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Odontogenic Tumors
Odontogenic Tumors
Odontogenic Tumors
Classification
Diagnostic Criteria
Features
Management
Recent Advances
References
Introduction
Tumor or neoplasm is an abnormal new, uncoordinated growth in the body, which results from excessive, autonomous, purposeless
proliferation of cells, which continues its growth, even after cessation of stimuli
Tumors are broadly divided into two groups Benign and Malignant, depending upon their behaviour pattern and cellular structure.
A benign tumor is a mass of cells, tissues/organs resembling those normally present, but arranged atypically and behave abnormally (The term
benign implies mild and non-progressive). It grows slowly and is usually encapsulated and it enlarges by peripheral expansion, pushes away the
adjoining structures and exhibits no metastasis, however, it may be locally aggressive.
A malignant tumor, rapidly infiltrates the surrounding tissues, including vital structures and endangers the life of its host. It also shows
metastasis in the distant parts of the body usually through lymph and blood streams. The malignant tumors may arise as de novo, or often from
their benign precursor.
Introduction
According to WHO - “Odontogenic tumours and tumour-like lesions constitute a group of heterogeneous diseases that range from hamartomatous or
non-neoplastic tissue proliferations to benign neoplasms and finally malignant tumours with metastatic potential. They are derived from epithelial,
ectomesenchymal and/or mesenchymal elements of the tooth-forming apparatus. Odontogenic tumours are rare, some being extremely rare, but can
pose a significant diagnostic and therapeutic challenge”
The tumours are sub - classified into Epithelial origin, Mixed origin and Mesenchymal origin
In 2022 - The only newly defined entity in odontogenic lesions is adenoid ameloblastoma, which is classified under benign epithelial odontogenic
tumors.
A very important change was made that added ‘essential and desirable diagnostic criteria’ for each entity to highlight the features considered
indispensable for diagnosis.
Ectodermal vs Mesenchymal
Dental Papilla
Dental Sac
Classification
The World Health Organization Classification of Odontogenic Lesions - 2022
Ameloblastic fibroma
Odontogenic carcinosarcoma
Dentinogenic ghost cell tumour
Odontogenic sarcomas
Diagnostic Criteria
There is always a possibility of odontogenic lesions developing from these tissues; resulting in the development of malformations,
hamartomas and neoplasms.
A malformation: It is not neoplastic, but it can cause a functional or aesthetic problem, because of its size or anatomical site.
A hamartoma: It is a benign lesion composed of a new overgrowth of mature cells on existing blood vessels. (A lesion resulting from
faulty development of the embryo).
Odontogenic tumors: A group of neoplasm or tumor like malformations arising from cells of odontogenic apparatus or their remnants.
The abnormal tissue in each of these tumors often can be correlated with similar tissue in normal odontogenesis. They are slow-growing
and tend to spread by direct extension and not by metastasis.
Diagnostic Criteria
Soluk-Tekkesin M, Wright JM. The World Health Organization Classification of Odontogenic Lesions: A Summary of the Changes of the 2022 (5th) Edition. Turk Patoloji Derg. 2022;38(2):168-184. doi: 10.5146/tjpath.2022.01573. PMID: 35578902; PMCID: PMC9999699.
Tumors that are composed of Odontogenic epithelium, Mature connective tissue, Absence of odontogenic ectomesenchyme
Features - Ameloblastoma
Other names
Adamantinoma
Adamantoblastoma
Ameloblastomas occur in 3 different clinico-radiographic situations requiring different therapeutic considerations and having different
prognosis.
Stage II: Maximum Tumor size more than 6 cms or invading the Maxillary sinus or orbital floor
Stage III: Tumor invasion of the skull base or metastasis into regional lymph nodes
There is a significant relationship between time to recurrence and tumor stage, with the earliest recurrence in stage III tumors.
