Squamous Odontogenic Tumor: Review of the Literature and Report of a New Case.

PURPOSE: Squamous odontogenic tumor (SOT) is a rare, benign, locally infiltrative odontogenic tumor of the gnathic bones. It is composed of islands of bland, well-differentiated squamous epithelium of varying shape and size. Because of histologic overlap, SOT has often been overdiagnosed as ameloblastoma and squamous cell carcinoma. It thus becomes important to understand the clinical, radiologic, histopathologic, and treatment characteristics of this tumor. MATERIALS AND METHODS: Using the PubMed and Google Scholar databases, we searched for reported cases of SOT published in the English-language literature. We were able to retrieve 49 acceptable cases and perform a comprehensive literature review of the intraosseous SOTs, with emphasis on their clinical, radiographic, and pathologic characteristics, as well as treatment strategies. In addition, we present an additional case of SOT affecting the posterior mandible in a 44-year-old female patient. RESULTS: The tumor in the posterior mandible in our patient was accompanied by acute pain and treated by enucleation. Histopathologic evaluation showed variably sized islands of benign squamous epithelium scattered in a fibrous stroma, consistent with the diagnosis of an SOT. Uneventful healing was noted at the 1-month postoperative appointment. However, the patient was lost to long-term follow-up. Our literature review showed that the average age at the time of diagnosis of SOT is 34.2 years. Men and women are equally affected, and the tumor does not show a predilection for either jaw bone. The most common locations are the anterior maxilla and posterior mandible. Most SOTs are treated conservatively by enucleation or curettage, whereas aggressive or recurrent tumors require radical resection. CONCLUSIONS: Careful evaluation of the excised specimen, with immunohistochemical investigations, may prove rewarding in differentiating an SOT from other odontogenic neoplasms and thus minimize the patient's chances of undergoing an unnecessary aggressive intervention.

Surgical Management of an Aggressive Multifocal Squamous Odontogenic Tumor.

Squamous odontogenic tumor is a rare benign epithelial odontogenic tumor of the jaw. Most are solitary lesions, although rare multifocal lesions have been described. Maxillary lesions have more aggressive behavior. Because of their benign character, these lesions are usually treated using a conservative surgical approach with curettage and surgical enucleation. This report describes the case of a 29-year-old woman with multifocal lesions who was initially treated with conservative surgical therapy. Early recurrence 6 months after surgery prompted more aggressive resection. This case is discussed in the context of current evidence related to the epidemiology, etiology, diagnosis, and therapy of squamous odontogenic tumors.

Ameloblastoma desmoplásico con predominio quístico: presentación de un caso clínico / Desmoplastic ameloblastoma with cystic predominance: presentation of a clinical case

El ameloblastoma es una neoplasia odontogénica que muestra un comportamiento agresivo local. Algunas variantes como la uniquística y la desmoplásica tienen un mejor pronóstico que la sólida/multiquística. Presentamos un caso en un varón de 76 años con una lesión radiolúcida interradicular mandibular de apariencia quística, la cual se enucleó quirúrgicamente. Histológicamente correspondió a una lesión desmoplásica con una amplia zona quística que fue diagnosticada como ameloblastoma desmoplásico con predominio quístico. Trascurridos 30 meses no se evidencia recidiva. En este artículo se discuten los principales aspectos clinicopatológicos de estas entidades (AU)

Ameloblastoma is an odontogenic neoplasm with locally aggressive behavior. Some variants, like the unicystic or desmoplastic, have a better prognosis than solid/multicystic. The case is presented of a 76 year-old male, who suffered a mandibular interradicular radiolucent lesion of cystic appearance, which was surgically enucleated. Histologically, this was a desmoplastic lesion that exhibited a predominantly cystic area which was diagnosed as a desmoplastic ameloblastoma with cystic predominance. After 30 months of follow-up there are no signs of recurrence. The most important clinical and pathological features of these conditions are also discussed (AU)

Fibroma ameloblástico asociado a un odontoma compuesto. La importancia del estudio histopatológico / Ameloblastic fibroma associated with a compound odontoma. Importance of the histopathological study

