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Atlas of Genetics and Cytogenetics

in Oncology and Haematology

Solid Tumour Section

Head and Neck: Odontogenic tumor:
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 :
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

Alias: Adamantinoma
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
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

Atlas Genet Cytogenet Oncol Haematol. 2011; 15(2) 223

displacement and root resorption may also be group. unicystic ameloblastoma group i.15%).Peripheral ameloblastoma .8 years) and involvement. Within the mandible.e. Based on the clinical. The slow growth also allows for reactive bone formation leading to gross enlargement and distortion of the jaw.Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV epithelium i. displacement of teeth. 15(2) 224 . four subtypes of ameloblastoma are currently identified namely: . small tumors may be identified on routine radiography. containing an impacted tooth.Unicystic ameloblastoma . in black individuals. However. Most patients thus typically present with a complaint of swelling and facial asymmetry.Desmoplastic ameloblastoma Clinics Ameloblastoma grow slowly and usually are asymptomatic until a swelling is noticed. Ameloblastoma occurs in all areas of jaws.7% . gingiva or alveolar mucosa may give rise to peripheral Peripheral ameloblastoma usually presents as normal ameloblastoma. paraesthesia if the inferior alveolar radiolucency with specks of radiopacities in mandibular anterior region. C: Desmoplastic ameloblastoma depicting a ill-defined resorption of roots. Any saucerisation of the Epidemiology underlying bone is due to pressure rather than invasion.Classic/solid/multicystic ameloblastoma . Pain Figure 2. histopathologic and behavioral aspects. papillary surface. In the maxilla. They are slow growing and cause little or no bone erosion. Although some radiolucent radiopaque lesion reminiscent of a fibro- reports claim an increased incidence of ameloblastoma osseous lesion. radiographic. more than 80% of cases are seen here. A: Ameloblastoma presenting as a multilocular radiolucency involving the left mandibular angle and ramus has been reported as an occasional finding which could region. most usually occur in the molar area. If the tumor is neglected. racial predominance. They are rare in children (8. smooth surfaced nodules or enlargements but include gingival surface epithelium and lining of occasional tumors may present with erythematous or odontogenic cysts. There is conflicting evidence on Desmoplastic ameloblastoma present with a ill-defined the incidence rates in different races. B: Unicystic ameloblastoma presenting as an unilocular be attributed to secondary infection.e. is the most common Radiology: Ameloblastoma usually present as a well odontogenic tumor accounting for 1% of all tumors in defined. 20-40 years with highest incidence noted in typically presents as a unilocular radiolucency 33 years. failure of eruption of teeth and very Atlas Genet Cytogenet Oncol Haematol. but the mandible is the most commonly affected area i. molar. but may be seen occasionally in the anterior region. Ameloblastoma although rare. They typically infiltrate Maxillary ameloblastoma and extraosseous through the medullary bone. Other effects radiolucency associated with an impacted mandibular third include tooth mobility. If these rests rarely the ameloblastoma can ulcerate through the are situated outside the bone in the soft tissues of mucosa. Other possible sources of origin colored. maxillary sinus and nasal cavity. As the tumor enlarges. Occasionally.e. They usually occur in middle age bubble appearance. it may perforate the bone and spread into the soft tissues making excision extremely difficult. the molar angle ramus area is involved three times more commonly than are premolar and anterior regions combined. 2011. 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. it forms a hard swelling and later may cause thinning of the cortical bone resulting in an egg shell crackling which can be elicited. a large study identifies Asians as the population with greatest number of affected patients. multilocular radiolucency with scalloped head and neck region and around 11% of all border typically described as 'honeycomb' or soap odontogenic tumors. canal is involved. from rests of dental lamina. Expansion of buccal and lingual cortical granular cell ameloblastoma occur in a younger age plates. It shows equal sex predilection with no specific seen.

Luminal and intraluminal UA: Is the simple type sparsely cellular connective tissue.3.e. Mixtures of different patterns are maxilla and the anterior region of the jaws. unicystic and desmoplastic to typical ameloblastoma. Histochemical evaluation metaplasia. classification modified from Ackermann Plexiform ameloblastoma: The tumor epithelium is et al.2 features. Palisading of these basaloid cells in cell making it a distinct entity. many are confluent with the overlying mucosa. this variant frequents the ameloblastoma. commonly observed and the lesions are usually Unicystic ameloblastoma: This is considered as an in- classified based on the predominant pattern present. transformation i. Luminal. 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. These nization or desmoplasia surrounding compressed basaloid cells have scanty cytoplasm and dense oval small/irregular islands of odontogenic epithelium nucleus. plexiform. 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. Cyst polarization of the basal cell layer. radiopacity mimicking a fibrosseous lesion.2. Occasionally. The intramural ameloblastoma tissue that the granules consist of pleomorphic. Additionally. surrounded by well organized single diagnosis being 22 years and generally involve the layer of cuboidal or tall columnar cells with nuclei mandible. 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. osmiophilic.2. thin band like area of hyalinization. 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. placed at the opposite pole of basement membrane This variant histologically presents as a single cyst resembling pre-ameloblasts. Sometimes this change. this form of UA extensive squamous metaplasia sometimes with keratin can produce an intraluminal plexiform pattern of formation within the epithelial islands. Reichart et al. In most cases. it is seen of SMA tissues. 2011. intraluminal and intramural UA: Here eosinophilic granular cells. Atlas Genet Cytogenet Oncol Haematol. ameloblastoma along with subgroup 1. Cyst formation is common clusters is a noticable feature. the tumor evidence of palisading at the periphery resembling is well separated from the overlying epithelium but those seen in basal cell carcinoma. may be seen as an infiltration from the cyst lining or as lysosome like organelles. in 1984 and is Ameloblatoma cytologically usually presents with characterized by extensive stromal collage- basaloid cells arranged in cells and clusters. This peripheral cell layer lined by ameloblastomatous epithelium and is divided tends to show cytoplasmic vacuolization. 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. Ultrastructurally. scar tissue but represents active denovo synthesis of Pathology extracellular matrix proteins. It presents clinically similar to a histologically.e. epithelium. 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. in the form of sheets of large -1. The stroma consists of loose. cells. 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). free islands of follicular ameloblastoma. 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.: arranged in form of network/tabeculae which is bound -1. basal cell. squamous and ameloblast like areas are present only in small foci. The background is usually eosinophilic and of the collagen suggests that the dense stroma is not shows scattered spindle cells and inflammatory cells. In contrast acanthomatous. No extension is seen into the surrounding typical follicular ameloblastoma except it shows connective tissue wall.3. may be so extensive that the peripheral columnar cells -1. vascular -1. the tumors are referred to as there is occurrence of intramural proliferation of granular cell ameloblastoma. They are usually cells or loosely arranged angular cells resembling seen in younger patients with an average age of stellate reticulum. 15(2) 225 . but exhibits one or more nodules projecting into the Acanthomatous ameloblastoma: It resembles a lumen.Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV Cytology Desmoplastic ameloblastoma: This variant was first described in detail by Eversole et al. It typically presents There are six histopathologic subtypes which have been radiographically as a mixed radiolucency and identified for ameloblastoma i. 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. follicular.

