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.

Dentinogenic ghost cell tumor associated with odontoma: report of a rare case and review of literature.

Dentinogenic ghost cell tumor (DGCT) is an uncommon odontogenic tumor considered to be the solid, tumorous variant of calcifying odontogenic cyst. DGCT is characterized by islands of ameloblastoma-like epithelial cells that resemble the enamel organ, the presence of ghost cells, and dentinoid material. This article reports a rare case of dentinogenic ghost cell tumor associated with an odontoma in an adult patient, with a review of the literature. To the best of the authors' knowledge, there have only been four case reports of DGCT associated with odontoma, all of which occurred in children and adults younger than 30 years old.

Timing and treatment sequence in the management of odontomas associated with impacted teeth: A literature review and report of two cases.

AIM: This study aims to highlight the importance of early diagnosis, timing, optimal treatment sequence and multidisciplinary approach as key factors in the orthodontic management of impacted and retained teeth associated with odontomas. METHODS: Literature about classification, epidemiology, aetiopathogenesis, histopathology and therapeutic options about odontomas and impacted teeth in orthodontics was reviewed. Two case reports are presented, showing different timing in diagnosis and surgical removal of odontomas and some biomechanical approaches. CONCLUSION: An early removal of the odontoma is certainly a more effective and simpler procedure in the approach to this problem.

Ameloblastic Fibro-Odontoma in a Free-Ranging Rocky Mountain Bighorn Sheep (Ovis canadensis canadensis), Alberta, Canada.

An adult female Rocky Mountain bighorn sheep (Ovis canadensis canadensis) was euthanized because of a large mandibular mass. Histopathology revealed an ameloblastic fibro-odontoma, a rare odontogenic neoplasia.

Dentinogenic Ghost Cell Tumor associated with Odontoma: A unique Histopathological Entity and its Surgical Management.

We report a rare case of dentinogenic ghost cell tumor (DGCT) associated with complex composite odontoma in a 17 years male affecting the posterior segment of the mandible. On radiographic examination, there was a well-defined multilocular radiolucency surrounding the radio opaque mass with respect to 44, 45 and 46. Histopathologically it showed ameloblastomatous proliferation with dentin like areas and ghost cells. It was associated with tooth like structures consisting of dentin, cementum and pulp like areas. DGCT with odontoma is extremely rare with only two cases being reported in literature till date. The management with its rare occurrence is discussed here.

Odontoma malformation and disturbances of eruption subsequent to traumatic dental injuries: A literature review and a case report.

Due to the close proximity of the primary teeth with the developing permanent successors, several developmental alterations in the permanent successors have been reported as consequences of traumatic dental injuries to the primary dentition. When they occur at a very young age such trauma could disturb normal tooth development and cause rare anomalies, including sequestration of the permanent tooth germ and odontoma-like malformations. A literature review of case reports with a history of trauma and odontoma formation is discussed in this paper. This paper also includes a case report of a patient, who was 3-years and 5-months old, when she was seen at the University of California, Los Angeles Children's Dental Center for the first time. The grandmother reported that the child was dropped from the father's lap when she was 2 weeks old and suffered head trauma and multiple skull fractures.

Surgical management of compound odontoma associated with unerupted tooth: a case report.

Compound odontoma has been reported to be the most common of all odontogenic neoplasms and tumor- like lesions. It is a slow-growing, asymptomatic neoplasms found incidentally during a routine radiography examination. In general, the clinical indicators of odontoma may include eruption disturbance (non-eruption of permanent teeth, retention of deciduous teeth), expansion of the cortical bone, teeth malposition and pain. In this case, the presence of odontoma prevented the physiological eruption of permanent mandible incisor. We describe the surgical procedure to remove a compound odontoma of 21 small tooth-like structures localized in the mandible of a child boy associated with an unerupted permanent mandible incisor.

Compound odontoma associated with a calcifying odontogenic cyst. Case report and systematic review.

The objectives of the present study were to comprehensively evaluate all the published cases on compound odontoma associated with calcifying odontogenic cyst (COaCOC) in the English literature and to describe the clinical, imaging and therapeutic variables for this condition. In August 2020, an electronic search of the PubMed / MEDLINE, Web of Science, ScienceDirect, Springer, and Scopus databases was carried out. The eligibility criteria included publications with enough information to confirm the diagnosis. Furthermore, we present a clinical case of a 16-year-old male patient with OCCaC, who was treated with enucleation, obtaining favorable and functional results. A total of 32 cases reported in the literature that met the inclusion and exclusion criteria, including ours, were analyzed and discussed. The mayority of the patients were women (n = 17) with an average age of 14.4 years, the maxilla was the most affected bone (n = 22) and the maxillary anterior region was the area with the highest number of cases (n = 18), the main clinical presentations were the volume increase (n = 14) and asymptomatic (n = 14). The choice treatment was enucleation (n = 26) and, in most cases, no recurrence was reported (n = 20). This study allows to update the characteristics of the OCCaC, giving an effective vision of how to treat this rare pathological association made up of two conditions that are completely different from each other.

