Synchronous cemento-ossifying fibromas: a systematic review.

BACKGROUND: This systematic review aimed to incorporate published data regarding synchronous cemento-ossifying fibromas (COF), with an analysis of their demographic and clinicopathological characteristics. MATERIAL AND METHODS: Case reports and case series of synchronous COF were searched in PubMed, Web of Science, Scopus, EMBASE, and LILACS according to the PRISMA (2020) statement. Also, a manual search was carried out and the grey literature was assessed. A descriptive statistical analysis was performed. RESULTS: Nineteen studies comprising 20 cases of synchronous COF were included. The mean age at diagnosis was 35 years (±13.8), with a predominance of female patients (n=12/60%). In 13 cases (65%) the mandible and the maxilla were affected simultaneously. In two cases (10%) first-degree relatives (parents or siblings) had been previously diagnosed with COF. The diagnostic hypotheses were reported in 8 cases (40%), with florid cemento-osseous dysplasia, ameloblastic fibroodontoma, calcifying cystic odontogenic tumor, osteoma and cementoblastoma being cited in the differential diagnosis. Among the cases with details about management (n=17), eleven were treated by surgical enucleation and/or excision (64.7%). Follow-up was provided for 10 cases (50%), with a mean period of 44.7±62.19 months. Recurrence occurred in three of informed cases. CONCLUSIONS: Synchronous manifestation of COF is rare. Female patients around the 3rd decade of life are more commonly affected. Bilateral involvement of the mandible and maxilla is the most common clinical presentation.

Central Myoepithelioma of the Maxilla.

Myoepithelioma is a benign salivary gland tumor. Central myoepitheliomas are very rare. The aim of this report was to describe a case of maxillary myoepithelioma. A 14-year-old female patient presented with an multilocular lesion in the anterior maxilla, with nearly 8 months of duration. The lesion was asymptomatic, and the patient's dental history was unremarkable. The diagnostic hypothesis was an odontogenic tumor. Biopsy specimen consisted of nests of plasmacytoid cells in a myxoid stroma without duct formation. No cellular atypia or bone and cartilage formation were noted. The neoplastic cells were positive for Pan-cytokeratin, S100, CK7, and CK8. The final diagnosis was myoepithelioma. The patient was treated by surgical excision followed by bone curettage, and no signs of recurrence were found after 8 years of treatment.

Tumor Odontogénico Adenomatoide Quístico, presentación inusual. Reporte de dos casos / Cystic Adenomatoid Odontogenic Tumor, unusual presentation. Report of two cases

RESUMEN: El tumor odontogénico adenomatoide es un tumor benigno, poco común, que corresponde al 2-7% de los tumores odontogénicos. Se presenta con mayor prevalencia en la segunda década de vida y se ubica preferentemente en maxilar anterior. Es una lesión encapsulada, no agresiva, de crecimiento lento, que puede pasar desapercibida durante años. Deriva del epitelio odontogénico, y se caracteriza histológicamente por células epiteliales dispuestas en nidos, remolinos o cordones con escaso tejido conjuntivo. Puede presentar estructuras canaliculares un patrón ductiforme, con calcificaciones y presenta cápsula. El presente estudio reporta dos casos, el primero es una mujer de 30 años y el segundo el de un hombre de 36 años, ambos con un tumor odontogénico adenomatoide ubicado en zona maxilar anterior derecha, asintomático, sin asociación con diente incluido y de presentación quística a nivel imagenológico e histopatológico. Se discuten características clínicas, radiográficas, histopatológicas y terapéuticas de los casos.

ABSTRACT: Adenomatoid odontogenic tumor is a benign, rare tumor that accounts for 2-7% of odontogenic tumors. It appears with greater prevalence in the second decade of life and is located preferentially in the anterior maxilla. It is a slow-growing, non-aggressive, encapsulated lesion that can go unnoticed for years. It is derived from the odontogenic epithelium, and is characterized histologically by epithelial cells arranged in nests, eddies, or cords with little connective tissue. It may have canalicular structures and a ductiform pattern, with calcifications and it has a capsule. The present study reports two cases, the first is a 30-year-old woman and the second is a 36-year-old man, both with an adenomatoid odontogenic tumor located in the right anterior maxillary area, asymptomatic, without association with an included tooth and cystic presentation at the imaging and histopathological levels. The clinical, radiographic, histopathological and therapeutic characteristics of the cases are discussed.

