Arenavirus as a potential etiological agent of odontogenic tumours in humans.

Odontogenic tumors (OT) are considered rare events and their epidemiologic data are scarce and under-estimated in developing countries because there is no systematic collection of clinical features including histological analyses of the tissue samples. Furthermore, there is an underestimation of the disease relevance and affected people are often marginalized in spite of severe functional impairment of aero-digestive tract. Etiology of OT in humans is still unknown and it represents an important therapeutic and diagnostic challenge.Lassa fever is an acute viral haemorrhagic illness caused by Lassa virus, a member of the arenavirus family of viruses. The disease is endemic in the rodent population in West-East Africa. Humans usually become infected with Lassa virus through exposure to the food or household items contaminated with urine or feces of infected rats. It is also reported person-to-person infections. About 80% of people infected by Lassa virus have no symptoms but the virus establishes a life-long persistent infection.The present commentary significance is to start, for the first time ever, a systematic collection of clinical features and tissue sample collection at the St. Mary's Hospital in Lacor (Gulu) North Uganda where the considered pathologies have an important frequency. The systematic collection will allow to corroborate the possible association between arenaviruses infection and pathogenesis of odontogenic tumors in humans.

Controversies in Odontogenic Tumours: Review.

Odontogenic tumours are lesions that occur solely within the oral cavity and are so named because of their origin from the odontogenic (i.e. tooth-forming) apparatus. Odontogenic tumours comprise a variety of lesions ranging from non-neoplastic tissue proliferations to benign or malignant neoplasms. However, controversies exist regarding the pathogenesis, categorisation and clinical and histological variations of these tumours. The recent 2017 World Health Organization classification of odontogenic tumours included new entities such as primordial odontogenic tumours, sclerosing odontogenic carcinomas and odontogenic carcinosarcomas, while eliminating several previously included entities like keratocystic odontogenic tumours and calcifying cystic odonogenic tumours. The aim of the present review article was to discuss controversies and recent concepts regarding odontogenic tumours so as to increase understanding of these lesions.

Ortho Keratinized Odontogenic Cyst with Dentinoid Formation.

Orthokeratinized odontogenic keratocyst (OOC) was first identified as orthokeratinized variant of odontogenic keratocyst in 1981 by Wright, due to its different histology and relatively low recurrence rate. Parakeratinized odontogenic cyst is now considered as keratocystic odontogenic tumor (KCOT) owing to its neoplastic nature. Although rare, calcification in the form of dystrophic calcification, dentinoid and cartilage has been reported in KCOT, but calcification in OOC is extremely rare and no case in English literature has been reported so far. Here, we report the first case of OOC with calcification located in the mandible of a 40-year female.

Odontogenic Fibromyxoma in a Cat: First Confirmed Case in This Species.

An inflammatory gingival mass surrounding resorbing teeth was diagnosed via biopsy in a 9-year-old domestic shorthair cat. A dorsal rim excision was performed to remove the entire mass with associated teeth and bone. Histopathological diagnosis of the en bloc tissue revealed an odontogenic fibromyxoma. Extensive literature review revealed few case reports of companion animals with this neoplasm, and none in a feline patient. This report documents the clinical presentation, diagnostic differentials, surgical therapy, and long-term follow-up of an odontogenic fibromyxoma in a cat.

Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome.

Gardner syndrome (GS) is caused by mutations in the APC and besides adenomatous colorectal polyps includes such manifestations as osteomas, epidermoid cysts (ECs) and occasionally multiple pilomatricomas. More than 50 % of ECs in patients with GS exhibit pilomatricoma-like ghost cell keratinization. The latter may be explained by the fact that the development of both GS and pilomatricoma is driven by activation of the Wnt/ß-catenin signaling pathway. A 62-year-old, Caucasian male with history of GS presented with a unilocular, mixed radiopaque/radiolucent mandibular lesion causing divergence and external root resorption of involved teeth. Histopathologically, the lesion was composed of two cystic components, an orthokeratinized odontogenic cyst (OOC) and a smaller one with characteristics of keratocystic odontogenic tumor (KCOT) featuring, focally, ghost cells and an epithelial morule-like structure. Dystrophic calcifications essentially similar to those seen in pilomatricomas were observed in the fibrous connective tissue wall. The KCOT and OOC epithelia revealed strong and diffuse cytokeratin (AE1/AE3) and ß-catenin immunoreactivity. CD10 positive immunostaining was seen in the keratin and superficial spinous cell layers in both OOC and KCOT. The intraepithelial and mural ghost cells showed a cytokeratin (+), ß-catenin and CD10 (-) immunophenotype. The diagnosis of OOC with ghost cell calcifications in association with KCOT was rendered. The patient was lost to follow-up. Although a coincidental co-existence cannot be excluded, ghost cell calcifications mimicking pilomatricoma-like changes in an unusual odontogenic cyst combining OOC and KCOT features as seen in this patient with GS may be explained by the common molecular mechanisms underlying the pathogenesis of cutaneous pilomatricomas and GS.

