Chondroblastic and fibroblastic osteosarcoma of the jaws: Report of two cases and review of literature.

This study aims to report of two variants of gnathic osteosarcoma with highlights on the varied histopathological presentation of osteosarcomas (OS). OS present with diverse histological appearances. Despite significant advances in molecular pathogenesis and biomarkers, clinicopathologic correlation is still considered as the important criteria in diagnosis. Chondroblastic osteosarcoma in a 52-year-old female and fibroblastic osteosarcoma in a 35-year-old female. Osteosarcoma is a relatively rare disease of the oral and maxillofacial region. Regular screening and follow-up is highly recommended, as recurrence rates are higher. Thorough understanding of the histologic spectrum of osteosarcoma reduces the diagnostic difficulties in categorizing the OS and separating these neoplasms from benign bone diseases.

Tumor central de células gigantes en mandíbula: reporte de un caso clínico / Central giant-cell tumor of the jaw: report of a clinical case

Las lesiones de células gigantes son un grupo de diversas patologías de etiología desconocida, poco entendidas y que representan un reto para el diagnóstico, por el hecho de presentar características clínicas e histológicas semejantes. En este grupo están: tumor de células gigantes, granuloma central de células gigantes, granuloma periféricode células gigantes, tumor pardo del hiperparatiroidismo, querubismo y quiste óseo aneurismático. A seguir, describiremos un caso de tumor de células gigantes en el hueso mandibular removido a través de procedimiento quirúrgico. Paciente del género femenino de 27 años, raza blanca, se presentó al Servicio de Cirugía y Traumatología del Hospital Escuela ®José Francisco de San Martín¼, en diciembre de 2013, reclamando hinchazón en la región anterior de maxilar inferior. Radiográficamente de observó lesión osteolítica con pérdida total de hueso de la zona basal en la región anterior, a nivel de la sínfisis mentoniana, que abarcaba desde la pieza dentaria 45 a 34. Se optó por realizar una biopsia incisional, obteniendo de esta forma el diagnóstico definitivo y así se pudo realizar posteriormente el procedimientoquirúrgico correspondiente. A partir de los resultados de la biopsia con los aspectos radiográfi cos en las diferentes etapas de la lesión, concluimos que se trataba de un caso de tumor central de células gigantes. Es prudente la realización de una biopsia incisional para el diagnóstico histopatológico defi nitivo de determinadas lesiones parala ejecución de un plan de tratamiento correcto y seguro...

A new subtype of high-grade mandibular osteosarcoma with RASAL1/MDM2 amplification.

In contrast to long bone osteosarcoma, mandibular osteosarcoma is highly heterogeneous and morphologically overlaps with benign tumors, obscuring diagnosis and treatment selection. Molecular characterization is difficult due to the paucity of available specimens of this rare disease. We aimed to characterize the spectrum of mandibular osteosarcoma using immunohistochemistry and molecular techniques (quantitative polymerase chain reaction and sequencing) and compare them with benign fibro-osseous lesions. Forty-nine paraffin-embedded mandible osteosarcoma tissue samples were collected retrospectively and compared with 10 fibrous dysplasia and 15 ossifying fibroma cases. These were analyzed for molecular markers thought to differ between the different diseases and subtypes: MDM2 (murine double-minute type 2) overexpression, GNAS (guanine nucleotide-binding protein/α subunit) mutations, and amplification of MDM2 and/or RASAL1 (RAS protein activator like 1). Five fibroblastic high-grade osteosarcoma subtypes showed MDM2 amplification, including 2 with a microscopic appearance of high-grade osteosarcoma with part low-grade osteosarcoma (differentiated/dedifferentiated osteosarcoma) and MDM2 overexpression. The other 3 contained a coamplification of MDM2 and RASAL1, a signature also described for juvenile ossifying fibroma, with no overexpression of MDM2. These were of the giant cell-rich high-grade osteosarcoma, with areas mimicking juvenile ossifying fibroma (ossifying fibroma-like osteosarcoma). Our results show that some diagnosed high-grade osteosarcomas are differentiated/dedifferentiated osteosarcomas and harbor an overexpression and amplification of MDM2. In addition, juvenile ossifying fibromas can potentially evolve into giant cell-rich high-grade osteosarcomas and are characterized by a RASAL1 amplification (osteosarcoma with juvenile ossifying fibroma-like genotype). Thus, the presence of a RASAL1 amplification in ossifying fibroma may indicate a requirement for closer follow-up and more aggressive management.