Pindborg tumor associated with a supernumerary tooth: a case report

The calcifying epithelial odontogenic tumor is a rare benign neoplasm that accounts for approximately 1% of all odontogenic tumors. Most of the cases occur in the posterior mandible, and a few involve the maxilla. Despite their relatively indolent biological behavior, tumors in the maxilla tend to grow fast. We report the case of a 33-year-old female patient exhibiting swelling in the right maxilla. An isodense area associated with an impacted supernumerary tooth was found on imaging examination. The histopathologic diagnosis was a calcifying epithelial odontogenic tumor. The treatment of choice was surgical removal of the lesion and associated dental elements. The patient has been followed up for 11 months and shows no signs of recurrence. Besides describing this case, we reviewed the literature on the association of calcifying epithelial odontogenic tumors with supernumerary teeth and found two case reports addressing this subject.

Fully digital pathology laboratory routine and remote reporting of oral and maxillofacial diagnosis during the COVID-19 pandemic: a validation study.

The role of digital pathology in remote reporting has seen an increase during the COVID-19 pandemic. Recently, recommendations had been made regarding the urgent need of reorganizing head and neck cancer diagnostic services to provide a safe work environment for the staff. A total of 162 glass slides from 109 patients over a period of 5 weeks were included in this validation and were assessed by all pathologists in both analyses (digital and conventional) to allow intraobserver comparison. The intraobserver agreement between the digital method (DM) and conventional method (CM) was considered almost perfect (κ ranged from 0.85 to 0.98, with 95% CI, ranging from 0.81 to 1). The most significant and frequent disagreements within trainees encompassed epithelial dysplasia grading and differentiation among severe dysplasia (carcinoma in situ) and oral squamous cell carcinoma. The most frequent pitfall from DM was lag in screen mirroring. The lack of details of inflammatory cells and the need for a higher magnification to assess dysplasia were pointed in one case each. The COVID-19 crisis has accelerated and consolidated the use of online meeting tools, which would be a valuable resource even in the post-pandemic scenario. Adaptation in laboratory workflow, the advent of digital pathology and remote reporting can mitigate the impact of similar future disruptions to the oral and maxillofacial pathology laboratory workflow avoiding delays in diagnosis and report, to facilitate timely management of head and neck cancer patients. Graphical abstract.

Aggressive Intraosseous Myofibroma of the Maxilla: Report of a Rare Case and Literature Review.

Myofibroma (MF) is a benign mesenchymal myofibroblast-derived tumor, which occurs most frequently in children, and rarely affects the maxilla. We reported a case of an aggressive intraosseous lesion found in the maxilla of a 9-year-old female child. Intraorally, the swelling extended from tooth 12 to 16, causing displacement of teeth 13, 14, and 15. Computed tomography revealed a large osteolytic lesion causing thinning and cortical erosion. Microscopically, the lesion showed a proliferation of spindle-shaped cells, with elongated nuclei and eosinophilic cytoplasm, arranged in interlaced fascicles. The immunohistochemical analysis revealed cytoplasmic positivity for α-SMA and HHF-35, and negativity for desmin, laminin, S-100, ß-catenin, and CD34. Ki-67 was positive in 8% of tumor cells. The diagnosis was MF. Herein, we describe an additional case of central MF arising in the maxilla, including clinical, imaging, microscopical, and immunohistochemical features, as well as a review of the literature.

Quality of Life and Maxillary Defects: A Cross-Sectional Study / Calidad de Vida y Defectos Maxilares: Un Estudio Transversal

ABSTRACT: There has been little discussion about the quality of life of patients with maxillary defects. This article evaluates the issues related to the condition. We performed a cross-sectional study of patients with maxillary defects from referral centers in Brazil. To avoid subject burden, a questionnaire was developed, based on questions from seven instruments, which dealt with domains and conclusions that were similar to those from other studies. The predictor variable was the patients' score for each question. The outcome measure was the presence of the best-ranked items on the questionnaire as the impact factor. Six experts assessed these items and suggested which questions to include or exclude. Patients scored each item according to its occurrence and importance. Descriptive statistics and the items' rank according to the impact factor were computed to determine whether there is a comprehensive instrument available. Thirteen patients and six professionals were included in this study. The patients' age ranged from 24 to 72 years (mean (standard deviation, SD), 50.41 (14.46) years). We obtained a 60-item instrument from the selected questionnaires and subject interviews. Only 12 (37.5 %) out of the 32 best-rated items were verified by the existing instruments, two (6.25 %) were suggested by professionals and 18 (56.25 %) were conclusions from other studies. To the best of our knowledge, this is the first study to provide many features related to the quality of life in patients with maxillary defects.

