A rare case report of ameloblastic fibrodentinoma with imaging features in a pediatric patient.

Ameloblastic fibrodentinoma (AFD) is a rare benign odontogenic tumor that resembles an ameloblastic fibroma with dysplastic dentin. This report presents a rare case of mandibular AFD with imaging features in a young patient. Panoramic radiography and computed tomography revealed a well-defined lesion with internal septa and calcified foci, causing inferior displacement of the adjacent molars as well as buccolingual cortical thinning and expansion of the posterior mandible. The lesion was surgically removed via mass excision, and the involved tooth was extracted under general anesthesia. During the 5-year follow-up period, no evidence of recurrence was observed. Radiologic features of AFD typically reveal a moderately to well-defined mixed lesion with varying degrees of radiopacity, reflecting the extent of dentin formation. Radiologists should consider AFD in the differential diagnosis when encountering a multilocular lesion with little dense radiopacity, particularly if it is associated with delayed eruption, impaction, or absence of involved teeth, on radiographic images of young patients.

Spontaneous Eruption of Permanent Teeth That Had Eruption Disturbances After Extirpation of Odontomas: A Report of Two Cases.

Odontomas, often found adjacent to impacted teeth, are tumors of abnormal tissue morphology arising from the tooth germ and are usually asymptomatic. They are often found by accident on X-ray images, and the eruption of permanent teeth is often caused by odontomas. In most cases, the tooth is extracted with the permanent tooth or orthodontic treatment is performed after extraction. However, the criteria are not clear. We encountered two cases of dental eruption in which permanent teeth, which originally seemed to be suitable for orthodontic treatment, spontaneously erupted after odontoma removal. It is necessary to examine the indications and timing of tooth extraction.

Frequency and Demographic Analysis of Odontogenic Tumors in Three Tertiary Institutions: An 11-Year Retrospective Study.

Odontogenic tumors (OTs) are distinct conditions that develop in the jawbones, exhibiting diverse histopathological features and variable clinical behaviors. Unfortunately, the literature on this subject in Saudi Arabia remains sparse, indicating a pressing need for more comprehensive data concerning the frequency, demographics, treatment modalities, and outcomes of OTs. OBJECTIVES: The study aims to evaluate the frequency, demographic features, treatment, and outcomes of OTs across three tertiary medical centers. METHODS AND MATERIAL: OT cases were identified in King Abdulaziz Medical City (KAMC), King Fahad Medical City (KFMC), and Prince Sultan Military Medical City (PSMMC) from January 2010 to December 2021. RESULTS: Ninety-two OT cases were identified from the anatomical pathology laboratories of three tertiary hospitals. KFMC contributed the highest number of cases (43.5%), followed by KAMC (30.4%) and PSMMC (26.1%). The median age of OT patients was 29 years (range: 5-83), with males representing more than half of the patients (56.5%). The mandible was the most frequent site of OT occurrence (72.5%), with ameloblastoma being the predominant OT (63.0%), followed by odontoma (19.5%). Among the treatment modalities, bone resection was employed the most (51.0%), followed by enucleation (25.6%). Notably, 11.5% of OT cases with available follow-up data exhibited recurrence, with ameloblastoma accounting for eight recurrent cases. CONCLUSIONS: Although OTs are relatively common in the jaws, they are rare in anatomical pathology laboratories and the general population. This study contributes valuable insights into the epidemiology characteristics, treatment trends, and recurrence rates of OTs in Saudi Arabia.

Developing Odontoma in the Mandible of an Eight-Year-Old Boy.

Benign mixed odontogenic tumors have been repeatedly classified and reclassified over the past few decades. Odontoma is considered a hamartoma due to its slow growth and non-aggressive nature. We present an interesting case of developing odontoma in an eight-year-old boy. His complaint was a slow-growing swelling in the lower right back tooth region. Clinical examination revealed a carious deciduous second molar. The orthopantomogram revealed a well-defined radiolucency around the unerupted mandibular first premolar and impacted mandibular second premolar. Histopathology revealed an odontogenic epithelial lining overlying myxofibrous stroma with inflammatory cells and calcified structures with few odontogenic rests. Special staining methods including Van Gieson and modified Gallego stains led to the final diagnosis of a developing odontoma.