Tumor desmoide extraabdominal: descripción de un caso y revisión de la bibliografía / Extraabdominal desmoid tumor: case description and review of the literature

Objetivo: presentar un caso clínico de tumor desmoide de maxilar inferior en un niño de 4 años, analizar las características histológicas y el comportamiento clínico e informar el diagnóstico y tratamiento. Caso clínico: un varón de 4 años de edad, sin antecedentes patológicos, fue atendido por presentar tumoración en región mandibular izquierda de 7 meses de evolución. Se realizaron ecografía, tomografía axial computarizada y resonancia magnética, las cuales describieron masa ocupante de espacio sólida, de límites escasamente definidos con resorción perióstica. La biopsia informó neurofibroma submandibular. Se efectuó la extirpación quirúrgica que confirmó el diagnóstico de tumor desmoide extraabdominal. Conclusiones: el tumor desmoide es de histología benigna pero infiltrante, con una tasa de recurrencia alta, por lo que el tratamiento indicado es la resección quirúrgica amplia. La radioterapia puede controlar las lesiones irresecables.

Osteochondroma of the mandibular condyle: a classification system based on computed tomographic appearances.

OBJECTIVE: The objectives of this study were to introduce the classification of osteochondroma of the mandibular condyle based on computed tomographic images and to present our treatment experiences. MATERIALS AND METHODS: From January 2002 and December 2012, a total of 61 patients with condylar osteochondroma were treated in our division. Both clinical and radiologic aspects were reviewed. The average follow-up period was 24.3 months with a range of 6 to 120 months. RESULTS: Two types of condylar osteochondroma were presented: type 1 (protruding expansion) in 50 patients (82.0%) and type 2 (globular expansion) in 11 patients (18.0%). Type 1 condylar osteochondroma presented 5 forms: anterior/anteromedial (58%), posterior/posteromedial (6%), medial (16%), lateral (6%), and gigantic (14%). Local resection was performed on patients with type 1 condylar osteochondroma. Subtotal condylectomy/total condylectomy using costochondral graft reconstruction with/without orthognathic surgeries was performed on patients with type 2 condylar osteochondroma. During the follow-up period, tumor reformation, condyle absorption, and new deformity were not detected. The patients almost reattained facial symmetry. CONCLUSIONS: Preoperative classification based on computed tomographic images will help surgeons to choose the suitable surgical procedure to treat the condylar osteochondroma.

Calcifying odontogenic cyst: dilemma in classification.

Calcifying odontogenic cyst (COC) shows extreme diversity in its clinical and histopathological features, as well as in its biological behavior. Because of this diversity, there has been confusion and disagreement on the terminology and classification of this lesion. Attempts to classify COC can be divided into two concepts: monistic and dualistic. We present a case of COC with coexisting histopathologic features of a cyst and a neoplasm, thus posing a dilemma in the terminology used to categorize and classify it.

Fibro-odontoma ameloblástico de la mandíbula / Ameloblastic fibro-odontoma of the jaw

El fibro-odontoma ameloblástico (FOA) es un tumor odontogénico poco frecuente que afecta a personas jóvenes, sin predilección por género y usualmente se localiza en la zona posterior de la mandíbula. Clínicamente el paciente puede presentar retraso en la erupción dentaria acompañado de aumento de volumen indoloro. Radiográficamente es una lesión radiolúcida uni o multilocular de tamaño variable con áreas radiopacas. El tratamiento varía de acuerdo a su tamaño y se debe tener especial cuidado en la decisión de conservar o no los dientes adyacentes, ya que existe la posibilidad de recurrencia de la lesión. Presentamos un caso de FOA de gran tamaño en un paciente de 4 años. Se describen las características específicas, histológicas y clínicas. Se discute en la conducta terapéutica conservadora y la necesidad de control postoperatorio prolongado en el tiempo(AU)