Ameloblastoma
Classification according to radiological Features
Solid / Multicystic
Unicystic
Extra-osseous / peripheral
Solid / Multicystic Ameloblastoma
Clinical Features
Radiographic Appearance:
Histological Feature:
Plexiform
Follicular
Acanthomatous
Granular
Desmoplastic
Basal Cell
The follicular type is composed of islands of epithelium which resemble the enamel organ in a mature fibrous connective tissue stoma.
The Acanthomatous type shows evidence of extensive squamous metaplasia with keratin formation in the island of odontogenic epithelium.
Solid / Multicystic Ameloblastoma
In the granular cell type there is transformation of groups of epithelial cells to granular cells;
Clinically aggressive.
Solid / Multicystic Ameloblastoma
The basal cell type is the least common and is composed of uniform basaloid cells with no stellate reticulum.
Solid / Multicystic Ameloblastoma
The plexiform type is composed of long, anastomosing cords or larger sheets of odontogenic epithelium.
Treatment
Simple enucleation
Curettage
En bloc resection.
Marginal resection - most widely used method of treatment with the least recurrences reported (up to 15 %).
Most surgeons advocate a margin of at least 1.0 cm beyond the radiographic limits of the tumor as the tumor often extends beyond the
apparent radiologic/clinical margins.
Solid / Multicystic Ameloblastoma
Prognosis
Ameloblastomas arising in the maxilla are particularly dangerous as it is often difficult in getting adequate margins.
Unicystic Ameloblastoma
Clinical Features
Location:
90% occur in the mandible usually in the
posterior region.
Unicystic Ameloblastoma
Radiographic Appearance
Typically appears as a radiolucency around the crown of an unerupted tooth (most commonly a mandibular third molar).
Unicystic Ameloblastoma
Histologic Features
Lining epithelium
Cytoplasmic vacuolization
Nuclear palisading
Intraluminal/plexiform: the tumor projects from the cystic lining; sometimes resembles the plexiform type of solid/multicystic
ameloblastoma.
Enucleation of the cyst is probably adequate for the luminal and intraluminal/plexiform types.
Treatment of the mural type is controversial with some surgeons believing that local resection is best.
10-20 % recurrence after enucleation and curettage with all unicystic ameloblastomas.
Peripheral Ameloblastoma
These tumors are extra-osseous and therefore occupy the lamina propria underneath the surface epithelium but outside of the bone.
Histologically, these lesions have the same features as the intraosseous forms of the tumor.
Peripheral Ameloblastoma
Clinical Features
Patient Age:
Wide age range but most occur during
middle-age.
Radiographic Appearance:
Although not in bone, a few cases have shown superficial erosion of the alvelolar bone.
Histologic Appearance:
Islands of ameloblastic epithelium are observed in the lamina propria; plexiform and follicular patterns are the most common; in 50% of the
cases the tumor connects with the basal cell layer of the surface epithelium.
Peripheral Ameloblastoma
Unlike its intraosseous counterpart, this tumor has an innocuous clinical behaviour.
Some reports indicate a 25 % recurrence rate but in these cases as second surgical procedure results in cure.
This epithelial tumor has an inductive effect on the odontogenic ectomesenchyme with dentinoid frequently being produced.
Adenomatoid Odontogenic Tumour
2/3rd in maxilla
Adenomatoid Odontogenic Tumour
Clinical Features
Location:
65% of them occur in the maxilla with 65%
occurring in the canine region. 75% of the cases are associated with the crown
of an unerupted tooth. On rare occasion the lesion is extraosseous.
Frequently asymtomatic
Painless expansion
Adenomatoid Odontogenic Tumour
These lesions are frequently asymptomatic and therefore are
discovered upon routine radiographic examination. AOTs may also
block the eruption of a permanent tooth and be discovered when
radiographs are taken to "search for" the unerupted tooth.
Adenomatoid Odontogenic Tumour
Radiographic and Additional Features
AOTs typically appear as pericoronal radiolucencies, which may have radiopaque material («snowflake" calcifications) within the lucency.
Adenomatoid Odontogenic Tumour
Adenomatoid Odontogenic Tumour
Histologic Features
The tumor is composed of spindle-shaped epithelial cells that form sheets, strands or whorled masses with little connective tissue.