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.

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.

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.

Immunohistochemical expression of beta-catenin, BMP4 and TGF-beta in odontomas.

Changes in the expression of nuclear ß-catenin are responsible for tumorigenesis. Beta-catenin acts synergistically with the TGF-ß/BMPs pathway. This interaction leads to greater dentin deposition and may explain the differences between distinct tooth morphologies and hamartomas. The aim of this study was to investigate the role of ß-catenin, BMP4 and TGF-ß in the development of odontomas. This cross-sectional, retrospective, immunohistochemical study evaluated 30 compound odontomas, 30 complex odontomas and 17 tooth germs. The results showed that BMP4 and TGF-ß were more immunoexpressed in the ectomesenchyme of complex odontomas (median = 33.7, p < 0.001; median = 76.4, p = 0.002, respectively). Higher immunoexpression of BMP4 and TGF-ß was also observed in the epithelium of tooth germs (median = 2.0, p < 0.001; median = 120.3, p < 0.001, respectively). TGF-ß and BMP4 showed a positive and significant correlation (p < 0.001). Both TGF-ß and BMP4 were positively correlated with nuclear ß-catenin in ectomesenchyme (p = 0.047 and p = 0.023, respectively). Developing teeth exhibited higher concentrations of the proteins studied in odontogenic epithelium, especially during the bud and cap stages. Higher immunoexpression in odontomas occurred mainly in the ectomesenchyme. We therefore suggest that changes in the ectomesenchyme can lead to the development of odontomas.

Maxillary Odontoma Associated With Noonan Syndrome: A Case Report.

Noonan syndrome (NS) is a common congenital syndrome characterized by multiple anomalies commonly observed in children. In this article, we describe a case of a patient with congenital heart disease, severe mitral regurgitation, and Nonaan syndrome presented with left maxillary swelling and pain, which was treated by complete surgical excision of the left maxillary odontoma. Based on this case, we conclude that numerous oral abnormalities may be related to NS and thus necessitate interdisciplinary treatment planning and prompt therapy. The importance of including oral manifestations as a scoring criterion in diagnosing NS cannot be overstated, as the significance of oral findings in NS has largely been overlooked.

A novel APC mutation associated with Gardner syndrome in a Chinese family.

Gardner syndrome (GS) is a specific form of familial adenomatous polyposis (FAP), which manifests as colorectal polyps, multiple osteomas and soft tissue tumors, and in the oral cavity as osteomas of the jaws, odontomas, and abnormal tooth counts. The underlying cause of GS is attributed to mutations in the APC gene. Mutations in this gene disrupt the normal functioning of the protein and lead to the development of GS. To further investigate GS, a family affected by the syndrome was selected from Dongguan, Guangdong Province. The family members underwent a comprehensive survey, which involved collecting clinical data and peripheral venous blood samples. The samples were then used for genetic analysis. Whole exome sequencing (WES) and Sanger sequencing techniques were utilized to screen and identify specific mutation sites in the APC gene. The clinical findings for the GS family included the presence of gastrointestinal polyps and odontomas. After analyzing the genetic sequencing results, a novel mutation site c.4266dupA on the APC gene was found in the patients, which leading to the APC protein truncation. As a result of this study, it is suggested that odontoma may be an early indicator of GS. Additionally, the identification of this novel mutation site in the APC gene expands the known spectrum of genetic mutations associated with the disease. This discovery has significant implications for the early diagnosis of GS, thus enabling timely intervention to reduce the risk of developing colon cancer and other related diseases.

Loss of Autophagy Disrupts Stemness of Ameloblast-Lineage Cells in Aging.