Ameloblastic fibro-odontoma (AFO) is an infrequent odontogenic tumor that affects young people of either sex and is usually located in the posterior mandibular zone. Clinically, the patient might present a delay in dental eruption with a painless increase in volume. Radiographically, it is a uni- or multilocular radiolucent lesion of variable size with radiopaque areas. Treatment varies with tumor size and the decision to conserve the adjacent teeth must be weighed carefully due to the possibility of recurrence. The case of a large AFO in a 4-year-old patient is reported. The specific histological and clinical characteristics are described and the conservative therapy and need for long-term postoperative follow-up are discussed(AU)

[Temporomandibular joint primitive tumors and pseudo tumors]. / Pseudotumeurs et tumeurs primitives de l'articulation temporomandibulaire.

The temporomandibular joint (TMJ) can be the site of bone, cartilaginous, or synovial tumors. There is no well-defined histological classification. We listed all benign tumors, malignant primitive tumors, and rare pseudo tumors of the TMJ. We provide a list to help for the diagnosis and the differential diagnosis of non-tumoral lesions by far the most frequent.

Radiographic analysis of ameloblastoma: a retrospective study.

BACKGROUND: Ameloblastoma is benign odontogenic tumor, usually affecting the posterior region of mandible. It is seen in the third to fifth decades of life. Radiographically the lesion is variable in appearance and may be unilocular or multilocular, with well-defined cortical borders in the mandible and ill-defined margins in the maxilla. OBJECTIVE: To analyze cases of ameloblastoma, with emphasis on the radiographic findings. We also review the current literature briefly and discuss the clinical and radiographic findings. MATERIALS AND METHODS: The present hospital-based retrospective study was conducted by reviewing the clinical and radiographic records of ameloblastoma cases from 2009 to 2011, available in the archives of the department. The data of a total of 14 patients were analyzed. RESULTS: We observed that the patients affected with ameloblastoma were in the age-group of 19-68 years. The male: female ratio was 1.3:1. The mandible (78.57%) was more commonly affected than the maxilla (14.28%). Six patients (42.86%) had unilateral involvement and eight cases (57.14%) had bilateral involvement. The multilocular and unilocular types of ameloblastoma were noted in 12 (85.72%) and 2 cases (14.28%), respectively. The soap-bubble (50.00%), spider-web (21.43%), and honeycomb (14.28%) appearances were seen in the multilocular variety. Root resorption of variable degree was distinctly observed in 11 cases (78.57%). CONCLUSION: Radiographs are an important aid for the diagnosis of oral lesions of various types, especially those that involve bone. It is important for the practicing clinicians to know the salient features of ameloblastoma which are peculiar to the local population.

Ameloblastoma in children: should we be radical?

Ameloblastoma is the most common aggressive benign odontogenic tumor of the jaws. The tumor is often asymptomatic, presenting as a slowly enlarging facial swelling. The physical presence of the tumor may cause symptoms such as pain, ulceration, loosening of teeth, or malocclusion. Ameloblastoma is a locally destructive tumor with a propensity for recurrence if not entirely excised. It is seen in all age groups, but the lesion is most commonly diagnosed in the third and fourth decades. The tumor is considered a rarity in the young age group. The treatment of ameloblastoma is still controversial and presents some special problems in children like the growth of the jaw, the different incidence, behavior and prognosis of the tumor in children, which make the surgical considerations different from adults. Some reports have encouraged conservative treatment for ameloblastoma in children.

Thirty years clinicopathological study of 60 calcifying cystic odontogenic tumors in Iranian population.