RESUMEN: Existe escasa discusión en la literatura sobre la calidad de vida de los pacientes con defectos maxilares. Este artículo evalúa los problemas relacionados con esta condición. Realizamos un estudio transversal de pacientes con defectos maxilares de centros de referencia en Brasil. Se desarrolló un cuestionario basado en preguntas de siete instrumentos, que trataba sobre dominios y conclusiones similares a las de otros estudios. La variable de estimación fue la puntuación de los pacientes para cada pregunta. La medida de resultado fue la presencia de los elementos mejor clasificados en el cuestionario como factor de impacto. Seis expertos evaluaron estos ítems y sugirieron qué preguntas incluir o excluir. Los pacientes puntuaron cada ítem según su ocurrencia e importancia. Se calcularon las estadísticas descriptivas y la clasificación de los ítems según el factor de impacto, para determinar si existe un instrumento completo. Trece pacientes y seis profesionales fueron incluidos en este estudio. La edad de los pacientes osciló entre 24 y 72 años [media (desviación estándar), 50,41 (14,46) años]. Obtuvimos un instrumento de 60 ítems de los cuestionarios y entrevistas de temas seleccionados. Solo 12 (37,5 %) de los 32 ítems mejor calificados se verificaron de acuerdo a los instrumentos existentes, dos (6,25 %) fueron sugeridos por profesionales y 18 (56,25 %) fueron conclusiones de otros estudios. De acuerdo a nuestro conocimiento, este es el primer estudio que proporciona características relacionadas con la calidad de vida en pacientes con defectos maxilares.

Odontogenic Carcinoma With Dentinoid in Long-Term Follow-up With 2 Recurrences.

Dentinoid has been mentioned as a frequent component in several types of benign odontogenic tumors; however, there are some other very rare dentinoid-producing odontogenic tumors that have been described, which are not recognized in the current World Health Organization Histological Classification of Odontogenic Tumours. In this context, we report an unusual malignant odontogenic tumor containing dentinoid located in the left maxilla of a 41-year-old man. The lesion was initially diagnosed and treated as a cemento-ossifying fibroma. After 7 years, a tumor was noted at the same location and was diagnosed as pleomorphic adenoma. The patient developed a new lesion 2 years later. Histological features included an epithelial proliferation of basaloid and clear cells, some with peripheral palisading, which were scattered both in a fibrous stroma and within an amorphous eosinophilic dentinoid product. Because of doubts about the first 2 diagnoses and the current situation, all histopathological slides were reviewed in our service as a consultation case, and the findings were consistent with the diagnosis of an odontogenic carcinoma with dentinoid. Immunohistochemical analysis was performed and an ultrastructural study by scanning electronic microscopy and energy-dispersive X-ray microanalysis was made to characterize dentinoid material. After 1 year of follow-up, the patient is alive and free of the disease. This case highlights the wide variability regarding cytological evidence of malignancy, and adds a new case of odontogenic carcinoma with dentinoid, which represents a distinct entity with locally aggressive behavior and should be considered be included in a future World Health Organization Histological Classification of Tumours.

A Rare Case of an Aggressive Clear Cell Variant of Calcifying Epithelial Odontogenic Tumor in the Posterior Maxilla.

A clear cell variant of calcifying epithelial odontogenic tumor is a rare benign odontogenic neoplasm, accounting for 33 cases described in the literature. In this article, we report a challenging example of clear cell variant of calcifying epithelial odontogenic tumor of the posterior maxilla in a 45-year-old female patient showing locally aggressive growth and recurrence. Microscopically, islands of polyhedral cells containing abundant cytoplasm, well-developed intercellular bridges blended with clear cells were observed. The nuclei were frequently pleomorphic and permeated by hyaline calcified material. Immunohistochemistry revealed positivity for pan-cytokeratin (AE1/AE3), cytokeratins (CK-14 and CK-19), Bcl-2, p53, and p63. The Ki-67 proliferative index was ~10%. As odontogenic tumors are rare, when a significant clear cell component is observed, the differential diagnosis with other lesions of the jaws with similar morphology, including other odontogenic tumors with prominent clear cell component, clear cell odontogenic carcinomas, and metastatic tumors, is difficult.