El fibroma ameloblástico es un tumor odontogénico mixto benigno de rara aparición, que constituye el 2% de todos los tumores odontogénicos, es de crecimiento lento, más común en niños y adultos jóvenes, compuesto por tejido conjuntivo fibroso embrionario y epitelio odontogénico primitivo, se caracteriza por la proliferación de tejido epitelial y mesenquimático. Aparece con más frecuencia en la mandíbula en zona de molares y premolares de pacientes jóvenes sin predilección de sexo, asociándose a veces a un diente incluido. El presente artículo tiene como objetivo describir un caso clínico de un paciente en la segunda década de vida, con aparente anodoncia en el maxilar superior, que se encontraba asintomático y en el cual fue diagnosticado fibroma ameloblástico en maxilar superior, zona de incisivos anteriores, lado izquierdo, se realiza una breve revisión de la literatura y diagnósticos diferenciales, se analizan sus características clínicas e histológicas y la actitud terapéutica a tomar. El tratamiento quirúrgico conservador con extirpación seguida de curetaje parece ser la opción terapéutica más adecuada, y teniendo presente que el porcentaje de recidiva es del 18,3% principalmente debido a escisión incompleta de la lesión, se deben realizar controles radiográficos postoperatorios 6 meses después y cada año por los siguientes 5 años (AU)

The ameloblastic fibroma is a benign mixed, rare odontogenic tumour, which accounts for 2% of all odontogenic tumours. It is slow growing, and more common in children and young adults. It is composed of embryonic fibrous connective tissue and early odontogenic epithelium, and characterised by the proliferation of epithelial and mesenchymal tissue. It appears most frequently in the jaw area of molars and premolars of young patients with no sex predilection, and is sometimes associated with an impacted tooth. This article aims to describe a clinical case of a child in the second decade of life, with apparent anodontia in the maxilla, which was asymptomatic and later diagnosed as an ameloblastic fibroma in the left side maxilla incisors area. A brief review of the literature and differential diagnoses was carried out, including an analysis of its clinical and histological features, and the therapeutic approach to take. Conservative surgical excision followed by curettage seems to be the most appropriate treatment option. It should be noted that the recurrence rate is 18.3%, mainly due to incomplete excision of the lesion. Radiographic controls should be performed six months postoperatively, and every year for the following five years (AU)

Tumor odontogênico queratocístico: atualidades / Keratocyst odontogenic tumor: an update

O tumor odontogênico queratocístico (TOQ) é uma neoplasia benigna dos ossos gnáticos, que apresenta agressividade local e alta taxa de recorrência. O TOQ é uma lesão intra-óssea, invasiva e destrutiva dos maxilares que apresenta crescimento lento e infiltrativo sendo, geralmente, uma lesão assintomática. A radiografia e a tomografia computadorizada auxiliam no diagnóstico, sendo necessária a biópsia e análise histopatológica para diagnóstico definitivo. Entre as técnicas de tratamento estão a ressecção, a enucleação, a curetagem, a descompressão e a marsupialização. É de suma importância que o cirurgião conheça a neoplasia e a recente classificação da Organização Mundial da Saúde para que, após o diagnóstico, os tratamentos sejam adequadamente escolhidos. Este estudo visou abordar atualidades acerca do TOQ por meio da revisão da literatura.

The keratocystic odontogenic tumor (KOT) is a benign neoplastic lesion of the jaws, disclosing a high local aggressiveness and a high recurrence rate. The KOT is an asymptomatic slow growing tumor of the jaws with an infiltrative, invasive and destructive intraosseous behavior. Radiography and computed tomography images aid the diagnosis but biopsy and histopathology must be carried out to point a definitive diagnosis. The treatment is a surgical procedure. The techniques are resection, enucleation, curettage, decompression and marsupialization. It is paramount that the surgeon knows the lesion features after the recent World Health Organization remarks to perform the best treatment after diagnosis. This study aimed to address updates about KOT through a literature review.