AIM: To reevaluate all cases previously diagnosed as calcifying odontogenic cyst (COC) from the archives of the Department of Oral Pathology at Mashhad Faculty of Dentistry from 1978 to 2008. MATERIALS AND METHODS: Clinical histories and microscopic slides of all cases diagnosed as COC were reviewed and analyzed statistically. The information about patients such as age, sex, clinical features, radiographic view and other necessary data was gathered from biopsy files. RESULTS: The majority of cases occurred in mandible and four cases were peripherally. Thirteen cases were associated with other odontogenic tumors and 11 cases were odontoma type. In two cases, recurrence was reported after surgical treatment which underwent malignant transformation. CONCLUSION: Calcifying cystic odontogenic tumor (CCOT) in Iranian population occurs in younger age group with slightly male predilection and more mandibular, unicystic and simple type. CLINICAL SIGNIFICANCE: It is better to address the noncystic CCOT lesions with their specific terms in order to make histopathological characteristics clear.

Carcinoma mucoepidermoide central mandibular: presentación de dos casos / Mandibular central mucoepidermoid carcinoma: report of two cases

Los carcinomas mucoepidermoides centrales de los maxilares son lesiones raras. Comprenden el 15 por ciento de todos los tumores malignos que se presentan en los maxilares. A la fecha hay sólo alrededor de 200 casos publicados en la bibliografía. Se presentan dos casos de pacientes tratados en 2005-2006. Los carcinomas mucoepidermoides de los maxilares provocan síntomas inespecíficos y deben ser diagnosticados por biopsia incisional. Su tratamiento primario es quirúrgico, permaneciendo aún poco claro el rol de terapéuticas adyuvantes.

Histopathologic diversity of Gorlin's cyst: a study of four cases and review of literature.

AIM: The purpose of the present article is to discuss four different case reports of the so-called calcifying odontogenic cyst and highlight the histopathological diversity of the same. BACKGROUND: Calcifying odontogenic cyst was first described by Gorlin et al in 1962. Ever since, its identification as a specific odontogenic lesion, controversies and confusions have existed regarding the relationship between cystic lesions and solid tumor masses that share cellular and histomorphologic features. Although several classifications were proposed, dilemma still persists regarding the nature of these lesions as cysts, neoplasms and even malignancies. CONCLUSION AND CLINICAL SIGNIFICANCE: The classifications discussed for the so-called calcifying odontogenic cyst by various authors have only added to further confusion rather than enlightening. Though many authors state that classifications remain only an academic exercise, it definitely has significance in treatment planning. Emphasis should, therefore, be laid on a universally accepted classification.

Notch4 overexpression in ameloblastoma correlates with the solid/multicystic phenotype.

OBJECTIVE: Notch signaling has been implicated in cell fate decisions during odontogenesis and tumorigenesis of some odontogenic neoplasms; however, its role in solid/multicystic (SA), unicystic (UA), and recurrent (RA) ameloblastoma remains unclear. The aim of this study was to determine Notch receptor and ligand expressions in these subtypes and to speculate on their significance. METHODS: Notch receptors (Notch1, 2, 3, 4) and ligands (Jagged1, 2, and Delta1) were examined immunohistochemically in SA (n = 23), UA (n = 22), and RA (n = 19). RESULTS: Notch4 overexpression in SA (n = 19/23; 82.6%) compared with UA (n = 1/22; 4.5%) or RA (n = 10/19; 52.6%) (P < .05) suggests positive correlation between Notch4 signaling and ameloblastomas with a solid/multicystic phenotype. Ligand (Jagged1 and Delta1) underexpression compared with their receptors (Notch1, 3, 4) (P < .05) and nonreactivity for Notch2 and Jagged2 in all 3 subsets suggests that ameloblastoma epithelium belongs to an earlier stage of differentiation (equivalent to inner enamel epithelium of developing tooth germ) before lineage commitment. CONCLUSION: Present findings suggest that Notch signaling molecules may play differing roles in the acquisition of different ameloblastoma phenotypes.

Aggressive fibromatosis of the head and neck: a new classification based on a literature review over 40 years (1968-2008).

