The Enigma Unveiled: Expansile Compound-complex Odontoma in the Anterior Maxilla of a Teenager.

Aim and objective: The objective of this case report is to offer insight into an expansive compound-complex odontoma located in the anterior maxilla of a 15-year-old male. The focus is placed on the importance of early detection and the progressive comprehension of odontomas. Background: Odontomas are common odontogenic lesions that are frequently discovered during examinations for delayed tooth eruption. There are two distinct classifications for odontomas-compound odontomas and complex odontomas. With its own each set of characteristics. A timely diagnosis is critical for avoiding complications. Case description: A male individual aged 15 years exhibited an expansive compound-complex odontoma located in the anterior maxilla. The clinical examination showed delayed tooth eruption and asymptomatic swelling. The radiographic images showed a radiopaque mass with tooth-like structures and radiolucent borders affecting the surrounding dentition. A surgical excision procedure was conducted, followed by a subsequent histopathological examination confirming the diagnosis of compound-complex odontoma. The patient continued orthodontic treatment after a 1-year follow-up without recurrence. Clinical significance: This case emphasizes the importance of regular dental exams in detecting odontomas early. This observation also highlights the growing understanding of odontomas as hamartomatous odontogenic malformations and the challenges of diagnosing them clinically. Additional molecular investigations are required to facilitate the classification and elucidation of genetic factors. How to cite this article: Alhazmi YA. The Enigma Unveiled: Expansile Compound-complex Odontoma in the Anterior Maxilla of a Teenager. Int J Clin Pediatr Dent 2024;17(1):82-85.

Giant cementoblastoma involving multiple teeth: A rare case report and review of differential diagnoses.

Cementoblastoma, a rare odontogenic benign tumor characterized by calcified cementum-like deposits produced by cementoblasts fused with the tooth root, represents a minute proportion of all odontogenic tumors, with a prevalence ranging from less than 1% to 6.2%. We present a case of a 19-year-old female experiencing pain, progressive swelling, and facial asymmetry in the left lower region over the 1 year ago. Physical examination revealed diffuse swelling in the left mandibular area, demonstrating tenderness and bony hardness on palpation. Radiographic findings displayed a giant, well-defined, relatively round, radiopaque mass, partially delineated by a thin radiolucent halo, and adhered to the roots of the teeth. Computed Tomography imaging revealed an osteoblastic hyperdense mass with extensive buccal and lingual extension. Sagittal images illustrated the mass's continuity with the root apex of the first molar, accompanied by a well-defined hypodense border. A biopsy confirmed the diagnosis of cementoblastoma, leading to the patient undergoing a left hemimandibulectomy. Given the various periapical radiopaque lesions that serve as potential differential diagnoses for cementoblastoma, the ability of the radiologist to distinguish their imaging characteristics plays a crucial role in determining an accurate diagnosis.

A Calcifying Odontogenic Cyst With Compound Odontoma in the Maxillary Sinus: A Case Report in a Pediatric Patient.

Calcifying odontogenic cysts (COCs) exhibit a diverse clinical course, commonly developing between the second and third decades of life, displaying no gender predilection. A 15-year-old female without medical history was under observation for a mixed lesion in the maxilla associated with an impacted tooth. She presented to the emergency department with sudden onset and worsening swelling of the left midface. Radiographic findings in the panoramic radiograph and a CT scan revealed a well-circumscribed mixed lesion localized in the left maxilla, extending into the left maxillary sinus and reaching the orbital floor. After an intercurrent infection of the cyst, the patient was hospitalized, received intravenous antibiotics, and went for surgical intervention under general anesthesia. Lesions that combine histological characteristics of two or more odontogenic tumors or individual cysts in the same location are called hybrid odontogenic lesions. This type of lesion poses a challenge for both pathologists and surgeons, because of its controversial histogenesis and poorly understood clinical evolution. The most common of these lesions are COCs associated with odontoma. Our case represents an exceptionally rare entity among odontogenic cysts.

Accuracy of machine learning in the diagnosis of odontogenic cysts and tumors: a systematic review and meta-analysis.