Manejo quirúrgico del ameloblastoma / Surgical management of ameloblastoma

El ameloblastoma es un tumor odontogénico de estirpe epitelial. Aunque se clasifica como una tumoración benigna, suele ser localmente agresiva presentando elevada invasión local, con gran tendencia a la recidiva y con posibilidad metastásica ocasional. Se manifiesta preferentemente durante la tercera, cuarta y quinta décadas de la vida, sin predilección por razón de sexo, aunque puede darse en cualquier grupo de edad, incluidos los niños. Lamayoría de los ameloblastomas se encuentran sobre todo enmandíbula (al nivel del ángulo y rama). En el tratamiento se debe valorar su tipología clínica (sólido, multiquístico, uniquístico, mixto o periférico), su localización y el tamaño del tumor, así como la edad y las condiciones clínicas del paciente. Presentamos una revisión de los pacientes afectos de ameloblastomas tratados en nuestro Centro durante los últimos 10 años. Se aportan datos acerca de su aparición clínica, sus características histológicas, el manejo terapéutico realizado y analizamos el seguimiento y comparamos la aparición de recidivas en los pacientes presentados. Las características clínicas, incluso si se complementan con radiografías y/omuestras histológicas, no son siempre determinantes del comportamiento biológico y, por tanto tampoco lo son del pronóstico de un ameloblastoma individual(AU)

The ameloblastoma is an odontogenic tumour of epithelial origin. Although it is classified as benign, there is usually aggressive local invasion, a great tendency to recurrence, and occasional metastatic potential. It generally appears during the third, fourth and fifth decades of life, without gender predilection, although it can occur at any age, including in children. Ameloblastomas are mostly found in the mandible (angle and branch). In treatment, its clinical type (solid, unicystic, desmoplastic, mixed or peripheral), its location and size, must be assessed, as well as the age and clinical condition of the patient. We present a review of patients diagnosed and treated for ameloblastoma in our hospital during the last 10 years.We present data on clinical appearance, histological characteristics, and therapeutic management, and we analyse and compare the rate of recurrence in these patients. The clinical features, even if they are supplemented with radiographs and/or histological samples, are not always biological determinants of its behaviour, or of the individual prognosis of the ameloblastoma(AU)

The diagnostic and surgical management of a multifocal calcifiyng epithelial odontogenic tumor in the mandible and maxilla associated with a squamous odontogenic tumor: first reported case in the literature.

The calcifiyng epithelial odontogenic tumor (CEOT) and the squamous odontogenic tumor (SOT) are rare and benign odontogenic tumors that affect the jaw. This article describes the diagnostic and surgical management of a multifocal CEOT in the mandible and maxilla associated with an SOT. This case is the first to demonstrate that there may be a multifocal variant of CEOT associated with an SOT that has not been previously recognized. Clinical, radiographic, and histologic signs are reported. Treatment modality, oral rehabilitation, and long-term follow-up are shown. Because of the rarity of this kind of simultaneous pathologic association, it is of paramount, and indeed obvious, importance to prospective reviewers that published reports are produced meticulously, including all clinically, radiographically, and histologically relevant data.

A clinicopathological study of malignant odontogenic tumours.

AIMS: Malignant odontogenic tumours (MOTs) are rare neoplasms occurring primarily within the jaw. The objective of this study was to determine the incidence, demographics and clinicopathological features of the MOTs from two institutions. METHODS AND RESULTS: The records of the Department of Oral Pathology, Faculty of Dentistry, Chulalongkorn University, Bangkok, Thailand and the Department of Oncology and Diagnostic Sciences, Dental School, University of Maryland, Baltimore, USA were searched from 1991 to 2010; we identified 17 cases of previously diagnosed MOTs. All cases were reviewed independently of the previous diagnosis by two blinded oral pathologists and reclassified based on the 2005 World Health Organization classification of head and neck tumours. In this study we describe in detail these 17 cases which presented with an average age of 50.29 years and a male to female ratio of 2.4:1. These cases included five ameloblastic carcinomas, four atypical ameloblastomas, three primary intraosseous squamous cell carcinomas, three intraosseous mucoepidermoid carcinomas and two clear cell odontogenic carcinomas. All cases were treated by surgical resection and one patient with ameloblastic carcinoma received postoperative radiotherapy. CONCLUSIONS: Malignant odontogenic tumours are considered rare central odontogenic lesions. Awareness of their existence, rapid diagnosis and successful treatment using surgery, radiation and/or chemotherapy is critical to patient survival.