Análise da imunoexpressão de Oct- 4 e C44 em lesões odontogênicas epiteliais benignas / Immunohistochemical analysis of oct-4 and C44 in benign epithelial odontogenic injuries

Lesões odontogênicas epiteliais benignas são entidades de grande importância clínica que se desenvolvem nos ossos maxilares a partir dos tecidos que formam os dentes. Tem sido demonstrado que em tumores benignos e malignos, estão presentes um grande número de células tronco tumorais, as quais tem grandes implicações no desenvolvimento dos tumores. Oct-4 e CD44 têm sido demostrados como importantes marcadores para células-tronco tumorais. O objetivo deste estudo foi identificar células epiteliais que expressam marcadores de células tronco através da expressão imuno-histoquímica de Oct-4 e CD44 em uma série de casos de lesões odontogênicas epiteliais benígnas. A amostra foi constituída por 20 casos de ceratocisto odontogênico (CCO), 20 caos de Ameloblastoma sólido/multicístico e 20 casos de Tumor Odontogênico Adenomatoide (TOA). A expressão de Oct-4 e CD44 foi avaliada no epitélio das lesões através do percentual de células positivas(PP) e da intensidade da expressão ( IE ), sendo realizado o somatório destes escores, resultando na Pontuação de Imunomarcação Total (PIT) que variou de 0 a 7. Os resultados do presente estudo foram analisados pelo valor da pontuação de PIT. Todos os casos apresentaram positividade para os dois marcadores e a maioria exibiu alta expressão para ambos os marcadores.

A análise da expressão de Oct-4 não revelou diferenças estatisticamente significativas (p = 0,406) entre as lesões estudadas. Com relação à expressão do CD44, houve diferença estatisticamente significativa entre os casos de ameloblastoma e CCO, apresentando este último maior número de casos no score 7 (p = 0,034). Na analise da correlação da imunoexpressão de ambos os marcadores nas três lesões estudadas, não houve correlação estatisticamente significativa . Os resultados do presente estudo identificaram a presença de células com características troncais dispostas em locais variados do componente epitelial das lesões ora estudadas sugerindo a sua possível participação na histogênese e diferenciação em lesões odontogênicas epiteliais benignas contribuindo assim para o desenvolvimento destas lesões. (AU)

Benign epithelial odontogenic lesions are great clinical importance entities that develop in the jaws from the tissues that form teeth. It has been shown that in benign and malignant tumors, are present in a large number of tumor stem cells, which has great implications in the development of these lesions. Oct-4 and CD44 have been demos as important markers for tumoral stem cells. The objective of this study was to identify epithelial cells expressing stem cell markers by immunohistochemical expression of Oct-4 and CD44 in a series of cases of benign epithelial odontogenic lesions. The sample was comprised of 20 cases of odontogenic keratocyst (OKC), 20 cases of solid/multicystic ameloblastoma and 20 cases of adenomatoid odontogenic tumor (AOT). The expression of Oct-4 and CD44 was evaluated in epithelial lesions using the percentage of positive cells (PP) and the intensity of expression (IE), being realized the sum of these scores, resulting in Total Immunostaining Score (TIS) ranging 0 to 7.

