large study identifies Asians as
the population with greatest number of affected
patients.
Ameloblastoma occurs in all areas of jaws, but the
mandible is the most commonly affected area i.e. more
than 80% of cases are seen here. Within the mandible,
the molar angle ramus area is involved three times
more commonly than are premolar and anterior regions
combined. In the maxilla, most usually occur in the
molar area, but may be seen occasionally in the anterior
region, maxillary sinus and nasal cavity. Based on the
clinical, radiographic, histopathologic and behavioral
aspects, four subtypes of ameloblastoma are currently
identified namely:
- Classic/solid/multicystic ameloblastoma
- Unicystic ameloblastoma
- Peripheral ameloblastoma
- Desmoplastic ameloblastoma
Clinics
Ameloblastoma grow slowly and usually are
asymptomatic until a swelling is noticed. Most patients
thus typically present with a complaint of swelling and
facial asymmetry. Occasionally, small tumors may be
identified on routine radiography. As the tumor
enlarges, it forms a hard swelling and later may cause
thinning of the cortical bone resulting in an egg shell
crackling which can be elicited. The slow growth also
allows for reactive bone formation leading to gross
enlargement and distortion of the jaw. If the tumor is
neglected, it may perforate the bone and spread into the
soft tissues making excision extremely difficult. Pain Figure 2. A: Ameloblastoma presenting as a multilocular
radiolucency involving the left mandibular angle and ramus
has been reported as an occasional finding which could region. B: Unicystic ameloblastoma presenting as an unilocular
be attributed to secondary infection. Other effects radiolucency associated with an impacted mandibular third
include tooth mobility, displacement of teeth, molar. C: Desmoplastic ameloblastoma depicting a ill-defined
resorption of roots, paraesthesia if the inferior alveolar radiolucency with specks of radiopacities in mandibular anterior
region.
canal is involved, failure of eruption of teeth and very

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Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV

Cytology Desmoplastic ameloblastoma: This variant was first


described in detail by Eversole et al. in 1984 and is
Ameloblatoma cytologically usually presents with
characterized by extensive stromal collage-
basaloid cells arranged in cells and clusters. These
nization or desmoplasia surrounding compressed
basaloid cells have scanty cytoplasm and dense oval
small/irregular islands of odontogenic epithelium
nucleus. Palisading of these basaloid cells in cell
making it a distinct entity. Cyst formation is common
clusters is a noticable feature. Additionally, squamous
and ameloblast like areas are present only in small foci.
cells with abundant cytoplasm and central nucleus can
Calcification in the fibrous stroma and occasional bone
also be seen if there is evidence of squamous
formation can also be seen. Histochemical evaluation
metaplasia. The background is usually eosinophilic and
of the collagen suggests that the dense stroma is not
shows scattered spindle cells and inflammatory cells.
scar tissue but represents active denovo synthesis of
Pathology extracellular matrix proteins. It typically presents
There are six histopathologic subtypes which have been radiographically as a mixed radiolucency and
identified for ameloblastoma i.e. follicular, plexiform, radiopacity mimicking a fibrosseous lesion. In contrast
acanthomatous, basal cell, unicystic and desmoplastic to typical ameloblastoma, this variant frequents the
ameloblastoma. Mixtures of different patterns are maxilla and the anterior region of the jaws.
commonly observed and the lesions are usually Unicystic ameloblastoma: This is considered as an in-
classified based on the predominant pattern present. situ or superficially invasive form of ameloblastoma
Follicular ameloblastoma: These are most readily and consists of a single cyst lined by ameloblastic
recognizable and common type of ameloblastoma epithelium. It presents clinically similar to a
histologically. The follicular pattern consist of islands dentigerous cyst and is usually associated with an
of epithelial cells with a central mass of polyhedral impacted tooth (usually 3rd molars). They are usually
cells or loosely arranged angular cells resembling seen in younger patients with an average age of
stellate reticulum, surrounded by well organized single diagnosis being 22 years and generally involve the
layer of cuboidal or tall columnar cells with nuclei mandible.
placed at the opposite pole of basement membrane This variant histologically presents as a single cyst
resembling pre-ameloblasts. This peripheral cell layer lined by ameloblastomatous epithelium and is divided
tends to show cytoplasmic vacuolization. Cystic into several subgroups based on pattern and extent of
formation is often seen in the center of the epithelial ameloblastomatous proliferation in relation to cyst
islands due to degeneration of stellate reticulum like wall.
cells. Reichart et al. classification modified from Ackermann
Plexiform ameloblastoma: The tumor epithelium is et al.:
arranged in form of network/tabeculae which is bound -1. Luminal UA: Cystic lesion lined by epithelium
by a layer of cuboidal or columnar cells and stellate which exhibits columnar differentiation and reverse
reticulum like areas are usually minimal. Cyst polarization of the basal cell layer. The connective
formation occurs but is usually due to stromal tissue adjacent to the epithelium often exhibits a
degeneration rather than cystic change in the uniform, thin band like area of hyalinization.
epithelium. The stroma consists of loose, vascular -1.2. Luminal and intraluminal UA: Is the simple type
sparsely cellular connective tissue. but exhibits one or more nodules projecting into the
Acanthomatous ameloblastoma: It resembles a lumen. No extension is seen into the surrounding
typical follicular ameloblastoma except it shows connective tissue wall. Occasionally, this form of UA
extensive squamous metaplasia sometimes with keratin can produce an intraluminal plexiform pattern of
formation within the epithelial islands. odontogenic epithelium that lacks typical
Granular cell ameloblastoma: When the central ameloblastomatous differentiation and is called as
stellate reticulum cells show extensive granular cell unicystic plexiform ameloblastoma.
transformation i.e. in the form of sheets of large -1.2.3. Luminal, intraluminal and intramural UA: Here
eosinophilic granular cells, the tumors are referred to as there is occurrence of intramural proliferation of
granular cell ameloblastoma. Sometimes this change, ameloblastoma along with subgroup 1.2 features.
may be so extensive that the peripheral columnar cells -1.3. Luminal and intramural UA: exhibits a cyst with a
may also be replaced making the diagnosis difficult luminal lining in combination with intramural nodules
especially in a small biopsy. Ultrastructurally, it is seen of SMA tissues. The intramural ameloblastoma tissue
that the granules consist of pleomorphic, osmiophilic, may be seen as an infiltration from the cyst lining or as
lysosome like organelles. free islands of follicular ameloblastoma.
Basal cell ameloblastoma: This variant shows Peripheral ameloblastoma: They present
predominant basaloid pattern consisting of darkly histologically with follicular or plexiform pattern as
stained cells with minimal cytoplasm and little well as acanthomatous pattern. In most cases, the tumor
evidence of palisading at the periphery resembling is well separated from the overlying epithelium but
those seen in basal cell carcinoma. many are confluent with the overlying mucosa.