Ameloblastic Fibroodontoma of Mandible Causing Tumor Induced Osteomalacia: A Case Report with Review of 88 Phosphaturic Oral Neoplasms.

Tumor induced osteomalacia (TIO) is a rare paraneoplastic syndrome associated with tumors secreting fibroblast growth factor 23, which induces osteomalacia. Microscopically, these tumors most commonly show benign phosphaturic mesenchymal tumors. We report the first case of phosphaturic ameloblastic fibro-odontoma (AFO) manifesting as osteomalacia. Our index patient was a 33-year-old male who was diagnosed with TIO and AFO in the mandible was identified as the cause. Our case is unique as AFO is considered as a hamartoma. To the best of our knowledge, there is no hamartoma reported till date causing phosphaturic osteomalacia. As AFO demonstrates mixed epithelial and mesenchymal origin, we propose a new histopathological subtype of TIO-"phosphaturic tumor of mixed epithelial and mesenchymal origin". A review of literature focused on TIO caused by oral lesions revealed 88 oral neoplasms which matched our search criteria. Due to the rarity and unpredictable behavior of TIOs, a high index of suspicion, a broad diagnostic approach, detailed history and multidisciplinary investigations are crucial for establishing the definitive diagnosis and proper treatment recommendations.

Delayed Eruption of Permanent Lower Lateral Incisor in Relation to Compound Odontoma: A Case Report.

AIM: To report a delayed tooth eruption following late surgical removal of a compound odontoma in a 10-year-old child. BACKGROUND: Odontomas are the most common odontogenic tumors. They are usually asymptomatic and discovered during routine radiographic examinations or during an assessment of delayed tooth eruption. Odontomas are classified into compound or complex based on the microscopic structure of dental tissues. Early diagnosis and surgical removal of odontomas is the keyword for favorable results. CASE DESCRIPTION: This paper describes a case of compound odontomas in a 10-year-old boy that prevented eruption of the permanent mandibular lateral incisor. Extraction of mandibular primary lateral incisors and surgical excision of the compound odontomas was performed. After 3 years of follow-up, at the age of 13 years, the permanent lateral incisor erupted and approached the occlusal plane. CONCLUSION: Late surgical removal of odontomas resulted in a delayed eruption of the affected teeth. CLINICAL SIGNIFICANCE: Surgical removal of odontomas and regular follow-up to monitor the eruption of the affected teeth should be considered for the teeth with an open apex before considering orthodontic traction.

Customized surgical guide with a bite block and retraction arm for a deeply impacted odontoma; a technical note.

Odontomas can cause impaction of permanent teeth. During the removal of odontomas associated with an impacted tooth, minimally-invasive surgical approaches are necessary. We present a technical note highlighting easy extraction of a deeply impacted odontoma using a patient-specific computer-aided design/computer-aided manufacturing (CAD/CAM) surgical guide. Its use and advantages are described.

Compound odontoma causing impaction of primary tooth in a 4-year-old child: case report / Impacção de dente decíduo por presença de odontoma composto em criança de 4 anos: relato de caso

Objetivo: relatar um caso raro de impacção de um incisivo decíduo inferior pela presença de odontoma composto, bem como descrever a sua abordagem clínica. Relato de caso: paciente do sexo masculino, com 4 anos de idade, apresentava ausência do incisivo lateral decíduo inferior esquerdo. O exame radiográfico mostrou impacção do incisivo não erupcionado próximo a estruturas radiopacas sugestivas de odontoma composto. O paciente foi acompanhado por dois anos, momento em que se realizou abordagem cirúrgica do caso. Após a cirurgia, a hipótese de diagnóstico de odontoma composto foi confirmada e com o acompanhamento ocorreu a erupção dos incisivos centrais permanentes inferiores. O paciente foi encaminhado para tratamento ortodôntico. Considerações finais: esse relato de caso aborda um caso raro de odontoma composto associado à não erupção de dente decíduo, uma vez que odontomas costumam ser detectados preferencialmente na segunda década de vida do paciente, sendo associados à impacção de dentes permanentes. Além disso, apresenta um protocolo de abordagem clínica para esses casos quando diagnosticados em idade precoce no paciente infantil.(AU)