Autophagy is one of the intracellular degradation pathways and maintains cellular homeostasis, regulating the stress response, cell proliferation, and signal transduction. To elucidate the role of autophagy in the maintenance of dental epithelial stem cells and the subsequent enamel formation, we analyzed autophagy-deficient mice in epithelial cells (Atg7f/f;KRT14-Cre mice), focusing on the influence of aging and stress environments. We also performed in vitro cell and organ culture experiments with an autophagy inhibitor. In young Atg7f/f;KRT14-Cre mice, morphological change was not obvious in maxillary incisors, except for the remarkable cell death in the stratum intermedium of the transitional stage. However, under stress conditions of hyperglycemia, the incisor color changed to white in diabetes Atg7f/f;KRT14-Cre mice. Regarding dental epithelial stem cells, the shape of the apical bud region of the incisor became irregular with age, and odontoma was formed in aged Atg7f/f;KRT14-Cre mice. In addition, the shape of apical bud culture cells of Atg7f/f;KRT14-Cre mice became irregular and enlarged atypically, with epigenetic changes during culture, suggesting that autophagy deficiency may induce tumorigenesis in dental epithelial cells. The epigenetic change and upregulation of p21 expression were induced by autophagy inhibition in vivo and in vitro. These findings suggest that autophagy is important for the regulation of stem cell maintenance, proliferation, and differentiation of ameloblast-lineage cells, and an autophagy disorder may induce tumorigenesis in odontogenic epithelial cells.

Diminutive compound odontoma in a child: a case report with emphasis on early detection.

The odontoma is regarded as a hamartomatous process of the jaws. Most are discovered as an incidental radiographic finding, averaging 15 mm in size. This report describes a case of a diminutive odontoma that was surgically removed before the onset of eruptive and pathologic consequences. A compilation of documented complications and syndromes associated with odontomas is also presented.

Ameloblastic fibro­odontoma in the posterior mandible: A case report.

Ameloblastic fibro-odontoma (AFO) is a rare, slow-growing neoplastic lesion classified as a benign, epithelial odontogenic mesenchymal tumor. This tumor exhibits histological features characteristic of both ameloblastic fibromas and complex odontomas. The clinical manifestation of AFO is typically characterized by the asymptomatic enlargement of the jawbones. Radiographically, it presents as a distinct radiolucent region, indicating the presence of radiopaque substances with varying degrees of irregularities in size and morphology. Standard therapeutic intervention involves enucleation. Despite its benign nature, AFO can cause significant morbidity if left untreated. Therefore, prompt diagnosis and appropriate management are essential to ensure optimal patient outcomes. The present study describes the case (clinical presentation and management) of an 18-year-old male patient with an AFO lesion located in the posterior mandible. This particular case was treated with conservative measures involving surgical enucleation along with the extraction of the impacted tooth and the curettage of residual bone.

Epidemiological and CBCT characterizations of odontomas: A retrospective study of 87,590 subjects.

OBJECTIVES: This study aimed to assess the epidemiological and three-dimensional (3D) radiological characterizations of odontomas, as well as the spatial relationship between odontomas and gubernaculum tracts (GT). MATERIALS AND METHODS: We retrieved the cone-beam computed tomography (CBCT) data of 87,590 patients. Dentition, location, type, diameter of the odontomas, width of the dental follicle (DF), the spatial relationship between the odontoma and GT, and the influence on adjacent teeth were evaluated. RESULTS: Significant differences were found in age, dentition, location, Max/Min diameter, width of DF, impaction, retention, and root bending of adjacent teeth among different spatial relationships between the odontoma and GT (all p < 0.05), as well as in age, type and size, absence, impaction, malposition, and retention of adjacent teeth among different locations of odontomas (all p < 0.05). Compared to the odontomas without impaction, those with impaction had larger diameter (p < 0.05 in all directions). This statistically significant association was consistent for odontomas with malposition, while no similar result was observed in the maximum diameter. CONCLUSION: Our findings provide the preliminary data for clinicians to comprehensively understand the incidence, radiographic characterizations and symptoms of odontoma in Chinese population.

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.

Concurrent Odontogenic Keratocyst and Odontoma: Report of an Unusual and Rare Entity.

