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Pediatric and Developmental Pathology :... 2023Odontogenic tumors are rare tumors of the jaws that arise from remnants of the tooth forming apparatus. Some odontogenic tumors demonstrate strong predilection for... (Review)
Review
Odontogenic tumors are rare tumors of the jaws that arise from remnants of the tooth forming apparatus. Some odontogenic tumors demonstrate strong predilection for pediatric patients including the unicystic ameloblastoma, adenomatoid odontogenic tumor, ameloblastic fibroma, ameloblastic fibro-odontoma, odontoma, and primordial odontogenic tumor. In this review, we discuss the clinical, radiographic, histopathologic, and molecular characteristics of select odontogenic tumors that demonstrate pediatric predilection and review management.
Topics: Humans; Child; Odontogenic Tumors; Ameloblastoma; Odontoma
PubMed: 38032744
DOI: 10.1177/10935266231200115 -
Oral Diseases Oct 2023This systematic review aimed to incorporate published information about synchronous odontogenic tumors (SOTs) with an analysis of the demographic and clinical... (Review)
Review
This systematic review aimed to incorporate published information about synchronous odontogenic tumors (SOTs) with an analysis of the demographic and clinical characteristics from the cases published in the literature. Case reports and case series of SOT were searched in PubMed, Web of Science, Scopus, and EMBASE. A descriptive statistical analysis was performed. Twenty-eight studies comprising 30 cases of SOTs were included. Considering all cases published, SOTs mostly occurred simultaneously in the maxilla and mandible (n = 19/63.3%). Lesions were bifocal in 13 (43.3% of all the 30 cases) and multifocal in 17 cases (56.7% of all the 30 cases). All SOTs available in the literature presented the same type of lesion, and two of them also involved another different SOT (n = 2/6.7% of all the 30 cases). Out of all published cases, the most frequent SOTs in the literature were odontomas (n = 10/33.3% of all the 30 cases), squamous odontogenic tumors (OTs) (n = 8/26.7% of all the 30 cases), calcifying epithelial OTs (n = 8/26.7% of all the 30 cases), and adenomatoid OTs (n = 2/6.7% of all the 30 cases). Considering all SOTs cases included, the overall recurrence was 13.3%. Inside a subgroup of the lesion, synchronous calcifying epithelial OT presented the highest (25%). Five cases (16.7% of all the 30 cases) had a previously associated syndrome, with two cases of Schimmelpenning syndrome being reported. Among published SOTs, odontomas were the most common. All SOTs available in the scientific literature showed the same type of OT and mainly affected both jaws simultaneously. Only a few of these cases were associated with a syndrome.
Topics: Humans; Odontoma; Odontogenic Tumors; Ameloblastoma; Maxilla; Mandible; Syndrome
PubMed: 36218070
DOI: 10.1111/odi.14401 -
Sultan Qaboos University Medical Journal Aug 2023Papilliferous keratoameloblastoma (PKA) is a rare entity, and not much is known about its clinicodemographic features or biological nature. This review aimed to provide... (Review)
Review
Papilliferous keratoameloblastoma (PKA) is a rare entity, and not much is known about its clinicodemographic features or biological nature. This review aimed to provide clarity regarding the characterisation of the demographic, clinical, radiological and histopathological features of PKA. Case reports of PKA were identified through a systematic search across multiple databases. The search yielded a total of 10 cases, half of which were of Indian origin. All the cases invariably occurred in the mandibular posterior region and involved the right side; only one case primarily involved the left side of the mandible. PKA should be considered a variant of the conventional ameloblastoma that is towards the more aggressive end of the spectrum. It tends to occur in older individuals (in their fifth decade or older), with a marked propensity to occur in the right mandibular posterior region. Surgical resection with diligent follow-up is warranted in the treatment of PKA.
Topics: Humans; Aged; Ameloblastoma; Mandible; Thorax
PubMed: 37655071
DOI: 10.18295/squmj.5.2023.021 -
Journal of Oral and Maxillofacial... 2023Odontogenic, non-inflammatory maxillofacial cysts and tumours vary greatly in their ability to grow and cause local tissue destruction. Despite their common embryologic...
INTRODUCTION
Odontogenic, non-inflammatory maxillofacial cysts and tumours vary greatly in their ability to grow and cause local tissue destruction. Despite their common embryologic origin, the biologic mechanisms responsible for this diverse array of clinical behaviour are largely unknown. Unfortunately, even with accurate tissue diagnosis and appropriate surgical management, these tumours have relatively high recurrence rates. While this may be related to surgical technique, it may also be due to intrinsic tumour biology. SOX2 is differentially expressed in odontogenic cysts and tumours, which has an impact over patient prognosis. This could be related to their diverse cells of origin or stages of histogenesis. SOX2 is expressed in OKC and ameloblastoma, and in this study, we look forward to find altered levels and intensity of SOX2 in the above-mentioned lesions.
AIM AND OBJECTIVES
To profile the expression of SOX2 in odontogenic keratocyst (OKC) and ameloblastomaTo compare the intensity of these lesions, analyse their intrinsic feature and predict their recurrence.
