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Objective: The present study aims to identify adenoid ameloblastoma (AdAM) from previously diagnosed cases of dentinogenic ghost cell tumor (DGCT), and gain insight to the possible relationship between AdAM and DGCT., Methods: DGCT cases diagnosed between 2006 and 2022 were re-examined with focus on the AdAM-like features., Results: A total of nine patients were included. Seven patients were males and two were females. The mean age was 38.0 ± 16.0 years. Five tumors occurred in the maxilla and four in the mandible, with a remarkable predilection for the posterior regions of both jaws. Microscopically, dentinoid material deposition was present in all cases. The ghost cells were absent in two cases. Rare ghost cells (<1%) were observed in three cases, and a higher proportion of ghost cells (5%-20%) were present in the remaining four cases. All cases showed prominent AdAM-like features, including duct-like structures, whorls/morules, and cribriform architecture. According to the diagnostic criteria proposed by the 2022 WHO classification, five cases without or with rare ghost cells were reclassified as AdAM. The other four cases including a higher proportion of ghost cells consisted of a mixture of DGCT and AdAM histopathologic patterns., Conclusion: Our results confirmed that the AdAM-like features had been largely overlooked in the diagnosis of DGCT at our institution in the past. Whilst a subset can now be more accurately classified as AdAM, some tumors showed overlapping morphological features between AdAM and DGCT, suggesting that the two may represent a spectrum of the same entity., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Purpose: To investigate the clinical characteristics of oral and maxillofacial tumors in children and adolescents., Methods: This is a retrospective study of patients who had oral and maxillofacial tumors under the age of 18 years and were treated at the Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology from January 1990 to July 2021 (31 y). Their general conditions, pathological diagnosis, gender, age, and anatomical location were counted to analyze their morbidity and composition characteristics., Results: This study contained 5405 cases, including 2903 male patients and 2502 female patients, with a median age of 9 years. Peak incidence was observed in the 14 to 18 years age group. The mandible (22.15%), maxilla (11.75%), and tongue (9.25%) were the most common sites of incidence. Malignant and intermediate type tumors accounted for 13.04%, benign tumors and tumor-like lesions for 55.67%, most often occurs in the maxillofacial bone, of which fibro-osseous lesions constitute an important part. Cysts accounted for 31.29%. Among the tumors occurring in the jaws, the most common malignant type was sarcoma, and ameloblastoma was the most common benign tumor. Malignant jaw tumors were mostly treated by resection, 10.64% by fibular flap reconstruction. While benign jaw tumors and tumor-like lesions were mostly treated by resection or curettage., Conclusions: The distribution of anatomical location and pathological types of oral and maxillofacial tumors in children has certain characteristics, so that the selection of their treatment options is different from that of adults due to the consideration of the growth and developmental characteristics of children., Competing Interests: The author reports no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Purpose: Benign fibro-osseous lesions (BFOLs) are heterogeneous intraosseous disease processes sharing overlapping histopathologic features and demonstrate a wide range of biological behavior. The aim of this study is to highlight the importance of clinical and radiological findings for the definitive histopathologic diagnosis of benign fibro-osseous lesions of the jaws., Materials and Methods: This is a cross-sectional retrospective study evaluating the three main groups of BFOLs: Cemento-osseous dysplasia, ossifying fibromas, and fibrous dysplasia. Previously diagnosed BFOL cases were searched for thoroughly from the archives of Istanbul University, Institute of Oncology, Department of Tumor Pathology in between 2005 and 2015. Clinical and radiological data of these cases were identified and recorded. The histopathologic features were reevaluated from the H&E-stained slides., Results: A total of 276 BFOL cases were identified and reevaluated for the study. The most common BFOL type was cemento-osseous dysplasia (n = 135), followed by cemento-ossifying fibroma (n = 77), fibrous dysplasia (n = 53) and juvenile ossifying fibroma (n = 11). The female/male ratio was 3.4:1 with a strong predilection for the 4th decade (n = 102). The mandible (n = 209) was the predominantly affected jaw. Prominent osteoblastic rimming around the bony lesion was commonly observed for cemento-ossifying fibroma (n = 60/77), followed by cemento-osseous dysplasia (n = 23/135). Predominant ossification patterns showed some differences among the groups. The most common radiological feature was the mixed radiolucent/radiopaque internal structure., Conclusion: Our results document various clinical, radiological and histopathologic findings of BFOLs. Even some histopathologic differences are observed, clinical and radiographic correlation is mandatory prior to the definitive histopathologic diagnosis of BFOLs for the prevention of possible misdiagnoses or inappropriate treatments., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Ameloblastoma is an odontogenic tumor occurring in jaws, with local aggressiveness and postoperative recurrence. This study was aim to investigate the clinical and radiographic risk factors for recurrence in ameloblastoma. Methods: Patients diagnosed with ameloblastoma between March 2009 and March 2019 were retrospectively analyzed. Clinical and Radiological data and follow-up records were collected. Survival analyses were performed by Kaplan-Meier and log-rank tests, as well as Cox proportional hazards model. Results: One hundred and fifty-eight patients (104 males and 54 females were enrolled. The overall recurrence rate for ameloblastoma was 13.29%, and 10.76% recurred within 5 years. Most of the tumors were located in mandible (86.71%), while the rest 21 cases were in maxilla (13.29%). More than half cases (55.06%) showed multilocular radiolucency, 61 cases (38.61%) showed unilocular radiolucency. Significant differences were found with amelobastoma recurrence rate related to treatment modality, impacted tooth and root resorption (P =0.002, 0.022 and 0.007 respectively). Conclusions: Treatment modality, impacted tooth and root resorption all showed statistically significant associations with the recurrence rate in ameloblastoma. However, due to the limitation of this study, further studies are needed to reveal the true mechanism of ameloblastoma recurrence., Competing Interests: Competing Interests: The authors have declared that no competing interest exists., (© The author(s).)

