Ameloblastoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Ameloblastoma may be classified based on histopathology into six subtypes: follicular, plexiform, acanthomatous, basal cell, granular cell, and desmoplastic. Based on the location, ameloblastoma may be classified into either intra-osseous or extra-osseous.

Classification

Based on the location, ameloblastoma may be classified into the following two subtypes:

  • Intra-osseous.
    • Intraosseous ameloblastoma is locally aggressive.
    • Intraosseous ameloblastoma may include the subtypes such as follicular, plexiform, acanthomatous, unicystic, granular cell, basal cell, or desmoplastic
  • Extra-osseous
    • Extraosseous ameloblastoma may include the subtypes such as follicular, plexiform, or basal cell
    • Extraosseous ameloblastoma is benign
    • Extraosseous ameloblastoma is also known as peripheral ameloblastoma

Based on histology, intraosseous ameloblastoma may be subclassified into two groups which includes the following:

  • Solid/multicystic
    • More commonly reoccur
  • Unicystic.
    • Unlikely to reoccur.
    • Classically found in younger individuals.

Ameloblastoma is divided into three clinicoradiologic groups:

  • Solid or multicystic
    • Solid ameloblastoma is the most common form of the lesion. Approximately 86% of the ameloblastomas are solid. It has a tendency to be more aggressive than the other types and has a incidence of recurrence. Multicystic ameloblastoma can infiltrate into the adjacent tissue and has the ability to recur and even metastasize. Its prevalence is a slightly older age group than the unicystic ameloblastoma. Radiographically, the appearance is generally unilocular or multilocular.
  • Unicystic
    • Unicystic ameloblastoma has a large cystic cavity with luminal, intraluminal or mural proliferation of ameloblastic cells. It is a less aggressive variant and it has a low rate of recurrence although lesions showing mural invasion are an exception and should be treated more agressively. The unicystic ameloblastoma usually appears as a “cystic” lesion with either an intraluminal or an intramural proliferation of the cystic lining.[7] Radiographically, it may resemble a well-circumscribed slow-growing radiolucency.
  • Peripheral.
    • Histologically, the peripheral ameloblastoma appears similar to the solid ameloblastoma. It is uncommon, usually presenting as a painless, non-ulcerated sessile or pedunculated gingival lesion on the alveolar ridge. Peripheral ameloblastoma mostly appears in the alveolar mucosa. It is a soft-tissue version of an ameloblastoma but can also involve the underlying bone.
  • Malignant
    • The malignant ameloblastoma is a rare entity. It is defined as an ameloblastoma that has already metastasized but still maintains its classical microscopic features.

The solid ameloblastoma is the most common form of the lesion (86%). It has a tendency to be more aggressive than the other types and has a higher incidence of recurrence. Unicystic ameloblastoma has a large cystic cavity with luminal, intraluminal or mural proliferation of ameloblastic cells. It is a less aggressive variant and it has a low rate of recurrence although lesions showing mural invasion are an exception and should be treated more agressively. Histologically, the peripheral ameloblastoma appears similar to the solid ameloblastoma. It is uncommon, usually presenting as a painless, non-ulcerated sessile or pedunculated gingival lesion on the alveolar ridge. Several histopathologic types of ameloblastoma are described in the literature, including those with plexiform, follicular, unicystic, basal cell, granular cell, clear cell, acanthamatous and desmoplastic patterns

Ameloblastoma is a true neoplasm of enamel organ type. Robinson described it as unicentric, nonfunctional, intermittent in growth, anatomically benign, and clinically persistent. It is the second most common odontogenic neoplasm. Histologically it is of six subtypes: follicular, plexiform, acanthomatous, granular, desmoplastic, and basilar. It affects mandible more than maxilla especially in the region of molar-ramus area. It causes a slow growing, painless expansion of jaw which causes thinning of cortical plates. Root resorption, tooth mobility, and paresthesia are features seen in advanced cases of ameloblastoma. Radiographically it can be unicystic, multicystic, or solid and peripheral type. Multicystic or solid type is prevalent in 86% of cases. Unicystic ameloblastoma is of three subtypes: luminal, intraluminal, and mural

Clinically, ameloblastomas can be classified into 4 groups: unicystic, solid or multicystic, peripheral, and malignant. The unicystic ameloblastoma usually appears as a “cystic” lesion with either an intraluminal or an intramural proliferation of the cystic lining.[7] Radiographically, it may resemble a well-circumscribed slow-growing radiolucency. Multicystic ameloblastoma can infiltrate into the adjacent tissue and has the ability to recur and even metastasize. Its prevalence is a slightly older age group than the unicystic ameloblastoma. Radiographically, the appearance is generally unilocular or multilocular.[8] Peripheral ameloblastoma mostly appears in the alveolar mucosa. It is a soft-tissue version of an ameloblastoma but can also involve the underlying bone.[9] The malignant ameloblastoma is a rare entity. It is defined as an ameloblastoma that has already metastasized but still maintains its classical microscopic features.[10]

A histological classification subdivides into follicular, plexiform, acanthomatous and granular ameloblastoma. Several histopathologic types of ameloblastoma are described in the literature, including those with plexiform, follicular, unicystic, basal cell, granular cell, clear cell, acanthamatous and desmoplastic patterns. Histologically it is of six subtypes: follicular, plexiform, acanthomatous, granular, desmoplastic, and basilar.