MATERIAL AND METHODS
Histopathologically diagnosed cases of OKC and ameloblastoma will be selected ( = 40). Paraffin-embedded, formalin-fixed sections of these lesions will be stained for SOX2 marker using a standard immunohistochemical technique. Positive control will be taken as oral squamous cell carcinoma and negative control will be taken as normal oral mucosa.
RESULTS
A comparison between the stained cell types in odontogenic keratocyst and ameloblastoma revealed statistically significant differences. The immunoreactivity scores of SOX2 were analysed in both groups. The results indicated that 45% of OKC cases exhibited strongly positive reactivity, while 65% of ameloblastoma cases were negative. Statistical analysis demonstrated highly significant differences in the frequency of SOX2 expression between the two groups, with a higher frequency of negative expression in ameloblastoma.
CONCLUSION
Stem cell markers have been observed in these lesions, suggesting the acquisition of stem-like properties by tumour cells, which can affect patient prognosis. Specifically, the marker SOX2 shows differential expression in odontogenic cysts and tumours. High expression of SOX2 in OKC indicates the presence of stem cells with significant self-renewal and proliferative properties, potentially signifying neoplastic behaviour. In contrast, weak or absent expression of SOX2 in ameloblastoma suggests different molecular pathways involved in its neoplastic behaviour.
PubMed: 38304494
DOI: 10.4103/jomfp.jomfp_265_23 -
Journal of Oral Pathology & Medicine :... May 2024Ameloblastoma (AM) is a benign, although aggressive, epithelial odontogenic tumour originating from tooth-forming tissues or remnants. Its aetiopathogenesis remains... (Review)
Review
Ameloblastoma (AM) is a benign, although aggressive, epithelial odontogenic tumour originating from tooth-forming tissues or remnants. Its aetiopathogenesis remains unclear; however, molecular analysis techniques have allowed researchers to progress in understanding its genetic basis. The high frequency of BRAF p.V600E as a main driver mutation in AM is well established; nevertheless, it is insufficient to explain its tumourigenesis. In this review, we aimed to integrate the current knowledge about the biology of AM and to describe the main genetic alterations reported, focusing on the findings of large-scale sequencing and gene expression profiling techniques. Current evidence shows that besides BRAF mutation and activation of the MAPK pathway, alterations in Hedgehog and Wnt/β-catenin pathway-related genes are also involved in AM pathogenesis. Recently, a tumour suppressor gene, KMT2D, has been reported as mutated by different research groups. The biological impact of these mutations in the pathogenesis of AM has yet to be elucidated. Further studies are needed to clarify the impact of these findings in the identification of novel biomarkers that could be useful for diagnosing, classifying, and molecular targeting this neoplasm.
Topics: Ameloblastoma; Humans; Mutation; Proto-Oncogene Proteins B-raf; Jaw Neoplasms; Wnt Signaling Pathway; Hedgehog Proteins; Gene Expression Profiling
PubMed: 38664938
DOI: 10.1111/jop.13538 -
La Revue Du Praticien May 2024
Topics: Humans; Ameloblastoma; Jaw Neoplasms
PubMed: 38833232
DOI: No ID Found -
Journal of Pharmacy & Bioallied Sciences Jul 2023Desmoplastic ameloblastoma (DA) is a rare variant of conventional ameloblastoma. It accounts for only 4%-13% of all ameloblastomas. DA was included in the World Health...
Desmoplastic ameloblastoma (DA) is a rare variant of conventional ameloblastoma. It accounts for only 4%-13% of all ameloblastomas. DA was included in the World Health Organization Classification of Head and Neck Tumors (WHO-2005) as a variant of ameloblastoma with specific clinical, imaging, and histological features. The desmoplastic variant of ameloblastoma usually appears in the anterior and premolar regions as a mixed radiolucent and radiopaque lesion, sometimes resembling a benign fibro-osseous lesion.Ameloblastoma is a locally aggressive tumor that may cause recurrence and in rare cases, malignant transformation with repeated postsurgical recurrences. In this paper, we present a case of a 28-year-old female with swelling in the left upper jaw, a biopsy of which turned out to be DA.
PubMed: 37654261
DOI: 10.4103/jpbs.jpbs_44_23 -
Oral Oncology Nov 2023Although complete excision is the standard of care for ameloblastoma, a subset of recurrent and/or metastasizing ameloblastomas are difficult to treat surgically. Over... (Review)
Review
Although complete excision is the standard of care for ameloblastoma, a subset of recurrent and/or metastasizing ameloblastomas are difficult to treat surgically. Over the past decade, several recurrent mutations in the mitogen-activated protein kinase pathway genes have been identified in ameloblastoma, based on which the efficacy of targeted therapy has been investigated. However, most of the literature has focused on BRAF V600E mutations, the most common oncogenic mutations in ameloblastoma. Hence, this study aims to review the current knowledge of targetable genetic alterations in ameloblastoma from a broader perspective. In addition, the therapeutic potential of immunotherapy for ameloblastoma will be briefly discussed in the context of tumoral PD-L1 expression and the tumor immune microenvironment.
Topics: Humans; Ameloblastoma; Precision Medicine; Jaw Neoplasms; Proto-Oncogene Proteins B-raf; Mutation; Tumor Microenvironment
PubMed: 37816291
DOI: 10.1016/j.oraloncology.2023.106585