Multidisciplinary approach to multiple dental anomalies in pediatric patients: a case report with 4-year follow-up.

The objective of this article is to report the clinical case and 4-year follow-up of a 5-year-old child with multiple dental anomalies, emphasizing the importance of early diagnosis and use of combined pediatric surgery and orthodontic approaches. A 5-year-old boy, accompanied by his mother, sought dental care for dental caries and tooth pain. Clinical and radiographic examinations revealed active caries, a supernumerary primary tooth in the region of the mandibular right second premolar, and severe ankylosis of the primary mandibular right second molar. The treatment plan involved extraction of the supernumerary tooth as well as sectioning and extraction of the ankylosed molar. When the patient was 6 years old, the permanent mandibular right first molar showed signs of an altered eruptive process, and orthodontic treatment was initiated. A unilateral band-and-loop space maintainer with coil springs designed to move the permanent first molar was placed on the primary first molar. A new panoramic radiograph, obtained when the patient was aged 7 years, suggested the presence of an odontoma in the apical region of the primary maxillary right canine. Surgical removal and histopathologic examination of the lesion confirmed that it was a developing odontoma. After surgery, due to occlusal anomalies that included transverse maxillary deficiency, deep overbite, and midline deviation, the patient underwent rapid maxillary expansion therapy with a Haas-type appliance. When the patient was 8 years old, orthodontic treatment continued with a removable palatal Hawley expander and a orthodontic mandibular lingual arch. Currently, at the age of 9 years, the child is still undergoing fixed orthodontic treatment after surgical exposure of the impacted permanent maxillary right canine and bonding of an orthodontic attachment to enable traction. A multidisciplinary approach to the management of dental anomalies promotes a favorable prognosis and ensures comprehensive treatment of young patients.

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-Odontoma.

Introduction: Ameloblastic fibro-odontoma (AFO) is a benign odontogentic tumor without an aggressive behavior, unlike the similar ameloblastic fibroma. Case Presentation: A 9-year-old boy, with tooth eruption failure, underwent enucleation and curettage of a well-defined variable radiolucent and radio-opaque right mandible lesion. There was odontogenic epithelium with peripheral palisading in a loose myxoid stroma as well as a disorganized component of dentin, enamel, and cementum, features of an AFO. Conclusion: AFO is an odontogenic mixed tumor of epithelium and mesenchyme.

Peripheral compound odontoma: A rare case report and literature review.

Peripheral odontoma is a very rare odontogenic hamartoma arising in soft tissues. Here, we report a case of peripheral odontoma in a pediatric patient and review the cases published in the literature. An 11-year-old male patient presented a nodular lesion in the anterior region of the palate for over 1 year. Under the clinical hypothesis of fibroma, an excisional biopsy was performed. Histopathological examination revealed the presence of tooth-like structures, formed by enamel, and dentin matrix, occasionally associated with the dental papilla and surrounding pulp tissue, thus, the histopathological diagnosis of peripheral odontoma was established. The patient has been undergoing follow-up for 6 months without any signs of lesion recurrence. Peripheral odontomas are uncommon lesions that usually affect young patients and display a preference for the maxilla and limited growth potential. The recognition of the clinical and histopathological features of the peripheral odontoma is indispensable for the establishment of its diagnosis.

Odontomas in Frogs.

Odontomas are variably differentiated, hamartoma-like proliferations of odontogenic epithelium, pulp ectomesenchyme (odontoblasts), and dental matrix. Frogs are polyphyodont and homodont. Their teeth also differ from mammals in that they are restricted to the upper jaw in adults and lack a periodontal ligament and cementum, attaching directly to the underlying bone. Odontomas were identified in an African clawed frog (Xenopus laevis), a false tomato frog (Dyscophus guineti), and a tomato frog of unknown species (Dyscophus sp.). All of the examined odontomas were composed of numerous tooth-like structures comprising an arc of dentinal matrix lined on the convex surface by ameloblasts and on the concave surface by odontoblasts. Masson's trichrome and immunohistochemistry with pan-cytokeratin supported these findings. The pathogenesis of these lesions may be displacement of the dental lamina, which has been shown in research studies to lead to de novo proliferation of dental elements in frogs.

Compound odontoma in a nine-years-old boy associated with impacted permanent central and lateral incisor - a case report.