[Therapy-resistant aggressive cystic lesion of the mandible]. / Therapieresistente aggressive zystische Läsion des Unterkiefers.

Several extensive surgical interventions of a cystic lesion in the left mandible were followed by recurrences. The lesion extended from the primary mandibular region into the area of pterygopalatine fossa finally infiltrating the orbital region and the skull base. Histological results could never demonstrate a malignancy with certainty. Due to the patient's poor general condition, the refusal for further surgical inventions and due to the malignoma-like growth pattern radiation treatment was performed. However, this had no effect on tumor progression.

Squamous odontogenic tumor.

A 24 year male presented with a radiolucent lesion associated with the right maxillary canine. Histologic examination of the biopsy material resulted in a diagnosis of squamous odontogenic tumor a rare, benign epithelial odontogenic neoplasm. The clinical, radiographic and morphologic features of the squamous odontogenic tumor are discussed.

Squamous odontogenic tumor of the mandible: a case report demonstrating immunoexpression of Notch1, 3, 4, Jagged1 and Delta1.

BACKGROUND: Squamous odontogenic tumor (SOT) is a rare benign odontogenic epithelial neoplasm. A slow-growing painless expansive swelling is the common presenting symptom. Histopathologically, SOT can be easily misdiagnosed as an acanthomatous ameloblastoma. Although Notch receptors and ligands have been shown to play a role in cell fate decisions in ameloblastomas, the role of these cell signaling molecules in SOT is unknown. CASE REPORT: This paper describes a case of SOT affecting the anterior mandible of a 10-year-old Indian female. The patient was treated by local surgical excision and there has been no follow-up clinical record of recurrence 5 years after primary treatment. Histo?pathological examination revealed a solid, locally-infiltrative neoplasm composed of bland-looking squamatoid islands scattered in a mature fibrous connective tissue stroma and the diagnosis was SOT. Immunohistochemical evaluation showed positive reactivity of varying intensity in the neoplastic epithelial cells for Notch1, Notch3, Notch4, and their ligands Jagged1 and Delta1. Expression patterns showed considerable overlap. No immunoreactivity was detected for Notch2 and Jagged2. CONCLUSIONS: Present findings suggest that Notch receptors and their ligands play differential roles in the cytodifferentiation of SOT.

Tumor odontogénico de células escamosas asociado a un quiste residual / Squamous cell odontogenic tumor associated to a residual cyst

El tumor odontogénico de células escamosas (TOCE) es una neoplasia sumamente infrecuente, derivada de restos de la lámina dental o restos de Malassez. Esta lesión denota una expresividad variable. Se presenta el caso de una mujer de 72 años con un aumento de volumen asintomático en el lado izquierdo del maxilar inferior con diagnóstico presuntivo de quiste residual y diagnóstico histopatológico de TOCE. Se discute y actualiza su presentación clínica, radiográfica y su tratamiento y se actualiza la información sobre la patología.

Tumor odontogénico de células escamosas asociado a un quiste residual / Squamous cell odontogenic tumor associated to a residual cyst

El tumor odontogénico de células escamosas (TOCE) es una neoplasia sumamente infrecuente, derivada de restos de la lámina dental o restos de Malassez. Esta lesión denota una expresividad variable. Se presenta el caso de una mujer de 72 años con un aumento de volumen asintomático en el lado izquierdo del maxilar inferior con diagnóstico presuntivo de quiste residual y diagnóstico histopatológico de TOCE. Se discute y actualiza su presentación clínica, radiográfica y su tratamiento y se actualiza la información sobre la patología.(AU)

[Benign epithelial odontogenic tumors]. / Benigne epitheliale odontogene Tumoren.