BACKGROUND: The recent impact of artificial intelligence in diagnostic services has been enormous. Machine learning tools offer an innovative alternative to diagnose cysts and tumors radiographically that pose certain challenges due to the near similar presentation, anatomical variations, and superimposition. It is crucial that the performance of these models is evaluated for their clinical applicability in diagnosing cysts and tumors. METHODS: A comprehensive literature search was carried out on eminent databases for published studies between January 2015 and December 2022. Studies utilizing machine learning models in the diagnosis of odontogenic cysts or tumors using Orthopantomograms (OPG) or Cone Beam Computed Tomographic images (CBCT) were included. QUADAS-2 tool was used for the assessment of the risk of bias and applicability concerns. Meta-analysis was performed for studies reporting sufficient performance metrics, separately for OPG and CBCT. RESULTS: 16 studies were included for qualitative synthesis including a total of 10,872 odontogenic cysts and tumors. The sensitivity and specificity of machine learning in diagnosing cysts and tumors through OPG were 0.83 (95% CI 0.81-0.85) and 0.82 (95% CI 0.81-0.83) respectively. Studies utilizing CBCT noted a sensitivity of 0.88 (95% CI 0.87-0.88) and specificity of 0.88 (95% CI 0.87-0.89). Highest classification accuracy was 100%, noted for Support Vector Machine classifier. CONCLUSION: The results from the present review favoured machine learning models to be used as a clinical adjunct in the radiographic diagnosis of odontogenic cysts and tumors, provided they undergo robust training with a huge dataset. However, the arduous process, investment, and certain ethical concerns associated with the total dependence on technology must be taken into account. Standardized reporting of outcomes for diagnostic studies utilizing machine learning methods is recommended to ensure homogeneity in assessment criteria, facilitate comparison between different studies, and promote transparency in research findings.

Reticular Myxoid Odontogenic Neoplasm with Novel STRN::ALK Fusion: Report of 2 Cases in 3-Year-Old Males.

Odontogenic tumors represent a collection of entities ranging from hamartomas to destructive benign and malignant neoplasms. Occasionally, pathologists encounter gnathic lesions which clearly exhibit an odontogenic origin but do not fit within the confines of established diagnoses. Here, we describe two such odontogenic tumors, both affecting 3-year-old males. Each case presented as a destructive, radiolucent mandibular lesion composed of mesenchymal cells, some with unique multi-lobed nuclei, frequently arranged in a reticular pattern and supported by a myxoid stroma with focal laminations. Production of odontogenic hard tissues was also seen. Because of their unique microscopic features, both cases were investigated by next-generation sequencing and found to harbor the same STRN::ALK oncogene fusion. To our knowledge, these cases represent the first report of an odontogenic tumor with a STRN::ALK gene rearrangement. We propose the possibility that this neoplasm could be separate from other known odontogenic tumors. Both patients were treated with surgical resection and reconstruction. The prognosis of patients with this entity is currently uncertain but shall become more apparent over time as more cases are identified and followed.

Association of MDM2 Overexpression in Ameloblastomas with MDM2 Amplification and BRAFV600E Expression.

Ameloblastoma is a rare tumor but represents the most common odontogenic neoplasm. It is localized in the jaws and, although it is a benign, slow-growing tumor, it has an aggressive local behavior and high recurrence rate. Therefore, alternative treatment options or complementary to surgery have been evaluated, with the most promising one among them being a targeted therapy with the v-Raf murine sarcoma viral oncogene homologue B (BRAF), as in ameloblastoma the activating mutation V600E in BRAF is common. Studies in other tumors have shown that the synchronous inhibition of BRAF and human murine double minute 2 homologue (MDM2 or HDM2) protein is more effective than BRAF monotherapy, particularly in the presence of wild type p53 (WTp53). To investigate the MDM2 protein expression and gene amplification in ameloblastoma, in association with BRAFV600E and p53 expression. Forty-four cases of ameloblastoma fixed in 10% buffered formalin and embedded in paraffin were examined for MDM2 overexpression and BRAFV600E and p53 expression by immunohistochemistry, and for MDM2 ploidy with fluorescence in situ hybridization. Sixteen of forty-four (36.36%) cases of ameloblastoma showed MDM2 overexpression. Seven of sixteen MDM2-positive ameloblastomas (43.75%) were BRAFV600E positive and fifteen of sixteen MDM2-positive ameloblastomas (93.75%) were p53 negative. All MDM2 overexpressing tumors did not show copy number alterations for MDM2. Overexpression of MDM2 in ameloblastomas is not associated with MDM2 amplification, but most probably with MAPK activation and WTp53 expression. Further verification of those findings could form the basis for the use of MDM2 expression as a marker of MAPK activation in ameloblastomas and the trial of dual BRAF/MDM2 inhibition in the management of MDM2-overexpressing/BRAFV600E-positive/WTp53 ameloblastomas.

METTL1 facilitates ameloblastoma invasive growth via MAPK signaling pathway.