The following six histopathologic subtypes have been identified for ameloblastoma:

  • Follicular
  • Plexiform
  • Acanthomatous
  • Granular cell
  • Basal cell
  • Desmoplastic
Subtypes of Ameloblastoma Features
Follicular
  • Histologically, follicular ameloblastoma are the most common type of ameloblastoma.
  • The follicular pattern consist of islands of epithelial cells with a central mass of polyhedral cells or loosely arranged angular cells resembling stellate reticulum, which are surrounded by well organized single layer of cuboidal or tall columnar cells with nuclei placed at the opposite pole of basement membrane resembling pre-ameloblasts.
  • This peripheral cell layer tends to show cytoplasmic vacuolization. Cystic formation is often seen in the center of the epithelial islands due to degeneration of stellate reticulum like cells.
Plexiform
  • The tumor epithelium is arranged in form of tabeculae which is bound by a layer of cuboidal or columnar cells and stellate reticulum like areas are usually minimal.
  • Cyst formation occurs but is usually due to stromal degeneration rather than cystic change in the epithelium. The stroma consists of loose, vascular sparsely cellular connective tissue.
Acanthomatous
  • It resembles a typical follicular ameloblastoma except it shows extensive squamous metaplasia sometimes with keratin formation within the epithelial islands.
Granular cell
  • The tumors are referred to as granular cell ameloblastoma, when the central stellate reticulum cells show extensive granular cell transformation i.e. in the form of sheets of large eosinophilic granular cells.
  • Ultrastructurally, it is seen that the granules consist of pleomorphic, osmiophilic, lysosome like organelles.
Basal cell
  • This variant shows predominant basaloid pattern consisting of darkly stained cells with minimal cytoplasm and little evidence of palisading at the periphery resembling those seen in basal cell carcinoma.
Desmoplastic
  • Desmoplastic ameloblastoma is characterized by extensive stromal collagenization or desmoplasia surrounding compressed small/irregular islands of odontogenic epithelium making it a distinct entity.
  • Cyst formation is common and ameloblast like areas are present only in small foci.
  • Calcification in the fibrous stroma and occasional bone formation can also be seen.
  • Histochemical evaluation of the collagen suggests that the dense stroma is not scar tissue but represents active denovo synthesis of extracellular matrix proteins.
  • It typically presents radiographically as a mixed radiolucency and radiopacity mimicking a fibrosseous lesion.
  • In contrast to typical ameloblastoma, this variant frequents the maxilla and the anterior region of the jaws.
Unicystic
  • Unicystic ameloblastoma is considered as an in-situ or superficially invasive form of ameloblastoma and consists of a single cyst lined by ameloblastic epithelium.
  • It presents clinically similar to a dentigerous cyst and is usually associated with an impacted tooth (usually 3rd molars).
  • They are usually seen in younger patients with an average age of diagnosis being 22 years and generally involve the mandible.
  • It histologically presents as a single cyst lined by ameloblastomatous epithelium and is divided into several subgroups based on pattern and extent of ameloblastomatous proliferation in relation to cyst wall.
  • Unicystic ameloblastoma may be classified into four subtypes based on a classification by Reichart et al. which was modified by Ackermann et al.
    • 1. Luminal unicystic ameloblastoma: It is a cystic lesion lined by epithelium which exhibits columnar differentiation and reverse polarization of the basal cell layer. The connective tissue adjacent to the epithelium often exhibits a uniform, thin band like area of hyalinization.
    • 1.2. Luminal and intraluminal unicystic ameloblastoma: It is the simple type but exhibits one or more nodules projecting into the lumen. No extension is seen into the surrounding connective tissue wall. Occasionally, this form of unicystic ameloblastoma can produce an intraluminal plexiform pattern of odontogenic epithelium that lacks typical ameloblastomatous differentiation and is called as unicystic plexiform ameloblastoma.
    • 1.2.3. Luminal, intraluminal and intramural unicystic ameloblastoma: Here there is occurrence of intramural proliferation of ameloblastoma along with subgroup 1.2 features.
    • 1.3. Luminal and intramural unicystic ameloblastoma: It exhibits a cyst with a luminal lining in combination with intramural nodules of SMA tissues. The intramural ameloblastoma tissue may be seen as an infiltration from the cyst lining or as free islands of follicular ameloblastoma.
Peripheral
  • Peripheral ameloblastoma present histologically with follicular or plexiform pattern as well as acanthomatous pattern. In most cases, the tumor is well separated from the overlying epithelium but many are confluent with the overlying mucosa.
Other rare varients
  • Clear cell variant which may contain clear PAS positive cells are localized in the stellate reticulum like areas.
  • Papilliferous keratoameloblastoma show occurrence of areas of ameloblastoma with keratinisation, tumor islands with papilliferrous appearance along with cystic areas resembling odontogenic keratocysts.
  • Mucous cells can also be demonstrated rarely in ameloblastoma.

References

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