Purpose: The aim of this retrospective study was to investigate the characteristics of cemento-osseous dysplasia (COD) lesions for devising a clinical algorithm to help management decisions, specifically discussing the need for a possible surgical intervention versus solely follow-up., Materials and Methods: One hundred thirty-five subjects were thus identified having previously diagnosed and treated for COD from the archives of the pathology in between 2005-2015. These were then re-evaluated. Demographic features, radiographic and histopathologic findings were assessed., Results: The most frequent variant was the focal type (n=83), and the mandible (n=131) was predominantly affected jaw bone. Female predilection was evident for all subtypes. Almost half of the lesions (n=72) presented with mixed radiolucent and radiopaque appearances in radiography at the time of the biopsy. Cementicles/psammoma body-like calcifications and trabecular pattern were the most common hard tissue appearances., Conclusion: COD lesions are non-neoplastic fibro-osseous lesions which can exhibit similar histopathologic and radiologic features with other bone lesions. Ideally, these lesions should be clinically identified and followed-up. Considering the possible complications, a thorough knowledge of these lesions and their differentials is essential in order to avoid unnecessary surgical interventions., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: To describe the radiographic features of odontogenic keratocysts (OKCs) and ameloblastomas and to compare the radiographic findings between these 2 lesions., Methods: Radiographs of OKCs and ameloblastomas were retrospectively reviewed. Location, border, shape, association with impacted tooth, tooth displacement, root resorption, and bone expansion were evaluated. Chi-squared or Fisher's exact tests were used for statistical analysis. A p value < 0.05 was considered to indicate statistical significance., Results: One hundred OKCs and 101 ameloblastomas were reviewed. The ratios of maxilla to mandible were 1:1.4 and 1:9.1 in OKCs and ameloblastomas, respectively. All evaluated features significantly differed between OKCs and ameloblastomas (p ≤ 0.001). Most OKCs showed smooth border (60%) and unilocular shape (82%), while most ameloblastomas showed scalloped border (77.2%) and multilocular shape (68.3%). Association with impacted tooth was found in 47% of OKCs and 18.8% of ameloblastomas. Adjacent tooth displacement was found in 33.7% of OKCs and 55.8% of ameloblastomas. Root resorption was more common in ameloblastomas (66.7%) than in OKCs (7%). Bone expansion was also more common in ameloblastomas (96.3%) than in OKCs (63.6%)., Conclusion: A unilocular radiolucent lesion with smooth border, no adjacent tooth displacement, no root resorption and causing mild or no bone expansion is suggestive of an OKC rather than an ameloblastoma.