OBJECTIVES: Ameloblastoma (AM), a common odontogenic epithelial tumor, exhibits aggressive growth due to incomplete encapsulation within the jawbone. Postoperative recurrence is a significant concern, closely associated with its invasive nature. We investigate the role of tRNA N-7 methylguanosine (m7G) modification mediated by Methyltransferase-like 1 (METTL1) in AM's invasive growth and prognosis. MATERIALS AND METHODS: METTL1 expression was analyzed in diverse cell lines and clinical AM tissues. Its association with postoperative AM recurrence was examined. Functional experiments included METTL1 gene silencing using shRNA in hTERT-AM cells, assessing cell proliferation, migration, and invasion. Xenograft tumor model was constructed to investigate tumor growth. Molecular mechanisms behind METTL1's role in AM invasiveness were elucidated using Ribosome nascent-chain complex-bound mRNA sequencing (RNC-seq) and experimental analysis. RESULTS: High METTL1 expression was significantly associated with postoperative recurrence in AM. The inhibition of AM development following METTL1 knockdown has been corroborated by experiments conducted both in vitro and in vivo. Analysis of RNC-seq data revealed that downregulated genes were predominantly enriched in the mitogen-activated protein kinase (MAPK) signaling pathway, suggesting that METTL1 may promote AM's invasive growth through the MAPK signaling pathway. CONCLUSION: Our study elucidates the functional role of METTL1 in AM's invasive development and prognosis. High METTL1 expression is linked to postoperative recurrence, and METTL1 appears to promote AM invasiveness through the MAPK signaling pathway. These findings contribute to a better understanding of AM pathogenesis and may guide future therapeutic strategies.

Clear Cell Odontogenic Carcinoma: A Rare JAW Tumor-A Case Report and Literature Review.

Clear cell odontogenic carcinoma (CCOC) is a rare epithelial malignant odontogenic tumor of the jaw with a distinct histology and deceptive behavior. As the clinical presentation is often misleading, the contribution of biopsy, molecular biology, immunohistochemistry and cytogenetics are essential in the diagnostic process. This article describes the presentation of an aymptomatic, painless swelling in the lower jaw of a 63 yrs old female, who was initially diagnosed as an ameloblastoma, taken up for segmental resection, subsequently histopathologically diagnosed as a case of clear cell odontogenic carcinoma and later managed with chemotherapy. Current management protocols and presentation of CCOC have also been reviewed.

Does YAP influence cell proliferation and apoptosis in benign epithelial odontogenic lesions?

OBJECTIVE: To analyze the immunohistochemical expression of YAP and its correlation with markers involved in cell proliferation and apoptosis in benign epithelial odontogenic lesions. STUDY DESIGN: The sample consisted of 95 cases of odontogenic lesions (25 dentigerous cysts, 30 non-syndromic odontogenic keratocysts, 30 conventional ameloblastomas, and 10 unicystic ameloblastomas) and 10 dental follicles used as normal odontogenic tissue. The histological sections were submitted to immunohistochemistry with YAP, cyclin D1, Ki-67, and Bcl-2 antibodies. Immunoexpression was analyzed qualitatively and quantitatively using an adapted method. The collected data were analyzed descriptively and statistically (p ≤ 0.05). RESULTS: The highest YAP expression was observed in odontogenic keratocysts, followed by unicystic ameloblastomas and conventional ameloblastomas, which exhibited moderate immunoreactivity predominantly in peripheral cells. Furthermore, significant differences in YAP immunoexpression were observed between the groups analyzed, with significant positive correlations between YAP and cyclin D1 in dentigerous cysts and unicystic ameloblastomas and between YAP and Ki-67 in unicystic ameloblastomas (p < 0.05). However, there were no statistically significant correlations between YAP and Bcl-2 immunoexpression in the groups studied. CONCLUSION: YAP may influence epithelial cell proliferation in odontogenic cysts and tumors, suggesting its possible participation in the progression of the odontogenic lesions studied.

Pigmented dentinogenic ghost cell tumor: a unique case report and a review of the literature.

Dentinogenic ghost cell tumors are rare tumors, and few cases of them were reported in the literature. The presence of pigment in odontogenic lesions is a rare unexplained histological finding. In this report, we describe a unique case of a 7-year-old girl that was referred to the Department of Oral and Maxillofacial Surgery complaining of a left mandibular swelling. Clinical examination revealed a huge, ulcerated mass. Both incisional and excisional biopsies revealed a benign infiltrative odontogenic tumor with admixed ameloblast-like cells and pigmented ghost cells, consistent with a pigmented dentinogenic ghost cell tumor. To the best of our knowledge, this is the youngest case of intraosseous dentinogenic ghost cell tumor reported in the English literature and the second report of a pigmented variant. This rare variant should be included in the differential of pigmented odontogenic lesions to avoid misinterpretation, especially in small biopsies.