Oral and maxillofacial cancer in pediatric patients: 30 years experience from a Brazilian reference center.

OBJECTIVE: The aim of this study is to determine the relative frequency, demographic distribution and clinicopathological features of pediatric oral and maxillofacial cancer (POMC). METHODS: Medical records were retrospectively reviewed for all cancer cases diagnosed from 1986 to 2016 affecting patients aged 19 years and younger. Demographic variables, anatomical site, and histopathological diagnoses were collected and analyzed by descriptive statistics. RESULTS: Fifty-five (0.77%) POMCs were found among 7181 pediatric malignancies. Mean age at diagnosis was 8 years and patients aged 5-9 years presented the higher prevalence of malignant tumors (40%). White male patients were more frequently affected (78.18% and 65.45%, respectively). The most common cancer type was lymphomas (52.73%) followed by sarcomas (27.27%) and carcinomas (20%). Burkitt lymphoma (32.73%), rhabdomyosarcoma (14.55%), diffuse large B-cell lymphoma (9.09%), and mucoepidermoid carcinoma (9.09%) were the most common histopathological diagnoses. The main affected anatomical site was the oropharynx (38.18%), followed by salivary glands (30.91%), maxillofacial bone (20%), and oral cavity (10.91%). CONCLUSION: POMC has a low incidence; however, highly aggressive tumors, such as lymphomas and sarcomas, are common in this scenario. A better knowledge about the clinicopathological distribution of POMC may contribute to early diagnosis and improve survival rates.

Neurofibromas of the oral and maxillofacial complex: A 48-year retrospective study.

Neurofibromas are benign neoplasms of the peripheral nerve sheath, characterized by the proliferation of Schwann cells, perineural cells and endoneural fibroblasts. Their occurrence in the oral and maxillofacial complex is uncommon. This study aimed to evaluate the clinical and histopathological characteristics of neurofibromas of the oral and maxillofacial complex excised at our institution over a 48-year period. Using light microscopy, two previously trained oral pathologists re-evaluated all hematoxylin and eosin slides. From a total of 15,375 cases diagnosed at a referred Oral Pathology Service, 24 cases were diagnosed as neurofibromas. Eighteen neurofibroma patients were female, with a mean age of 39.1 years. Three patients presenting neurofibromas exhibited neurofibromatosis type I. Clinically, most of the lesions presented as asymptomatic nodules, and the most frequent sites were the tongue (n = 6; 25.0%), gingiva (n = 6; 25.0%) and intraosseous maxillary bone region (n = 3; 12.5%). Histopathologically, the lesions were predominantly well delimited, exhibiting interlocking bundles of spindle-shaped cells that usually displayed wavy nuclei, associated with delicate collagen fibers. Thus, knowledge of their clinical and histopathological features by dentists and oral pathologists is essential for the correct diagnosis of these lesions.

Synchronous Calcifying Epithelial Odontogenic Tumor: Case Report and Analysis of the 5 Cases in the Literature.

Calcifying epithelial odontogenic tumor (CEOT), or Pindborg tumor, is infrequent and accounts for less than 1% of all odontogenic tumors. It is benign and usually asymptomatic but displays locally infiltrative and expansile behavior. Synchronous lesions affecting the jaws are exceedingly rare. We present a case of synchronous CEOTs affecting the maxilla and mandible and describe the clinical, radiographic, histologic, and immunohistochemical features. We further review five cases previously described in the literature.

Odontogenic carcinosarcoma: morphologic and immunohistochemical description of a case.

OBJECTIVES: The goal of this study was to describe an extremely rare case of odontogenic carcinosarcoma and compare the findings with those of a literature review. STUDY DESIGN: The clinical and pathologic data of an odontogenic carcinosarcoma affecting the posterior maxilla of a 42-year-old male patient was described. The lesion was immunostained for cell-cycle, cytokeratin, and mesenchymal markers. A review of literature from 1960 to 2017 was conducted in a search for similar well-documented case reports. Descriptive statistics were calculated to compare clinical and pathologic variables. RESULTS: In the reported case, the percentage of Ki-67-positive epithelial and mesenchymal cells was estimated as 40% and 25%, respectively. Epithelial cells were focally positive for cytokeratin 7, -8, -14, and -18, and diffusely positive for cytokeratin 19, p53, and p16. Mesenchymal cells were strongly positive for desmin, HHF-35, and vimentin. Our review showed that odontogenic carcinosarcoma is diagnosed mostly in the advanced stage. All patients with relapsed tumors had died as a result of the disease. CONCLUSIONS: Very few cases have been reported in the literature supporting that most odontogenic carcinosarcoma develop in the posterior mandible in a wide age range, without gender and racial predilections. Only one case of odontogenic carcinosarcoma in the maxilla other than the one described here has been reported. Until today, the best treatment remains unknown.