The results were submitted to the appropriate statistical test (nonparametric Kruskal-Wallis and Spearman correlation coefficient). All cases were positive for both markers and most showed high expression of both markers. The analysis of Oct-4 expression revealed no statistically significant differences (p = 0.406) among the studied lesions. Regarding the CD44 expression, there was a statistically significant difference between the cases of ameloblastoma and TOA in relation to the CCO, with the latter show more cases in the score 7 (p = 0.034). In the correlation analysis of the immunoreactivity of both markers in the three lesions studied, there was no statistically significant correlation. The results of this study identified the presence of cells with stemness characteristics arranged at various sites in the epithelial component of the studied lesions suggesting their possible role in the histogenesis and differentiation in benign epithelial odontogenic lesions, thus contributing to the development of these lesions. (AU)

Cáncer oromaxilofacial en niños: Parte II tumores odontogénicos y de glándulas salivales malignos / Maxillofacial cancer in children: Part II malignant odontogenic and salivary gland tumors

A nivel mundial, la información acerca de tumores malignos del territorio maxilofacial que afectan a niños es limitada. La mayoría de los resportes consiste principalmente en datos de la población adulta. Las neoplasias malignas originadas del aparato odontogénicos y glándulas salivales son lesiones que con cierta frecuencia pueden afectar a la población infantil. Los tumores odontogénicos malignos son entidades sumamente raras que, correspondiendo a menos del 5 % del total de tumores odontogénicos. Los sarcomas odontogénicos, si bien son poco frecuentes, corresponden a los tumores odontogénicos malignos más comunes en la infancia. Las neoplasias malignas de glándulas salivales corresponden al 35­60 % de los tumores de gándulas salivales en la infancia, siendo el más común de ellos el carcinoma mucoepidermoide. En general, el pronóstico de estas entidades es positivo sobre todo si es acompañado de un diagnóstico oportuno. A pesar de la baja frecuencia que presenta este grupo de patologías, no es menos cierto que es necesario saber con precisión cuales son los tejidos orales desde los cuales se pueden originar neoplasias malignas en los niños y tener una breve referencia diferencial entre ellos.

Globally, information about the maxillofacial malignant tumors affecting children is limited. Most reported data consists mainly of studies in the adult population. Malignant neoplasms arising from odontogenic apparatus and salivary glands are lesions that frequently can affect children. Malignant odontogenic tumors are extremely rare entities, corresponding to less than 5 % of all odontogenic tumors. Odontogenic sarcomas, although they are rare, correspond to the most common malignant odontogenic tumors in childhood. Malignant salivary gland neoplasms correspond to 35­60 % of tumors of salivary glands during childhood and the most common of these is mucoepidermoid carcinoma. In general, the prognosis of these entities is positive especially when there is a timely diagnosis. Despite the low frequency presented by this group of diseases, the fact remains that it is necessary to know precisely what the originating oral tissues are which can cause malignancies in children and have a brief reference differential between them.

Recurrent Maxillary Odontogenic Myxoma Following Partial Maxillary Resection and Consecutive Osseous Reconstruction Including Tooth Transplantation.

Odontogenic myxoma (OM) is a rare tumour arising in the jaws. The tumour is purported to be odontogenic in origin due to the frequent localisation of the tumour inside the jaws in close relation to teeth. The aim of this report was to detail the course of a patient who developed OM of the maxilla, underwent adequate ablative surgery and reconstruction, including tooth transplantation to the original tumour site, and subsequently developed a local recurrence in close proximity to the teeth transplanted to the reconstructed maxilla 6 years after the first diagnosis. Once again, a partial maxillary resection was performed, with no reconstruction. The patient has been free from tumour recurrence for over 20 years. We discuss the current hypothesis on OM pathogenesis and the possible impact of actively dividing cells on tumour re-growth.

Molecular markers of cell adhesion in ameloblastomas. An update.

Ameloblastoma is the most common odontogenic tumor of epithelial origin, and though it is of a benign nature, it frequently infiltrates the bone, has a high rate of recurrence and could potentially become malignant. Cellular adhesion potentially plays an important role in the manifestation of these characteristics and in the tumor biology of ameloblastomas. Losses of cell-cell and extracellular matrix adhesion and cohesion are among the first events that occur in the invasion and growth of tumors of epithelial origin. The present review includes a description of the molecules that are involved in cell adhesion as reported for various types of ameloblastomas and discusses the possible roles of these molecules in the biological behaviors of this odontogenic tumor. Knowledge of the complex mechanisms in which these molecules play a role is critical for the research and discovery of future therapeutic targets.