Odontomas are one of the most common tumours of odontogenic origin. They are usually asymptomatic but may be associated with retained primary teeth or missing permanent teeth. Though the exact aetiology is unknown, the postulated causes include trauma, infection, inheritance and genetic mutation. Early diagnosis and management will result in fewer complications. Conservative surgical excision is the treatment of choice. This case report presents a treated case of compound odontoma associated with delayed eruption of the permanent central incisor in a nine-yearsold boy.

Three years of follow-up of otodental syndrome in 3-year-old Chinese boy: a rare case report.

BACKGROUND: Otodental syndrome is an exceptionally rare autosomal dominant condition characterized by a delayed eruption of posterior teeth, globodontia, lisping, and sensorineural hearing loss. In this case report, we reported a 3-year-old Chinese boy with the otodental syndrome. CASE PRESENTATION: A 3-year-old Chinese boy was referred to our hospital with complaint of no eruption of primary canines and molars. Three years follow-up showed lately erupted bulbous primary canines with hypoplastic enamel spot, globe-shaped primary molars and sensorineural hearing loss at 4 and a half-year-old age. We diagnosed otodental syndrome in the patient's mother with hearing loss at 16-year-old age. Gene sequencing and analysis of deafness-related genes GJB2, GJB3, SLC26A4, and mtDNA did not reveal any mutation or SNPs in the patient and his mother. CONCLUSIONS: This case report highlights the importance of detailed medical, dental, and family history examination, as well as multi-disciplinary teamwork for diagnosis and treatment of otodental syndrome.

Patients with SATB2-associated syndrome exhibiting multiple odontomas.

Special AT-rich sequence-binding protein 2 (SATB2)-associated syndrome (SAS) is characterized by alterations of SATB2. Its clinical features include intellectual disability and craniofacial abnormalities, such as cleft palate, dysmorphic features, and dental abnormalities. Here, we describe three previously undiagnosed, unrelated patients with SAS who exhibited dental abnormalities, including multiple odontomas. Although isolated odontomas are common, multiple odontomas are rare. Individuals in families 1 and 3 underwent whole-exome sequencing. Patient 2 and parents underwent targeted amplicon sequencing. On the basis of the hg19/GRCh37 reference and the RefSeq mRNA NM_001172517, respective heterozygous mutations were found and validated in Patients 1, 2, and 3: a splice-site mutation (chr2:g.200137396C > T, c.1741-1G > A), a nonsense mutation (chr2:g.200213750G > A, c.847C > T, p.R283*), and a frame-shift mutations (chr2:g.200188589_200188590del, c.1478_1479del, p.Q493Rfs*19). All mutations occurred de novo. The mutations in Patients 1 and 3 were novel; the mutation in Patient 2 has been described previously. Tooth mesenchymal cells derived from Patient 2 showed diminished SATB2 expression. Multiple odontomas were evident in the patients in this report; however, this has not been recognized previously as a SAS-associated phenotype. We propose that multiple odontomas be considered as an occasional manifestation of SAS.

Ameloblastic fibroma and ameloblastic fibrosarcoma: A systematic review.

PURPOSE: To integrate the available data published to date on ameloblastic fibromas (AF) and ameloblastic fibrosarcomas (AFS) into a comprehensive analysis of their clinical/radiological features. METHODS: An electronic search was undertaken in July 2017. Eligibility criteria included publications having enough clinical, radiological and histological information to confirm a definite diagnosis. RESULTS: A total of 244 publications (279 central AF tumours, 10 peripheral AF, 103 AFS) were included. AF and AFS differed significantly with regard to the occurrence of patients' mean age, bone expansion, cortical bone perforation and lesion size. Recurrence rates were as follows: central AF (19.2%), peripheral AF (12.5%), AFS (all lesions, 35%), primary (de novo) AFS (28.8%) and secondary AFS (occurring after an AF, 50%). Larger lesions and older patients were more often treated by surgical resections for central AF. Segmental resection resulted in the lowest rate of recurrence for most of the lesion types. AFS treated by segmental resection had a 70.5% lower probability to recur (OR 0.295; P = .049) than marginal resection; 21.3% of the AFS patients died due to complications related to the lesion. CONCLUSIONS: Very long follow-up is recommended for AF lesions, due to the risk of recurrence and malignant change into AFS. Segmental resection is the most recommended therapy for AFS.

Ameloblastic Fibrodentinoma and Ameloblastic Fibro-Odontoma: An Updated Systematic Review of Cases Reported in the Literature.