Hybrid lesions of jaws are rare entities defined as two different lesions co-occurring in the same location, with identical histopathological origin. Ameloblastoma, calcifying cystic odontogenic tumor and odontoma are among the most common lesions that have been reported to combine with other lesions. In this study, a hybrid lesion of odontogenic keratocyst (OKC) and odontoma in the mandible of a forty-five years old male reported. Additional to the rarity of this hybrid lesion, the present case had unique radiologic features, including atypical location and extension of the lesion and profound knife-edge root resorption of the teeth in the area, which was not a common feature for any of the two lesions. The surgical procedure was marsupialization to reduce the size of the lesion. As a result of the surgery, the healing of the surgical wound was uneventful. In addition, careful follow-up for the patient was conducted, which had no recurrence till now (after 15 months).

Ameloblastic Fibro-Odontoma of the Posterior Mandible: A Rare Pathological Entity.

Ameloblastic fibro-odontoma (AFO) is a rare, slow-growing neoplastic lesion classified as a benign, epithelial mixed odontogenic tumor with odontogenic mesenchyme. This tumor demonstrates the histological features characteristic of both ameloblastic fibromas and complex odontomas. The clinical manifestation of AFO is typically characterized by asymptomatic enlargement of the jawbones. Radiographically, it presents as a distinct radiolucent region, indicating the presence of radiopaque substances with varying degrees of irregularities in size and morphology. Standard therapeutic intervention involves enucleation. Despite its benign nature, AFO can cause significant morbidity if left untreated. Therefore, prompt diagnosis and appropriate management are essential to ensure optimal patient outcomes. The following case report details the clinical presentation and management of an 18-year-old male with an AFO lesion located in the posterior mandible. This particular case was treated with conservative measures involving surgical enucleation.

Clinicoradiopathologic Analysis of Odontomas: A Retrospective Study of 242 Cases.

Odontomas are considered hamartomatous lesions and are one of the two most common odontogenic tumors of the jaw. Odontomas are classified as compound or complex. Recently, ameloblastic fibro-odontoma (AFO) and ameloblastic fibro-dentinoma were reclassified as developing odontomas. Though clinically odontomas are usually asymptomatic, they have adverse effects on adjacent teeth such as tooth impaction, delayed eruption, displacement of teeth, over-retention of teeth, and can give rise to odontogenic cysts within the jaw. We sought to evaluate the clinicoradiopathologic presentations of odontomas by collecting and analyzing the clinical, radiographic, and pathologic data of odontomas diagnosed in our institution from 2013 to 2022. Over this 10-year period, there were 242 patients with a histopathological and/or radiographic diagnosis of odontoma. There was no gender predilection and ages ranged from 3 to 101 years (median, 14 years). The second decade of life was the most prevalent (57.4%). There was no jaw predilection; however, the anterior jaw was the most common location. Ninety-four (38.8%) cases presented with clinical findings. The most common finding was tooth impaction (n = 83). Nine (3.7%) cases were histopathologically confirmed to be associated with other lesions such as dentigerous cysts (n = 8) and nasopalatine duct cyst (n = 1). The median age (25 years) of patients diagnosed with odontomas associated with cysts was older than patients with odontomas (14 years) without associated cysts. Compound odontomas were the most common type of odontoma compared to complex and AFOs with 71.4%, 26.6%, and 2%, respectively. The majority of compound odontomas involved the anterior jaw (69.3%) and mandible (54.9%) while the majority of complex odontomas involved the posterior jaw (59.6%) and maxilla (54.7%). The four AFOs were in the posterior jaw and 75% involved the maxilla. The median age (12 years) of patients diagnosed with AFO was the youngest compared to patients diagnosed with compound (13 years) and complex (16 years). In conclusion, we analyzed the clinical, radiographic, and pathologic features of 242 new cases of odontomas. Our study reaffirms that odontomas frequently affect the pediatric population and can disrupt their dentition. Based on the result of this study, our clinical recommendation to prevent problems to adjacent teeth from odontomas is for dentists to be apt in the diagnose of odontomas to ensure that they are surgically removed in a timely manner.

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.