Figure 3D: Plexiform ameloblastoma with odontogenic epithelium arranged in the form of interlacing trabeculae and evidence of stromal degeneration. 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. Atlas Genet Cytogenet Oncol Haematol. Figure 4A and B: Granular cell ameloblastoma: the central stellate reticulum cells show presence of eosinophilic cells with granular cytoplasm. Figure 4C: Photomicrographs depicting basaloid areas in an ameloblastoma. Figure 3B and C: Photomicrographs depicting acanthomatous variant of ameloblastoma depicting follicles exhibiting extensive squamous metaplasia and keratin formation. B: demonstrates aplexiform pattern where the peripheral cells have also been replaced by the granular cells.Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV Figure 3. Figure 4D: Desmoplastic ameloblastoma: Photomicrographs depicting compressed epithelial islands surrounded by dense fibrous stroma with evidence of bone formation. 2011. Figure 4. 15(2) 226 .

Lifelong follow up is The diagnosis has to be confirmed by a biopsy. tumor fossa. location. proximity to vital structures makes recurrence Mucous cells can also be demonstrated rarely in potentially dangerous in this area. A recurrence leave small islands of tumor within the bone. Conventional ameloblastoma infiltrates into fibrous connective tissue wall of the cyst and isusually surrounding bone and extends beyond the apparent treated with complete enucleation. Rare malignant transformation occasionally in cystic variants it may be made only has also been documented. pterygomandibular areas of ameloblastoma with keratinisation. but strongly recommended. and follow up however remains mandatory. in the radiographic boundaries seen in plain radiographs. wall is thought represent a typical ameloblastoma and Treatment planning should take into consideration warrants a more aggressive treatment.Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV Figure 5. 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. 2011. contain clear PAS positive cells are localized in the Maxillary ameloblastoma are particularly dangerous stellate reticulum like areas. after excision.5 cm of clinically normal bone. cranium. 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. Figure 5A and B: Unicystic ameloblastoma: A: Luminal type and B: Plexiform unicystic ameloblastoma. Figure 5C and D: Photomicrographs depicting mucous cells in ameloblastoma. The intramural variants which show invasion into the cyst rate of recurrence reported varies from 20-90%. due to bony architecture and they grow upwards to Papilliferous keratoameloblastoma show occurrence of involve the sinonasal passages. 15(2) 227 . orbit. Other rare variants: Clear cell variant which may excision to include 1. and brain. clinical/radiographic presentation. Long term tumor size. ameloblastoma. the tumor is usually confined by the infiltrative. Peripheral ameloblastomas are not aggressive clinically Treatment with curettage is clearly inadequate and may and can be managed by local excision. For the luminal and intraluminal variants of unicystic Ameloblastomas. although benign are relentlessly ameloblastoma. Maxillary islands with papilliferrous appearance along with cystic ameloblastomas thus warrant radical surgery as close areas resembling odontogenic keratocysts. histopathology. However. therapy with many surgeons advocating margins of Atlas Genet Cytogenet Oncol Haematol.

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Lin S. Head and Neck: Odontogenic tumor: Genes Evol. The expression of This article should be referenced as such: nr0b1. 2009 Feb.Head and Neck: Odontogenic tumor: Ameloblastoma Angadi PV Powers J. 15(2) 229 . McCabe ER. Zhao Y. Dev Angadi PV. the earliest gene in zebrafish tooth development.219(8):419-25 Ameloblastoma. Aberrant beta-catenin expression and adenomatous polyposis coli gene mutation in ameloblastoma and odontogenic carcinoma. 2011. Tilakaratne WM. Kudo Y.45(2):103-8 Atlas Genet Cytogenet Oncol Haematol. 2011. Atlas Genet Cytogenet Oncol Haematol. Oral Oncol. Takata T. Ogawa I. 2009 Aug. Siriwardena BS. 15(2):223-229. is a marker for human tooth and ameloblastoma formation.