PURPOSE: To integrate the available data published on ameloblastic fibrodentinoma (AFD) and ameloblastic fibro-odontoma (AFO) into a comprehensive analysis of its clinical and radiologic features. MATERIALS AND METHODS: An electronic search was undertaken in August 2016. Eligibility criteria included publications reporting cases of AFD or AFO with enough clinical, radiologic, and histologic information to confirm the diagnosis. Demographic data, lesion site and size, treatment approach, and recurrence were analyzed and compared between AFD and AFO. RESULTS: Fifty-four publications reporting on 64 AFDs (60 central, 4 peripheral) and 137 publications reporting on 215 AFOs (211 central, 3 peripheral, 1 unknown) were included. The difference in recurrence rate (when the information about recurrence was provided) was not statistically relevant. The mean age of patients affected by AFD was not statistically different from that of patients affected by AFO. CONCLUSIONS: AFD and AFO presented several similarities: higher prevalence in men and in the mandibles, similar mean age of patients, rate of cortical bone perforation and of the lesions' association with displaced or unerupted teeth and tooth root resorption, mean lesion size, and recurrence rate. The lesions differed in the presence of radiopacities and locularity. Taken together, these data do not support the concept of progressive maturation of these tumoral conditions.

Association Between Odontoma and Impacted Teeth.

Odontoma is considered to be the most common odontogenic tumor of the oral cavity. Most odontomas are asymptomatic and are discovered during routine radiographic investigations and can cause disturbances in the eruption of the teeth, most commonly delayed eruption or deflection. By a retrospective study design, demographic and clinical data regarding patients who presented odontomas from year 1995 to 2015 were obtained in order and the influence of active therapy on the dentition and on the treatment of impacted teeth was analyzed. Forty-five patients (mean age 14.2 years) with 29 complex and 16 compound odontomas were included in this retrospective study. Initial symptoms were delayed eruption of permanent teeth (n = 25), pain (n = 6), swellings (n = 4), and no symptoms (n = 10); 31 patients were discovered by incidence, all of them via panoramic radiographs. The mandible/maxilla ratio was about 2:1 (31/15). Thirty-two out of 45 odontomas were in close proximity of at least 1 tooth (n = 21 at incisive). A total of 12 teeth were extracted (complex: n = 8; compound: n = 4). Of the nonextracted teeth, 33 teeth were displaced and retained. Of those, 29 teeth were aligned through orthodontic-surgical approach and 4 teeth erupted spontaneously after surgery during the follow-up period. An early detection of odontoma is more likely an accidental radiological finding, hence the need for routine radiographic analysis should be emphasized. Early diagnosis of odontomas in primary dentition is crucial in order to prevent later complications, such as impaction or failure of eruption of teeth.

Mandibular odontogenic sarcoma (ameloblastic fibrodentinosarcoma) in an aged cat - Short communication.

A 13-year-old male cat presented with an ill-defined mass in the rostral mandible causing destruction and loss of alveolar bone. Microscopically, the mass consisted of cords or islands of benign odontogenic epithelium and a malignant, pleomorphic spindle-shaped cell component with dysplastic dentine formation. Immunohistochemically, neoplastic mesenchymal cells proved to be strongly positive for vimentin and negative for cytokeratins, desmin, actin and S100 protein; the Ki67 proliferation index was high. Morphological and immunohistochemical features largely overlap those reported for ameloblastic fibrodentinosarcoma, an uncommon histologic subtype of odontogenic sarcoma recognised in humans but no reported previously in animals. Ki-67 expression assessment may help to discriminate between malignant and benign forms of odontogenic tumours but the final diagnosis is mainly morphological.

Odontomas in developmental age: confocal laser scanning microscopy analysis of a case.

BACKGROUND: Odontomas are the most common benign odontogenic tumors (especially in children and adolescents) and consist of odontogenic ectomesenchyma and odontogenic epithelium with the formation of dental hard tissues. They are also simply considered hamartomas. The WHO Classification defines them as complex and compound odontomas. The diagnosis is often occasional, in conjunction with x-ray routine examinations, or it is suggested by eruption disorders or abnormal position of teeth in the dental arch. The mainstay therapy is surgical excision of the lesion followed by orthodontic treatment to take in the arch the impacted teeth. CASE REPORT: The aim of this work is the presentation of a case of mandibular bilateral compound odontoma in a young patient, and the confocal laser scanning microscopic analysis of the surgical specimens.

Metachronous ameloblastic fibro-odontoma and dentigerous cyst in the posterior mandible.