The expression of cytokeratin in keratocystic odontogenic tumor, orthokeratinized odontogenic cyst, dentigerous cyst, radicular cyst and dermoid cyst.

The epithelial lining of odontogenic keratocysts exhibits either parakeratosis or orthokeratosis. In 2005, the WHO classified odontogenic keratocysts with parakeratosis as keratocystic odontogenic tumors (KCOT). Odontogenic keratocysts with orthokeratosis were not classified as odontogenic tumors, but instead referred to as orthokeratinized odontogenic cysts (OOC). To clarify the difference between these two lesions, we investigated their biological characteristics using immunohistochemical studies for cytokeratins (CK) in KCOT and OOC as well as in dentigerous cysts (DC), radicular cysts (RC) and dermoid cysts (DMC). We examined twenty-five cases of KCOT, fifteen cases each of OOC, DC and RC, and ten cases of DMC. We studied the immunohistochemical expression of CK10, 13, 17 and 19. To evaluate the immunohistochemical staining pattern, we divided the epithelial lining of the lesions into three layers (surface layer: su, spinous layer: sp, basal layer: ba). For CK10, most OOC and DMC specimens of su and sp were positive. For CK13 and 19, most KCOT, DC and RC specimens of su and sp were positive. For CK17, most KCOT specimens of su and sp were positive. The percentages of total CK expression of su and sp, and ba of CK19 differed significantly between the lesions (P < 0.001). These results support the hypothesis that OOC originate from not the odontogenic apparatus, but the oral epithelial component.

The keratocystic odontogenic tumor.

In 2005, the World Health Organization renamed the lesion previously known as an odontogenic keratocyst as the keratocystic odontogenic tumor. The clinical features associated with the keratocystic odontogenic tumor show it to be a unilocular or multilocular radiolucency, occurring most frequently in the posterior mandible. These tumors are normally diagnosed histologically from a sample of the lining. With simple enucleation, it seems that the recurrence rate may be from 25% to 60%.

Odontogenic myxomas are not associated with GNAS1 mutations.

UNLABELLED: Myxomas are rare tumors of unknown aetiology arising in the jaws. Myxomas are also diagnosed in soft tissues. Recent reports on Gs alpha subunit gene (GNAS1) mutations occasionally being identified in soft tissue myxomas in non-syndromatic patients and the effects of myxoma on bone in variants of fibrous dysplasia led us to re-examine the putative role of GNAS1 mutations in odontogenic myxoma. MATERIAL AND METHODS: Seven biopsies from patients with confirmed diagnosis of odontogenic myxoma and two cases of fibrous dyplasia of the jaw were investigated for GNAS1 mutations by polymerase chain reaction. RESULTS: Although GNAS1 was mutated in cases of fibrous dysplasia, no GNAS1 mutations were detected in odontogenic myxomas. CONCLUSION: The development of odontogenic myxoma is independent of mutations of GNAS1.

Expression of odontogenic ameloblast-associated protein, amelotin, ameloblastin, and amelogenin in odontogenic tumors: immunohistochemical analysis and pathogenetic considerations.

Screening for expression of amelogenesis-related proteins represents a powerful molecular approach to characterize odontogenic tumors and investigate their pathogenesis. In this study, we have examined the presence and distribution of odontogenic ameloblast-associated protein (ODAM), amelotin (AMTN), ameloblastin (AMBN), and amelogenin (AMEL) by immunohistochemistry in samples of adenomatoid odontogenic tumor (AOT), calcifying epithelial odontogenic tumor (CEOT), developing odontoma, ameloblastoma, calcifying cystic odontogenic tumor (CCOT), ameloblastic fibroma (AF), myxoma, odontogenic fibroma (OF), and reduced enamel epithelia (REE). Positive results were obtained in those tumors with epithelial component, except for AF, OF, and ameloblastoma. ODAM was found around mineralized structures (dystrophic calcifications) and CEOT's amyloid, whereas AMTN stained the eosinophilic material of AOTs. The CCOT transitory cells to ghost cells were strongly positive with all proteins except AMEL, and the REE as well as odontomas showed immunoexpression for ODAM, AMTN, AMBN, and AMEL similar to those found in normal rat tooth germs. Based on these results, some histopathogenetic theories were formulated.