The group of benign epithelial odontogenic tumors consists of the four member types of the ameloblastoma family (solid/multicystic, extraosseous/peripheral, desmoplastic, unicystic), squamous odontogenic tumors, calcifying odontogenic tumors, adenomatoid odontogenic tumors, and keratocystic odontogenic tumors, the former "keratocysts" that were recently reclassified by the World Health Organization and are now regarded as tumors. The latter are by far the most frequent tumors in this group, followed by solid/multicystic ameloblastoma. Although the etiology of these lesions is still unknown, a close relationship to normal tooth development is obvious, which is partially imitated by some tumors. Despite some similarities to each other, at least in part, the biological behavior of these lesions is quite different, as are treatment modalities. The diagnosis is essentially based on localization (intraosseous vs. extraosseous/peripheral) and histology, whereupon the correlation of histological findings with radiographic morphology may be of additional diagnostic value. Because of the range of variation, immunohistochemical investigations are not helpful in diagnosing a particular case.

Localized gingival enlargement associated with alveolar process expansion: peripheral ossifying fibroma coincident with central odontogenic fibroma.

BACKGROUND: Despite the common occurrence of localized gingival enlargements, which often represent reactive lesions, the temporal and spatial association of such a lesion with a central jaw lesion has not been reported. The purpose of this case report is to present the exceptional combination of a peripheral ossifying fibroma and a central odontogenic fibroma. The differential diagnosis and management of each lesion is reviewed. METHODS: A 45-year-old black female presented with a chief complaint of a painless protuberance in the left mandible of 1-year duration. Clinical and radiographic examination revealed a gingival enlargement localized between teeth #21 and #23 and a multilocular radiolucent lesion with radiopaque foci in the same area. Excisional biopsy of the gingival lesion and incisional biopsy of the central lesion were performed, and specimens were submitted for histopathological analysis. RESULTS: Biopsy of the gingival lesion revealed stratified squamous epithelium and highly cellular fibroblastic component presenting central areas of calcification, features consistent with a diagnosis of peripheral ossifying fibroma. The central lesion was characterized by cellular fibrous tissue admixed with rests of odontogenic epithelium and few calcification areas, features consistent with a diagnosis of central odontogenic fibroma/World Health Organization type. Subsequently, the central lesion was enucleated. After 1-year follow-up, no recurrence has been observed. CONCLUSIONS: The combination of a rare central lesion with a common gingival lesion may present unique diagnostic and therapeutic challenges. Clinician awareness regarding the possibility of such a combined presentation and its implications will help to ensure optimal treatment outcomes.

Aggressive maxillary squamous odontogenic tumour in a child: histological dilemma and adaptative surgical behaviour.

A case of a maxillary osteolytic tumour is described in a 9-year-old boy. Histological analysis led to an initial diagnosis of benign squamous odontogenic tumour, although this was not straightforward due to swelling, and cellular pseudo-malignant and non-specific signs. Because of the young age of the patient, a local surgical tumourectomy was first chosen with respect to the mixed dentition. For 10 months, the evolution was satisfactory. Then, a very aggressive tumoural recurrence with lip and palate infiltration led to doubts as to the histologic nature of the tumour. Efficient collaboration between several specialized pathologist teams finally confirmed that this was a squamous odontogenic tumour but in a very aggressive form. Radical surgery was then carried out.

Primary intraosseous carcinomas of the jaws arising within an odontogenic cyst, ameloblastoma, and de novo: report of new cases with reconstruction considerations.

Primary intraosseous carcinoma (PIOC) of the jaws has been rarely reported. The authors report 3 new cases of PIOC arising within an odontogenic cyst, ameloblastoma, and de novo origin, respectively. Surgeons should appreciate the elevated aggressiveness of this tumor despite adequate surgical treatment. The authors recommend initial aggressive surgical treatment to decrease the local recurrence rate.