Cysts and Benign Odontogenic Tumors of the Jaws.

This article addresses jaw lesions including cysts and benign odontogenic tumors in terms of their definition and clinical and imaging features and discusses pertinent differential diagnoses..

Growth rate per year of a soft tissue recurrent ameloblastoma.

Clinical Key Message: We present a case of recurring ameloblastoma in soft tissue, for which we have estimated the growth rate of the lesion. This information could help clinicians to establish follow-up protocols for the early diagnosis of recurrent ameloblastomas. Abstract: In the present paper, we present a case of recurring ameloblastoma in soft tissue, for which we have estimated the growth rate of the lesion. The area of the whole resected specimen was measured using the ImageJ guide for complex object area. After dividing the area of the recurrent tumor by the number of years during the follow-up, we found that the lesion growth rate was 5.3 cm2 per year. Although further studies are still necessary in the literature to assess the growth rate of ameloblastoma, the present report shows a different methodology to estimate it. This information could help clinicians to establish follow-up protocols for the early diagnosis of recurrent ameloblastomas.

Epidemiological and Clinicopathological Analysis of Odontogenic Tumors: A 20-Year Study

ABSTRACT Objective: To perform the epidemiological and clinicopathological analyses of odontogenic tumors in Kerman for 20 years. Material and Methods: The present study investigated collected records from pathology departments of the Faculty of Dentistry, Bahonar, and Shafa teaching-medical hospitals for 20 years. Data on odontogenic tumors was recorded based on age, sex, and tumor location in the information forms. The statistical t-test and the Kappa coefficient computer codes were utilized for data analysis. Results: 38 samples of odontogenic tumors were considered in the present study. The mean age of participants was 31.7± 10.3 years. The frequency of tumors was higher in women (63.2%) and in the lower jaw) 78.9%). Among various tumors, ameloblastoma (63.1%) and odontoma (18.4%) were the most common tumors, respectively. The correlation between clinical and histopathologic diagnoses was 71.8% using the kappa coefficient. Conclusion: Ameloblastoma is the most common odontogenic tumor. The incidence of lesions was higher in the mandible, and odontogenic tumors were higher in women. Since the diagnosis of odontogenic tumors is based on radiographic and histologic appearances, clinical physicians and pathologists should collaborate for the definitive diagnosis of the disease.

Prevalence of BRAF p.V600E and Detection Methods in Benign Mixed and Malignant Odontogenic Tumors: A Systematic Review.

BACKGROUND: The BRAF p.V600E genetic variant facilitates the pathogenesis of various tumors by triggering tumor proliferation and progression. The aim of this study was to analyze the prevalence of BRAF p.V600E in benign mixed epithelial and mesenchymal and malignant odontogenic tumors. In addition, we discussed the different detection methods used to assess for aberrant BRAF. METHODS: This systematic review followed the PRISMA guidelines and was registered in Prospero (CRD42023445689). A comprehensive search of the PubMed/MEDLINE, Scopus, Web of Science, and Embase electronic databases was performed to answer the question "What is the prevalence of the BRAF p.V600E mutation in benign mixed and malignant odontogenic tumors?" The methodological quality of the selected studies was assessed using the JBI's Critical Appraisal Tool. RESULTS: Initially, 387 records were identified, but only 11 articles met the inclusion criteria. A total of 70 patients with benign mixed epithelial and mesenchymal odontogenic tumors and 63 with malignant odontogenic tumors were included in the analysis. We found that the BRAF p.V600E mutation had a prevalence of 31.42% in mixed tumors and 26.98% in malignant odontogenic tumors. Moreover, immunohistochemistry showed high concordance with DNA-based molecular methods. CONCLUSION: In general, the BRAF p.V600E variant exhibited a prominent prevalence in mixed and malignant odontogenic tumors. However, most of the findings are based on small cohorts of patients and further studies with larger cohorts are needed.