Tumor de Pindborg relacionado con trauma facial / Pindborg's tumor in relationship with facial traumata

El tumor de Pindborg es una neoplasia benigna, rara, con carácter invasivo local y tendencia a la recidiva, que representa entre el 0,17 y el 1,8 por ciento de todos los tumores odontogénicos, del cual tan solo se han publicado unos 200 casos, con una media de 4 casos nuevos por año en el mundo. Se presentó el caso de un hombre de 39 años de edad que acudió a la consulta de cirugía maxilofacial remitido de neurocirugía postraumatismo craneofacial, por presentar un aumento de volumen en el ángulo mandibular derecho. Se tuvo como objetivo publicar la existencia de esta infrecuente neoplasia por lo interesantes que resultan estos tumores por su evolución, dificultad en el diagnóstico, variantes de tratamiento y tendencia a la recidiva. Después de realizar exámenes de laboratorio, radiografías, tomografía axial computarizada y biopsia de fragmento óseo, se obtuvo extensión y diagnóstico de tumor de Pindborg en hemimandíbula derecha. Se realizó la técnica quirúrgica de hemimandibulectomía derecha y reconstrucción con injerto óseo de cresta ilíaca. La evolución del paciente fue satisfactoria(AU)

The Pindborg's tumor is a benign and uncommon neoplasm with a local invasive character and a trend to relapse accounting for the 0.17 and the 1.8 percent of all odontogenic tumors with only 200 cases published in the literature and a mean of four cases per year at world scale. This is the case of a man aged 39 came our consultation of Maxillofacial Surgery referred from Neurosurgery Service after a craniofacial trauma and an increase of volume in right mandibular angle with the aim to publish the existence of this uncommon neoplasm due to the interesting of this type of tumor by its evolution, difficulty for diagnosis, variants of treatment and trend to relapse. After carry out laboratory examinations, X-rays, axial tomography computerize and biopsy of bone fragment, it was possible the extension and diagnosis of Pindbog's tumor in right hemi-mandible. The right hemimandibulectomy is performed as surgical technique and the reconstruction using bone graft of iliac crest. Evolution of patient is satisfactory(AU)

Multiple keratocystic odontogenic tumors associated with nevoid basal cell carcinoma syndrome having distinct PTCH1 mutations: a case report.

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder characterized by developmental abnormalities and a predisposition to cancers. Although multiple jaw tumors, such as keratocystic odontogenic tumors (KCOTs), are one of the most frequent complications in NBCCS, the molecular mechanism for how KCOTs develop in NBCCS is poorly understood. A 15-year-old girl with 2 jaw tumors was diagnosed as NBCCS according to the clinical criteria. The pathologic findings indicated that the 2 tumors were consistent with KCOTs. A PTCH1 mutation, c.1472delT, was detected in her peripheral blood as well as in the 2 tumors. Interestingly, an additional PTCH1 mutation, c.264_265insAATA, that was not present in the peripheral blood, was found in the maxillary tumor but not the mandibular tumor. The Ki-67 labeling index was significantly higher in the maxillary KCOT (17.7%) than in the mandibular KCOT (14.3%). These findings indicate distinct molecular mechanisms of tumorigenesis in these KCOTs.

[Maxillary and mandibular carcinogenesis: research and prospects]. / Tumorogenèse des maxillaires. Recherche et perspective.

Development and growth of odontogenic tumours depend on impairment of numerous genes and molecules. In recent years, most of the genes involved in dental development were identified. This produced a new basis for the study of oral pathology and maxillofacial carcinogenesis. A better understanding of these molecular phenomena should allow to better determine the evolution of such lesions. Research breakthroughs should facilitate the development of new molecular and genetic therapeutic perspectives.

Mixoma del maxilar superior: a propósito de un caso / Maxillary myxoma: a purpose of a case

Discutir sobre el adecuado manejo de pacientes con mixoma en maxilar superior, con un diagnóstico acertado y tratamiento precoz por lo agresivo de este tipo de tumor descripción de un caso clínico y revisión de la literatura. Paciente tratado en el Servicio de Cirugía Plástica, Reconstructiva y Maxilofacial, Hospital Universitario Dr. Luis Razetti, Barcelona. Estado Anzoategui. El tratamiento de elección es la resección en bloque con márgenes oncológicos de seguridad, corte congelado y definitivo, pues el pronóstico es mejor cuanto más radical sea a la extirpación. No responde a quimioterapia ni radiaciones. Se presenta caso de mixoma, tumor mesenquimal poco frecuente, de localización maxilar y mandibular, de características citológicas benignas pero puede mostrar un comportamiento agresivo recidivante tras una resección quirúrgica inadecuada.