Objective: to report a rare case of impaction of a primary mandibular incisor due to the presence of a compound odontoma and describe its clinical management. Case report: a 4-year-old boy presented with a "missing" primary left mandibular lateral incisor. Radiographs showed impaction of the unerupted incisor by adjacent radiopaque structures consistent with a compound odontoma. The patient was recalled periodically for 2 years, at which time surgical excision was performed. The diagnosis of compound odontoma was confirmed histologically, and the permanent mandibular central incisors erupted uneventfully; the patient was referred for orthodontic treatment. Final considerations: this case report describes an unusual case of compound odontoma associated with an unerupted deciduous tooth; odontomas are rare in this age range, occurring predominantly in the second decade of life and in association with impaction of permanent teeth. We also propose a protocol for clinical management of such early-onset cases.(AU)

Odontoma associated with mandibular transmigrated canine in a geriatric patient: Second case report.

OBJECTIVE: This report discusses the clinical relevance of a novel case of an odontoma and transmigrated canine in the setting of florid cemento-osseous dysplasia (COD), hypercementosis and moderate to severe periodontitis in a 66-year-old patient. BACKGROUND: An odontoma may infrequently impede tooth eruption and rarely has been implicated with canine transmigration. COD is a benign fibro-osseous lesion associated with decreased vascularity and poorer surgical intervention. MATERIALS AND METHODS: A partially edentulous woman, in no acute distress, underwent clinical and radiographic evaluation in pursuit of comprehensive dental care. RESULTS: Radiographic assessment demonstrated multiple occult pathologies of the mandible, including compound odontoma, canine transmigration, florid COD, hypercementosis and moderate to severe periodontitis, a concurrence thought to be previously unreported. Furthermore, the presence of the odontoma and transmigrated canine is apparently the second reported case in the geriatric population. CONCLUSIONS: The attending clinician should carefully weigh removal of an impacted and transmigrated canine associated with an odontoma in the setting of COD and compromised bone, particularly in older individuals. When surgical intervention is deferred in these clinical situations, patients should continue to be monitored for clinical and radiographic development of pathologic processes.

Cisto ósseo traumático associado a odontoma composto: relato de um caso incomum / Aneurismal bone cyst with a compound odontoma: unusual case report

Introdução: o cisto ósseo traumático (COT) é um pseudocisto que se apresenta assintomático e é descoberto frequentemente em exames de rotina. Outra lesão também presente nos maxilares é o odontoma, sendo dividido em dois subtipos, o composto e o complexo; os odontomas são geralmente descobertos como um achado acidental, visto que não apresentam sintomatologia. Objetivo: relatar um caso incomum de um COT, associado à odontoma composto (OC). Relato de caso: paciente do gênero masculino, 16 anos de idade, compareceu à clínica escola de odontologia da Universidade Federal de Campina Grande (UFCG), campus Patos, PB, referenciado pelo cirurgião-dentista após solicitar exame radiográfico para tratamento ortodôntico e observar lesão radiolúcida em região anterior da mandíbula. Durante a anamnese, o paciente não relatou nenhuma alteração sistêmica ou doença de base, mas relatou trauma de infância na região acometida. No exame clínico intraoral, não foi observado nenhum aumento de volume na região. Realizou-se palpação na região, não havendo relato de dor. Ao analisar a radiografia panorâmica, observou-se a presença de pequenas estruturas calcificadas com radiopacidade semelhante às estruturas dentárias, delimitada por uma linha radiolúcida, sugestiva de OC. Para melhor localização, delimitação, relação com estruturas anatômicas e planejamento cirúrgico da lesão, foi solicitado um exame de tomografia computadorizada de feixe cônico (TCFC). Considerações finais: com base nos achados clínicos e radiográficos, optou-se por abordagem cirúrgica da lesão cística e enucleação do OC, sob anestesia local. No pós-operatório de um ano, o paciente evoluiu satisfatoriamente sem queixas clínicas.(AU)