Fibro-odontoma Ameloblástico: Revisión de la Literatura a Propósito de un Caso

El fibro-odontoma ameloblástico (FOA) es una neoplasia odontogénica benigna poco frecuente que afecta a los huesos maxilares. Posee un componente de tejido epitelial y ectomesénquima, por lo que hasta hace un tiempo era incluido dentro de la clasificación de tumores odontogénicos de origen mixto. Actualmente estas lesiones no están incorporadas en la última clasificación de los tumores odontogénicos y huesos maxilofaciales de la organización mundial de la salud y son consideradas como un odontoma en desarrollo. Clínicamente se presenta con mayor frecuencia en mandíbula y asociado a la falta de erupción de un diente. Presentamos el caso clínico de un niño de 6 años de edad que acudió a nuestro servicio maxilofacial por la no erupción de un diente temporal mandibular. El cuadro clínico y las investigaciones confirmaron la hipótesis diagnóstica de FOA con una impactación del segundo molar temporal inferior izquierdo hacia el margen basilar mandibular y el germen dentario del premolar por sobre la corona del diente retenido.

Ameloblastic fibro-odontoma (AFO) is a rare benign odontogenic neoplasm that affects the maxillary bones. It possesses both an epithelial and ectomesenchymal component, for which it was previously included in the classification of mixed odontogenic tumors. The AFO is currently not included in the latest classification of odontogenic and maxillofacial bone tumors, and is considered a developing odontoma. Clinically, it predominantly manifests in the mandible, in frequent association with the lack of eruption of a tooth. In this article, the authors present a case of a 6 year old boy with the query of an unerupted primary mandibular tooth. Both the clinical examination and the subsequent investigation confirmed the diagnostic hypothesis of an AFO with subsequent impaction of the primary left mandibular second molar, which was displaced against the base of the mandible, and the tooth germ for the left mandibular second premolar positionedover the crown of the retained tooth.

Surgical management of odontogenic myxomas: A case series.

INTRODUCTION: Odontogenic myxoma is a relatively rare bone tumor involving exclusively the jaws. Despite its benign nature, odontogenic myxoma can exhibit aggressive, locally invasiveness and has a high potential of recurrence. Surgical treatment can be conservative of radical. The choice of surgical procedure is controversial, and there are no established guidelines. CASE SERIES PRESENTATION: We present a case series of three cases of odontogenic myxomas. This case series illustrates the variability of the radioclinical presentation of odontogenic myxoma and the resulting surgical management, ranging from simple enucleation to mandibular interruptive resection surgery with free flap reconstruction. DISCUSSION AND CONCLUSION: Through this case series, we highlighted and described decision criteria contributing to treatment choice and summarized this in an algorithm. Radiological tumor characteristics and also patient specific factors such as age need to be considered to make a personalized decision to each patient.

Pediatric Odontogenic Tumors.

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.

Ameloblastic fibro-dentinoma: a rare mixed odontogenic tumor case report with review of literature.

BACKGROUND: Ameloblastic fibro-dentinoma is considered a rare, benign, mixed odontogenic tumor that occurs mainly in the posterior mandible in the 1st-2nd decade of life. Although the clinical behavior of Ameloblastic fibro-dentinoma is similar to that of ameloblastic fibroma, there is a debate about whether Ameloblastic fibro-dentinoma is a developing hamartomatous odontoma or a separate neoplastic odontogenic tumor like ameloblastic fibroma. However, it is important to understand the histopathogenesis of this rare tumor. CASE PRESENTATION: A case report presenting an 11-year-old male child with a swelling in the posterior mandible. Radiographic examination revealed a multilocular lesion with mixed radiodensity related to the impacted lower left second premolar tooth. Incisional biopsy was done, and microscopic examination revealed cords and nests of odontogenic follicles lined by ameloblast-like cells and central stellate reticulum-like cells in the primitive ecto-mesenchymal stroma with areas of dentinoid material and osteodentin. The diagnosis was ameloblastic fibro-dentinoma. Surgical excision of the lesion was done, and the patient was followed up for 1 year without evidence of recurrence. CONCLUSION: Reporting such a rare entity clarifies the debate about its nature and the importance of early diagnosis of lesions that are associated with unerupted teeth showing how it is effective in early management and prognosis.

An interesting case-report of ex-ameloblastic carcinoma.

Ameloblastoma (AM) is considered one of the most common lesions of odontogenic origin. Although it is always considered as benign neoplasm, ameloblastic carcinoma (AC) represents its malignant counterpart. It is characterized by the expansion of jaws, rapid growth, and a perforated cortex with well-defined unilocular/multilocular radiolucent lesions. To confirm the diagnosis of AM and AC is extremely crucial. Immunohistochemistry such as SOX2 and Ki67 plays a significant role in the confirmation of diagnosis. Management of these cases is from surgical excision with radical neck dissection. The prognosis is poor with only 5 years of survival. This review presents an interesting case of ex-AC, in which the patient was diagnosed at the same site with peripheral AM 1 year ago.