An ameloblastic fibro-odontoma (AFO) is a rare mixed odontogenic tumor with histologic features of an ameloblastic fibroma in conjunction with the presence of dentin and enamel. It usually appears as a well-circumscribed radiolucency with radiopaque foci and slow growth and is commonly seen in children and young adults. A 13-year-old boy presented with an asymptomatic swelling in the posterior right region of the mandible and the right ascending ramus. The clinical, imaging, and histopathologic findings confirmed the diagnosis of an AFO. After 8 months, a radiolucent lesion involving the unerupted mandibular left third molar was observed; a final diagnosis of a dentigerous cyst (DC) was established for this lesion. Although coincidental events, metachronous odontogenic lesions suggest a possible common genetic origin, since both can be caused by related cellular signaling pathways. Complete enucleation is recommended for both AFOs and DCs; rates of recurrence are low.

Primordial Odontogenic Tumor: Report of a Case.

Primordial odontogenic tumor (POT) was first described in 2014. It typically presents in the posterior mandible of a child or adolescent as a "dentigerous cyst-like" well-circumscribed radiolucency associated with an unerupted molar. POT consists of an ellipsoidal mass of dental papilla-like myxoid connective tissue entirely enveloped in a delicate membrane of ameloblastic epithelium. It shows features of a developing tooth with a huge dental papilla, and because it is devoid of dental hard tissue, it could be regarded a soft tissue odontoma. The lesion histologically mimics early (primordial) stages of tooth development. This report describes a case of POT and POT-like proliferations in an unrelated complex odontoma.

Extensive Mandibular Ameloblastic Fibro-Odontoma.

Ameloblastic fibro-odontoma (AFO) is a mixed odontogenic tumor that presents epithelial and mesenchymal components. Ameloblastic fibro-odontoma is generally diagnosed between the first and second decades of life and normally shows a slow clinical growth in the posterior portion of the maxilla or mandible, being mostly associated with 1 or more impacted teeth. Radiographic features of AFO show a radiolucent well-defined, uni, or multilocular defect due to containing variable amounts of calcified material. The enucleation of the tumor is the usual conduct and should be followed up for a long period of time. Here, the authors report the case of 17-year-old male patient who presented an extensive AFO on the right posterior side of the mandible. The panoramic radiograph and the tomographic examination revealed a multilocular radiolucent lesion with impacted teeth. Histological examination revealed connective tissue resembling the dental papilla along with epithelial strands or islands, as well as dental hard tissue such enamel and dentin. Enucleation and curettage was performed and led to good outcome. There was no recurrence after an 8-year follow-up, and oral rehabilitation was performed with dental implants.

Retrospective study of 289 odontogenic tumors in a Brazilian population.

BACKGROUND: Odontogenic tumors (OTs) are considered important among oral lesions because of their clinicopathological heterogeneity, and variable biological behavior. This paper aims to determine the frequency and distribution of OTs, over a period of 10 years, at a public university in Northeastern Brazil and compare this data with previous reports. MATERIAL AND METHODS: We reviewed all cases of OTs from oral pathology laboratory of University of Pernambuco (UPE), from 2004 to 2014. Diagnoses were re-evaluated and the tumors were classified according to the latest (2005) World Health Organization Classification of Tumors. In addition, we searched in the English-language literature retrospective studies on OTs that used the same classification. RESULTS: Within the total of 6028 oral biopsies, 289 (4.79%) were OTs. Of these, 287 (99.3%) were benign and 2 (0.7%) were malignant. The overall incidence was 31.1/million. Mandible-maxilla ratio was 2.5:1 and mean age 35 years. Keratocystic odontogenic tumor (KCOT) (34.6%) was the most frequent lesion, followed by ameloblastoma (AMB) (32.9%) and odontoma (ODO) (11.4%). CONCLUSIONS: OTs are uncommon neoplasms with geographic variation. Our clinicopathological features are according to literature. In the present study, KCOT was the most frequent one, showing that the new classification of OTs altered the distribution of these lesions and possibly made KCOT the most common OT observed in diagnostic services worldwide.

Extensive Maxillary Odontomas in 2 Dogs.

Two immature male dogs were presented for the treatment of extensive maxillary compound odontomas. In both cases, clinical evaluation and diagnostic imaging revealed extensive lesions invading and displacing anatomical structures of the maxilla. Histopathology confirmed the diagnoses in both cases. One dog was treated with partial rostral maxillectomy, and the other dog was treated with enucleation of the cystic lining and removal of tooth-like structures through a lateral rhinotomy utilizing an intraoral approach. This report illustrates the growth potential of maxillary compound odontomas in 2 dogs and highlights their subclinical appearance and excellent prognosis despite their extensive nature.