Introduction: traumatic bone cyst (TBC) is a pseudocyst that usually presents asymptomatically and is found frequently in routine exams. Another lesion also present in the jaws is odontoma. The odontoma is divided into two subtypes, the compound and the complex; odontomas are usually discovered as an accidental finding, since they do not present symptomatology. Objective: the present article aims to report an unusual case of a TBC associated with a composite odontoma. Case report: a 16-year-old male patient attended the Clinic School of Dentistry of the Universidade Federal de Campina Grande (UFCG), Patos-PB campus, referenced by the dentist after identify radiolucent lesion in the anterior region of the mandible on radiographic examination for orthodontic treatment. During the anamnesis, the patient did not report any systemic alteration or underlying disease, but reported trauma in the region affected in childhood. The intra oral clinical examination, was not observed any increase in volume in the region. Palpation was performed in the region, and there was no report of pain. When analyzing panoramic radiography the presence of small calcified structures with radiopacity similar to dental structures was observed, delimited by a radiolucent line, suggestive of compound odontoma. To better location, delimitation, compared with anatomy and surgical planning of the injury, it was requested an cone beam computed tomography (CBCT). Final considerations: based on the clinical and radiographic findings, we opted for a surgical approach to cystic lesion and enucleation of composite odontoma, under local anesthesia. In the one-year postoperative period, the patient progresses satisfactorily without clinical complaints.(AU)

Complex-Compound Odontoma: A Rare Clinical Presentation / Odontoma compuesto complejo: Una presentación clínica rara

ABSTRACT Odontomas can be detected as complex or compound variants and they rarely show the histologic characteristics of both types together. The tumor commonly associated with malocclusion, eruption disturbances and pathological anomalies, but they seldom cause bony expansion. Early detection and management of odontoma with multidisciplinary approach pose an important role to prevent disturbances associated with this common odontogenic tumor. Here we report a rare case of an odontoma which show the features of both complex and compound types and also cause bony expansion, eruption failure in an 8-year-old boy.

RESUMEN Los odontomas pueden detectarse como variantes complejas o compuestas y rara vez muestran la característica histológica de ambos tipos juntos. El tumor comúnmente se asocia con maloclusión, alteraciones de la erupción y anomalías patológicas, pero rara vez causan expansión ósea. La detección temprana y el manejo del odontoma con abordaje multidisciplinario representan un papel importante para prevenir las alteraciones asociadas con este tumor odontogénico común. Aquí presentamos un caso raro de un odontoma que muestra las características de los tipos complejos y compuestos y también causa expansión ósea, falla de erupción en un niño de 8 años.

Evaluation of dentofacial asymmetry caused by odontomas using panoramic radiographs.

AIMS: To evaluate the presence of dentofacial asymmetry in patients with odontoma by panoramic radiography. METHODS: Panoramic images with odontoma were selected among all panoramic radiographs (3058 patients). Maxillary odontoma was detected in 27 patients while mandibular odontoma was detected in 25 patients. In addition, 30 patients with similar age and gender characteristics were selected as the control group. Skeletal angular, skeletal linear and dental measurements were performed on panoramic radiographs. The odontoma region and the opposite side of the odontoma of the individuals were examined. The dentofacial asymmetry of the odontoma groups was compared with the control group. Paired t-test was used to determine dentofacial asymmetry on the right and left side of the patients with odontoma. The ANOVA test was used for testing the differences among groups. RESULTS: As a result of study, no significant difference was found between the region of the odontoma and the symmetrical region in the maxilla and mandibula (P > 0.05). In the control group, a statistically significant difference was found in the angle between the mandibular canal and the mental foramen, lower incisor size, PFH/CutCat(°), and Co-Mc-Me(°) measurements (P < 0.05). In the maxillary and mandibular odontoma groups, a statistically significant difference was found in the angle between the mandibular canal and the menton, CH (mm), RH (mm), and CrH (mm) in the comparison of the odontoma and the control group (P < 0.05). CONCLUSIONS: No difference was found between the right and left sides of the jaws related with the asymmetry of the maxilla and mandible.

Ameloblastic fibro-odontoma in an unusual area of the maxilla: two case reports / Fibro-odontoma ameloblástico en una región inusual de los maxilares: reporte de dos casos

Ameloblastic fibro-odontoma (AFO) is a mixed odontogenic tumor, which has an epithelial and mesenchymal component. It can be observed in imaging tests as a radiomixed lesion, with some authors claiming it represents only the predecessor of an odontoma. Epidemiologically, it appears between the second and third decade of life, predominantly in males and with a predilection for the posterior area of the mandible. This lesion presents a good prognosis and it responds well to enucleation by curettage with a low rate of recurrence. In the present article, two cases of ameloblastic fibro-odontoma in an unusual region of the maxilla are presented.

El Fibro-odontoma ameloblástico (FOA), es una lesión tumoral mixta de origen odontogénico, el cual tiene un componente epitelial y mesenquimático. En su imagenología se observa como una lesión radiomixta, debido a que algunos autores afirman que este no es más que el predecesor de un odontoma. Epidemiológicamente, se presenta entre la segunda y tercera década de la vida, preferencia por sexo masculino y predilección por el área posterior de la mandíbula. Es una lesión de buen pronóstico que responde bien a la enucleación por curetaje con baja tasa de recidiva. En el presente artículo, se presentan dos casos de fibro-odontoma ameloblástico en una región inusual de los maxilares.