The epithelial cells may form rosette-like structures, tubular or duct-like structures may be prominent or absent.
Enucleation is the treatment of choice as the tumor is easily removed from the bone.
Stratum intermedium.
Enamel organ
Dental lamina
Calcifying Epithelial Odontogenic Tumor
Clinical Features
Location: 75 % of the CEOs occur in the mandible with most occurring in the posterior region.
Anterior gingiva
Calcifying Epithelial Odontogenic Tumor
Bony lesions most commonly present as painless, slow-growing swellings.
Radiographic Features
Slightly over 50% of the CEOTs are associated with an unerupted tooth.
Calcifying Epithelial Odontogenic Tumor
Calcifying Epithelial Odontogenic Tumor
Histologic Features
This lesion is typically composed of islands, sheets or strands of polyhedral epithelial cells in a fibrous stroma.
Nuclei show considerable variation with giant nuclei and pleomorphism observed.
Calcifications may be noted as well as amyloid-like material. Liesegang rings also may be present.
Calcifying Epithelial Odontogenic Tumor
Treatment and Prognosis
Conservative local resection is the treatment of choice as these lesions are typically less aggressive than the ameloblastoma.
With this treatment the recurrence rate is approximately 15 % and the overall prognosis is good.
Squamous Odontogenic Tumor
Clinical Features
Location: SOTs occur with about equal frequency in maxilla and mandible. They are more common in the anterior regions of the jaws than in
the posterior. The lesions occur in the alveolar process.
Squamous Odontogenic Tumor
Radiographic Features
SOTs appear as non-specific radiolucent lesions. They may be well-circumscribed or ill-defined. They often appear triangular in shape and
lateral to the tooth root.
Squamous Odontogenic Tumor
Histologic Features
The peripheral cells do not show the characteristic polarization seen in the ameloblastoma.
Squamous Odontogenic Tumor
Treatment and Prognosis
SOTs often present as painless gingival swellings associated with tooth mobility. Approximately 25 % are asymptomatic.
Conservative local excision or curettage appears to be effective treatment and there have only be a few recurrences reported.
Odontogenic Tumors
This type of tumors is composed of proliferating odontogenic epithelium in a cellular ectomesenchyme resembling the dental papilla
Ameloblastic Fibroma
Clinical Features
This true mixed odontogenic tumor is more common in patients in the first and second decades of life with a mean of 14 years.
The tumor is composed of a cell-rich mesenchymal tissue resembling the primitive dental papilla admixed with proliferating odontogenic
epithelium.
Ameloblastic Fibroma
Additional Features, Treatment and Prognosis
• The tumor is often encapsulated with small tumors usually being asymptomatic.
Most ameloblastic fibromas are treated by conservative surgical excision; however, a 20 % recurrence rate has led some surgeons to
recommend a more aggressive approach.
Odontoma
The odontoma is the most common odontogenic tumor.
Complex: this lesion is composed of a conglomerate mass of enamel and dentin, which bears no anatomic resemblance to a tooth.
Odontoma
Clinical Features
Patient Age: Most cases are recognized during the second decade of life with a mean of 14 years.
Location: Some what more common in the maxilla. The compound type is more often in the anterior maxilla while the complex type occurs
more often in the posterior regions of either jaw.
Odontoma
Radiographic Features
The compound type shows apparent tooth shapes while the complex type appears as a uniform opaque mass with no apparent tooth shapes
present.
Odontoma
Odontoma
Additional Features
Most odontomas are small and do not exceed the size of a normal tooth in the region.
However, large ones do occur and these may cause expansion of the jaw.
Most odontomas are asymptomatic and as a result are discovered upon routine radiographic examination.
Odontomas may block the eruption of a permanent tooth and in these cases are often discovered when "searching for" the "missing" tooth
radiographically.
Odontoma
Histologic Features
The compound odontoma is composed of enamel, dentin and cementum arrange in recognizable tooth forms; some enamel matrix may be
retained in immature and hypomineralized specimens.