Central odontogenic fibroma: An updated systematic review of cases reported in the literature with emphasis on recurrence influencing factors.

PURPOSE: To integrate the available data published on central odontogenic fibroma (COF) into a comprehensive analysis of its clinical/radiologic/histological features. METHODS: An electronic search was undertaken in September 2017. Eligibility criteria included publications reporting cases of COF having enough clinical, radiological and histological information to confirm the diagnosis. Demographic data, lesion site and size, treatment approach, and recurrence were analysed. The cases included in the analysis presented follow-up time. Concerning recurrence analysis, tumour location, cortical bone perforation, lesion locularity, radiopacities, lesions associated with a tooth, tooth displacement, histological type and treatment used were evaluated. RESULTS: Eighty-three publications reporting 173 COFs were included. Lesions were slightly more prevalent in men than women (M: F - 1.13: 1); mean age was 31.6 years, with the highest prevalence in the second decade of life. Lesions were more prevalent in the posterior mandible. The difference in recurrence rate (when information about recurrence was provided) presented a statistically significant result for COF for location, cortical bone perforation and locularity of the lesion. CONCLUSIONS: Our paper highlights that patients with COF who present a lesion located in the maxilla with multilocular aspects and cortical bone perforation tend to show a higher recurrence rate.

Calcifying Odontogenic Cyst Associated With Dentigerous Cyst in a 15-Year-Old Girl.

Calcifying odontogenic cyst (COC) is a rare odontogenic cyst with ameloblastic epithelial lining containing clusters of ghost cells. COCs have been described in association with several odontogenic tumors, more commonly odontomas and rarely with dentigerous cyst (DC). In this article, we describe a case of COC associated with DC in a 15-year-old girl, who presented with a swelling on the right middle third of the face, producing facial asymmetry. Panoramic radiography showed a well-circumscribed, corticated, and unilocular radiolucency at the level of the right maxillary sinus, involving 2 unerupted premolars. The lesion was enucleated and histologically revealed a COC associated with DC, which presented mucous metaplasia. Immunohistochemical reactions were performed to better illustrate this rare synchronous occurrence of COC and DC, showing positivity for CK5, CK14, CK19, and p63 in both lesions. CK18 was negative in COC, and Bcl-2 was negative in DC. Periodic acid Schiff highlighted the mucous cells in the DC lining.

Comparative Study of the Minichromosome Maintenance Proteins Complex (MCM 4/5/6) in Ameloblastoma and Unicystic Ameloblastoma.

INTRODUCTION: Solid/conventional ameloblastoma (AM) and unicystic ameloblastoma (UAM) are the most frequent benign epithelial odontogenic tumors located in the maxillary region, and their treatment usually consists of extensive surgical resection. Therefore, it is relevant to study molecular markers to better understand the biological behavior of these tumors. The aim of this study was to describe and compare the expression of proteins related to cellular proliferation: Ki-67 and MCM4-6 complex. MATERIALS AND METHODS: An immunohistochemistry technique was performed, with antibodies against Ki-67, MCM4, MCM5, and MCM6, in 10 AM and 10 UAM tumors. The results were quantified using label index and analyzed statistically. RESULTS: AM and UAM had greater expression of MCM6, followed by MCM5, MCM4, and Ki-67 ( P < .05). Immunoexpression of Ki-67 and MCM5 was exclusively nuclear, whereas the expression of MCM4 and MCM6 was nuclear and cytoplasmic. CONCLUSION: The results suggest that MCM5 is a trustable cell proliferation marker with higher sensitivity compared with Ki-67 and may be useful to predict the biological behavior of AM and UAM. Despite this, further studies are necessary, including a correlation with clinical parameters to confirm these findings.

Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla.

Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.