Retraso de la erupción de un incisivo inferior permanente asociado a un odontoma compuesto: a propósito de un caso / No disponible

Los odontomas son tumores de origen odontogénico de crecimiento lento y benignos y representan el 22% de los tumores odontogénicos, aunque raramente ocurren en la dentición primaria. Este tipo de lesión generalmente es asintomática siendo detectada en una radiografía de rutina o cuando adquieren un tamaño tal que provoca la dilatación de los tejidos. Suelen estar localizados entre las raíces de los dientes erupcionados o entre la dentición temporal y la permanente. Su tratamiento de elección es la exéresis quirúrgica por su potencial como obstrucción en la erupción dentaria y se deberá hacerse siempre el estudio histológico que nos proporcionará el diagnóstico de certeza. Presentamos el caso clínico de un paciente varón, de 9 años de edad, que presentaba un osontoma compuesto que impedía la erupción de un incisivo central permanente inferior (AU)

Odontomas are benign odontogenic tumours with a slow growth. They rarely occur solely in the primary dentition. Although the lesions are commonly asymptomatic, they may be discovered on routine radiographic examination. They are localized between temporal and definitive teeth. Surgical treatment it´s the best option for these cases, because of the risk of delayed eruption, and it is very important to do a histological examination. This case report presents a nine years-old boy with a compound odontoma located in the mandible, which caused the impaction of a permanent incisive (AU)

Hamartomatous proliferations of odontogenic epithelium within the jaws: a potential histogenetic source of intraosseous epithelial odontogenic tumors.

BACKGROUND: The jawbone is replete with a vestige of odontogenesis. The overall consensus is that intraosseous remnants of the enamel organ and dental lamina are the only histogenetic option for central epithelial odontogenic tumors. Curiously, incipient tumors or possible precursor conditions of residual odontogenic epithelium have rarely been reported in the literature. METHODS: We microscopically evaluated 39,660 biopsy samples to determine the presence of a tumor-like odontogenic epithelial nodule in the maxilla and mandible. RESULTS: Seven intraosseous specimens that associated with a focal proliferation of odontogenic epithelium were retrieved. Six hamartomatous processes showed four different morphologic patterns comparable with the tumor nests comprising ameloblastoma (n = 1), squamous odontogenic tumor (n=1), calcifying epithelial odontogenic tumor (n=2) and calcifying cystic odontogenic tumor (n=2). Among six lesions, four were the intrafollicular development. The remaining case of interest was multiple hyperplastic clear rests of Malassez in association with an impacted tooth. CONCLUSION: Although it is impossible to predict the fate of these microscopic structures of hamartomatous character, the present case series indicates that any of the dormant embryonic residues of odontogenic epithelium can return to an active state, capable of non-reactive, probably neoplastic proliferation of pathological significance.

Tumor odontogênico adenomatóide associado a cisto dentígero: relato de um caso incomum / Adenomatoid odontogenic tumour associated with dentigerous cyst: unusual case report

O tumor odontogênico adenomatóide é uma lesão relativamente incomum, que acomete preferencialmente indivíduos do sexo feminino durante a segunda década de vida, exibindo como sítio de predileção a região anterior da maxila. A lesão geralmente está associada à coroa de um dente incluso, comumente o canino. Neste trabalho é relatado o caso de um tumor odontogênico adenomatóide associado a cisto dentígero ocorrendo na região maxilar esquerda, em paciente do sexo feminino com 13 anos de idade, discutindo-se, ainda, as características clínicas, radiográficas, histopatológicas e terapêuticas do caso.

The adenomatoid odontogenic tumor is a relatively uncommon lesion which mainly affects females in their second decade of life, exhibiting predilection for the anterior region of the maxilla. The lesion is usually associated with the crown of an enclosed tooth, most commonly the maxillary canine. In this paper we present a case of adenomatoid odontogenic tumor associated with a dentigerous cyst affecting the left maxillary region in a 13-year-old female. The authors also discuss clinical, radiographic, histopathologic and therapeutic features of the case.