BACKGROUND: Fibromatosis is an aggressive fibrous tumor of unknown etiology that is, in some cases, lethal. Until now, there has been no particular classification for the head and neck. Therefore, the aim of the present study was to review the current literature in order to propose a new classification for future studies. METHODS: An evidence-based literature review was conducted from the last 40 years regarding aggressive fibromatosis in the head and neck. Studies that summarized patients' data without including individual data were excluded. RESULTS: Between 1968 and 2008, 179 cases with aggressive fibromatosis of the head and neck were published. The male to female ratio was 91 to 82 with a mean age of 16.87 years, and 57.32% of the described cases that involved the head and neck were found in patients under 11 years. The most common localization was the mandible, followed by the neck. All together, 143 patients were followed up, and in 43 (30.07%), a recurrence was seen. CONCLUSION: No clear prognostic factors for recurrence (age, sex, or localization) were observed. A new classification with regard to hormone receptors and bone involvement could improve the understanding of risk factors and thereby assist in future studies.

Unicystic plexiform ameloblastoma: an insight for pediatric dentists.

Ameloblastomas have been categorized broadly into three biologic variants: cystic (unicystic), solid, and peripheral. The term plexiform unicystic ameloblastoma refers to a pattern of epithelial proliferation that has been described in cystic lesions of the jaws. Although the histology suggests that cystic ameloblastomas follow a biologically low-grade course, recent evidence suggests that they may often behave clinically as biologically aggressive tumors. This is supported by the high incidence of cortical perforation, tooth resorption, lesion size, bony destruction, and a high rate of recurrence after simple enucleation. This article tries to provide an insight for pediatric dentists regarding this biologically distinct entity. A literature review on the topic has been added along with a case report highlighting the state-of-the-art approach and management of such ameloblastomas, in pediatric patients.

Odontogenic tumors in childhood: a retrospective study of 86 treated cases. Importance of a correct histopathologic diagnosis.

The odontogenic tumors are an unusual group of lesions of the jaws derived from embryologic tooth-forming tissues and presenting in a large number of histologic patterns. More common in pediatric age and adolescence than in adult age, the odontogenic tumors can be observed casually or after the appearance of nonspecific symptoms. Because of their slow-growth tendency, usually they do not cause pain. The odontogenic tumors grow in the jaw, through the haversian system, without metastasis but with and high probability of relapse. A retrospective study of 86 cases treated between 1997 and 2005 is reported. The percent of diagnosed cases that were benign was 98.8%, and just one case of malign neoplasm is reported. The most frequent tumor accounted for in the reported sample was odontoma (39.5%) followed by odontogenic fibroma (12.8%). Ameloblastoma and myxoma showed the same incidence (11.6%). Early diagnosis, together with a correct histologic diagnosis, allows a preservative and effective surgical treatment and is necessary to reduce the risk of relapse.

[Benign odontogenic ectomesenchymal tumors]. / Benigne odontogene ektomesenchymale Tumoren.

The group of odontogenic ectomesenchymal tumors consists of odontogenic fibroma (epithelium-rich and epithelium-poor types), odontogenic myxoma, and cementoblastoma. Whereas odontogenic fibromas and cementoblastomas are very rare lesions, odontogenic myxoma is the fourth common odontogenic tumor, preceded only by keratocystic odontogenic tumor, the odontomas, and ameloblastoma. The diagnosis of cementoblastoma rests on its connection to the root of a tooth. The differentiation of odontogenic fibroma and myxoma from other lesions, especially from normal structures such as dental follicles and papillae, may be challenging if the X-ray appearance (localized osteolysis containing a tooth) is not appreciated and subtle histological clues (remainders of inner enamel epithelium at the surface of the lesion, dentin fragments) are not properly recognized. While odontogenic fibromas have almost no tendency for recurrence and are treated by enucleation or local excision, cementoblastomas and especially odontogenic myxomas have a high percentage of recurrence if intralesional procedures are applied. Hence, complete resection with free margins is recommended--at least for larger odontogenic myxomas and, especially, lesions in the maxilla--to prevent further extension to the orbita or base of the skull.