The complex odontoma is composed of enamel, dentin and cementum but these tissues are arranged in a random manner that bears no
morphological resemblance to a tooth.
Odontoma
Treatment and Prognosis
Odontomas are treated by simple local excision and the prognosis is excellent.
Odontogenic Tumors
Benign mesenchymal odontogenic tumors are lesions derived from the mesenchymal components of the tooth-forming apparatus and are
consequently found within the jawbone
Odontogenic Fibroma
Clinical Features
Patient Age: Age from 9-80 years old with a mean of 40 years.
Well-defined
Unilocular radiolucency
The larger lesions may be associated with localized bony expansion of the jaw or with the loosening of adjacent teeth.
Odontogenic Fibroma
Histologic Features
The simple odontogenic fibroma is composed of stellate fibroblasts arranged in a whorled pattern with fine collagen fibrils and a lot of
ground substance.
Odontogenic epithelium in the form of long strands or isolated nests is present throughout the lesion.
Location: May occur in any area of the jaws but more common in the mandible.
Radiographic Appearance:
Radiolucent lesion often with a multilocular appearance. The borders may be indistinct.
Odontogenic Myxoma
Odontogenic Myxoma
Histologic Features
Stellate
Spindle-shaped
May be confused with a chrondromyxoid fibroma or with myxoid change in an enlarged dental follicle or papilla.
Cementoblastoma
Clinical Features
Patient Age: This lesion is most commonly occurs in the 2nd and 3rd decades.
Location: The cementoblastoma is associated with the roots of posterior teeth and is more common in the mandible than the maxilla.
Radiologic Features:
Radiographically, the lesion appears as an opaque lesion attached to and replacing the root of the involved tooth.
Histologic Features
The lesion is composed of sheets or thick trabeculae of mineralized material with irregularly placed lacunae and prominent basophilic reversal
lines. Multinucleated giant cells are often present.
The cementoblastoma is a slow-growing lesion that may cause local expansion of the jaw.
Treatment
Ameloblastoma
This is followed by KRAS (mostly p.G12R), NRAS, HRAS, FGFR2, and mutations reported in a few BRAF wild- type cases.
frequent KRAS codon 12 (either p.G12V or p.G12R, and in a single case p.G12D)
Recent Advances
Extra Oral Unit
Intraoral Unit
After successful surgery for ameloblastoma, prevention of recurrence has been a long-standing issue. Recently, a machine learning algorithm has
been used to predict recurrence with reliable accuracy. The four most important variables influencing ameloblastoma recurrence were the time
elapsed from treatment, initial surgical treatment, tumor size, and radiographic presentation. These were used in the algorithm to reliably predict
recurrence.
Similarly, Yang et al. have developed a favourable nomogram that accurately predicted the recurrence-free survival of patients with
ameloblastoma based on individual characteristics, which could optimise tailored therapy and follow-up.
Recent Advances
Discovery and elucidation of the activated molecular pathways discussed helps in novel molecular targeted therapies in the management of
ameloblastoma with genetic mutations. The key benefit of molecular targeted therapies is that it can significantly reduce the surgical morbidity
The results showed that the model was able to predict recurrence of ameloblastoma with reliable accuracy. This study provides insights into the
detection of high-risk patient groups to monitor recurrence.
Yang et al in 2021 stated that the recurrence of ameloblastoma was significantly associated with Cortical Bone Perforation, WHO Classification,
and surgical treatment pattern
Recent Advances
helical tomotherapy,
proton beam therapy. Some of these treatment modalities have been combined with surgery and/or chemotherapy. The therapeutic use of adjuvant radiotherapy with or without
chemotherapy for positive margins of recurrent and unresectable ameloblastomas has resulted in mixed outcomes
The drugs which have potential to be used in molecular targeted therapies of ameloblastoma are those which inhibit the functions of mutated BRAF and MEK.
These are vemurafenib and dabrafenib, which inhibit mutated BRAF gene;
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