Odontoma compuesto como causa de retención dentaria. Presentación de dos casos clínicos / Odontoma compound as a cause of tooth retention. Presentation of two clinical cases

Los odontomas son entidades odontogénicas mixtas, compuestas por una mezcla de células odontogénicas epiteliales y mesenquimatosas diferenciadas. Por lo inusual del hecho el objetivo de nuestro trabajo fue presentar un caso de gemelos dicigóticosbivitelinos masculinos de 10 años de edad con presencia de retención dentaria del incisivo central superior derecho en ambos niños causado por la presencia de un odontoma compuesto, cuyo diagnóstico se corrobora clínica e histológicamente. La lesión se aprecia como una zona radiolúcida bien definida en la que en su interior se ve una estructura radiopaca similar a un diente. Los odontomas fueron extirpados quirúrgicamente y se realizó una ventana quirúrgica en la zona donde se encontró el incisivo central superior para después enlazarlo y llevarlo al arco ortodóncicamente(AU)

Odontogenic entities noontimes are mixed, made by a mixture of odontogenic epithelial cells and differentiated mesenchymal. It made unusual aim of our study was to present a case of fraternal dizygotic male 10 years of age with the presence of dental retention upper right central incisor in both children caused by the presence of a compound odontoma, whose diagnosis is confirmed clinically and histologically the lesion is seen as a well-defined radiolucent area which is inside a structure similar to a tooth radiopaque. Odontomas were surgically removed and underwent surgical window in the area where they found the upper central incisor and then link it and take it to the orthodontic arch(EU)

Odontoma composto associado a incisivo central superior impactado: relato de caso / Compound odontoma associated with impacted upper central incisor: case report

Introdução: o odontoma é um tipo de tumor do epitélio odontogênico com ectomesênquima, podendo estar relacionado à presença de dentes não irrompidos. O tratamento consiste em excisão cirúrgica da lesão. Nor-malmente, dentes impactados são assintomáticos, sendo descobertos em radiografias de rotina. A impactação é causada por fatores sistêmicos ou etiológicos locais. Objetivo: reportar um caso clínico de uma criança com impactação dentária de um incisivo central superior esquerdo associado a um odontoma, enfatizando e discutindo a técnica cirúrgica e o planejamento realizado. Relato de caso: paciente de 13 anos de idade, do gênero feminino, com a não erupção do incisivo central superior esquerdo, ao exame clínico, apresentou ausência clínica do dente 21, persistência do dente 61 e um discreto aumento de volume entre os elementos 61 e 22. Foi solicitada uma tomografia computadorizada de feixe cônico (cone beam) da região, na qual se diagnosticou a presença de uma massa radiopaca envolta por um halo radiolúcido sugestivo de odontoma composto, alterando a posição e impedindo a erupção do dente 21, que se encontrava em posição transal-veolar. O plano de tratamento proposto foi remoção cirúrgica do odontoma e tracionamento do dente não irrompido. Conclusão: a remoção cirúrgica de patologias associadas e as manobras referentes aos dentes envolvidos devem ser realizadas no momento mais oportuno e com adequado planejamento, evitando lesões a estruturas nobres e permitindo, assim, um processo de reparo adequado e uma completa restauração da saúde bucal do paciente. (AU)

Introduction: Odontoma is a type of tumor of the odontogenic epithelium with ectomesenchyme and it may be related to the presence of unerupted teeth. The treatment consists of surgical excision of the lesion. Usually, unerupted teeth are asymptomatic and discovered in routine radiographs. Impaction is caused by local systemic or etiological factors. Objective: The present study aims to report a clinical case of a child with an impacted upper central incisor associated with an odontoma, highlighting and discussing the surgical technique and treatment planning. Case report: A 13-year-old female patient without eruption of the left upper central incisor. After clinical examination, the absence of tooth 21, persistence of tooth 61, and a discrete volume increase between elements 61 and 22 were observed. A cone beam computed tomography of the region was requested, which diagnosed the presence of a radiopaque mass involved by a radiolucent halo suggestive of compound odontoma. This condition changed the position and prevented the eruption of tooth 21, which was found in a transalveolar position. The treatment plan proposed was the surgical removal of the odontoma and orthodontic traction of the unerupted tooth. Conclusion: The surgical removal of associated pathologies and maneuvers related to the teeth involved should be performed in a timely manner, with adequate planning, preventing lesions in noble structures, thus allowing an adequate repair process and a complete restoration of the oral health of the patient. (AU)