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Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV

Figure 3. Figure 3A: Photomicrograph depicting a typical follicular ameloblastoma with islands demonstrating peripheral columnar cells
exhibiting reversal of polarity and central stellate-reticulum lie cells demonstrating cytic degeneration. Figure 3B and C:
Photomicrographs depicting acanthomatous variant of ameloblastoma depicting follicles exhibiting extensive squamous metaplasia and
keratin formation. Figure 3D: Plexiform ameloblastoma with odontogenic epithelium arranged in the form of interlacing trabeculae and
evidence of stromal degeneration.
Figure 4. Figure 4A and B: Granular cell ameloblastoma: the central stellate reticulum cells show presence of eosinophilic cells with
granular cytoplasm. B: demonstrates aplexiform pattern where the peripheral cells have also been replaced by the granular cells. Figure
4C: Photomicrographs depicting basaloid areas in an ameloblastoma. Figure 4D: Desmoplastic ameloblastoma: Photomicrographs
depicting compressed epithelial islands surrounded by dense fibrous stroma with evidence of bone formation.

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Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV

Figure 5. Figure 5A and B: Unicystic ameloblastoma: A: Luminal type and B: Plexiform unicystic ameloblastoma. Figure 5C and D:
Photomicrographs depicting mucous cells in ameloblastoma.

Other rare variants: Clear cell variant which may excision to include 1.5 cm of clinically normal bone.
contain clear PAS positive cells are localized in the Maxillary ameloblastoma are particularly dangerous
stellate reticulum like areas. due to bony architecture and they grow upwards to
Papilliferous keratoameloblastoma show occurrence of involve the sinonasal passages, pterygomandibular
areas of ameloblastoma with keratinisation, tumor fossa, orbit, cranium, and brain. Maxillary
islands with papilliferrous appearance along with cystic ameloblastomas thus warrant radical surgery as close
areas resembling odontogenic keratocysts. proximity to vital structures makes recurrence
Mucous cells can also be demonstrated rarely in potentially dangerous in this area.
ameloblastoma. Reichart et al. reveal that the overall recurrence rate is
around 22% and half of them appear 5 years after the
Treatment
initial surgical management. Lifelong follow up is
The diagnosis has to be confirmed by a biopsy, but strongly recommended. Rare malignant transformation
occasionally in cystic variants it may be made only has also been documented.
after excision. For the luminal and intraluminal variants of unicystic
Ameloblastomas, although benign are relentlessly ameloblastoma, the tumor is usually confined by the
infiltrative. Conventional ameloblastoma infiltrates into fibrous connective tissue wall of the cyst and isusually
surrounding bone and extends beyond the apparent treated with complete enucleation. However, in the
radiographic boundaries seen in plain radiographs. The intramural variants which show invasion into the cyst
rate of recurrence reported varies from 20-90%. wall is thought represent a typical ameloblastoma and
Treatment planning should take into consideration warrants a more aggressive treatment. Long term
tumor size, location, histopathology, and follow up however remains mandatory.
clinical/radiographic presentation. Peripheral ameloblastomas are not aggressive clinically
Treatment with curettage is clearly inadequate and may and can be managed by local excision. A recurrence
leave small islands of tumor within the bone. Marginal rate of 8% has been reported and a further and wider
or en bloc resection is the most widely used form of local excision can be curative.
therapy with many surgeons advocating margins of

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