The aim of this study was to describe a series with 66 cases of infected cemento-osseous dysplasia (COD) and to discuss the demographic distribution, clinicoradiographic features and treatment of this condition. A study looking back on the diagnoses made at a single Brazilian centre within a 28-year timeframe was performed. A literature review with searches across five databases was also conducted to identify reports on osteomyelitis/infected COD. Descriptive and statistical analyses were performed. The case series study showed a female/male ratio of 21:1. Affected individuals' mean age was 57.4 years. Mandible was the most affected site (95.5%) and florid subtype was the most frequent infected COD (62.1%). Tooth extraction was the main factor associated with the development of infection associated within a COD lesion. The literature review retrieved 30 studies reporting 46 cases of this condition. Asian women in their 40 s and 50 s were more affected. Surgery for removal/curettage of necrotic bone was acknowledged as an appropriate approach to the treatment of this infection. The clinicodemographic data of the study were similar to data collected across the literature. Clinicians, maxillofacial surgeons and oral rehabilitation providers should be alert to the diagnosis of COD, since infection is a frequent complication whose management is challenging.

Objectives: To evaluate the global incidence of ameloblastoma and to provide a profile of ameloblastoma patients., Material and Methods: A systematic review and meta-analysis was conducted. Searches were performed in PubMed, EMBASE, SCOPUS, and Web of Science for articles published from 1969 to 2018 for the global incidence and from 1995 to 2018 for the profile of ameloblastoma patients., Results: Seven studies on the incidence rate of ameloblastoma were included in the meta-analysis. These studies only covered Europe, Africa, and Australia. The pooled incidence rate was 0.92 per million person-years (95% CI: 0.57-1.49), with significant heterogeneity between studies. Forty-two articles provided profile data of 6,446 ameloblastoma patients. Mean age was 34 years and the peak age incidence in the third decade of life. In Europe and North America, ameloblastoma mostly occurred at an older age when compared to Africa and South America. A slight male preference (53%) was found, and the mandible appeared to be the preferred site. The most common type of ameloblastoma was multicystic. The histopathologic patterns were mostly follicular and plexiform., Conclusions: This is the first study assessing the global incidence of ameloblastoma. The pooled incidence rate was determined to be 0.92 per million person-years., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. All rights reserved.)

Introduction: odontogenic tumors originate from neoplastic transformation of the remnants of tooth forming apparatus. There are varying degrees of inductive interactions between odontogenic ectomesenchyme and epithelium during odontogenesis, leading to lesions that vary from benign to malignant. Malignant odontogenic tumours (MOTs) are very rare and are classified according to embryonic tissue of origin. Recently, there has been a few changes to the classification of MOTs according to the World Health Organization's (WHO) classification in 2017. This study aims to evaluate and reclassify MOTs, using a multi-centre approach in some major tertiary dental hospitals in Nigeria., Methods: this study reviewed the clinicopathological data on 63 cases of MOT diagnosed over 25 years in five major tertiary dental hospitals in Nigeria. All MOT cases were reclassified according to the recent revision to the 2017 WHO classification of odontogenic tumours., Results: from a total of 10,446 biopsies of oral and jaw lesions seen at the 5 study centres over the 25-year study period, 2199 (21.05%) cases were found to be odontogenic tumours (OTs), of which 63 were MOT. MOTs constituted 0.60% of the total biopsy cases and 2.86% of OTs. Odontogenic carcinomas presented with a mean age higher than odontogenic sarcomas. According to our 2017 WHO reclassification of MOTs, odontogenic carcinomas, ameloblastic carcinomas and primary intraosseous carcinomas were found to be the top three lesions, respectively. Carcinosarcomas were found to be extremely rare., Conclusion: using a multi-centre approach is a robust way to reduce diagnostic challenges associated with rare maxillofacial lesions such as MOTs., Competing Interests: The authors declare no competing interests.

The aim of the present study was to integrate the available data published on melanotic neuroectodermal tumor of infancy (MNTI) of the jaw bones. An electronic search was undertaken in April 2018. Hundred forty-seven publications and 371 patients were included. The lesion was more prevalent in males and in the second to sixth months of life. The lesions mostly presented as a rapidly growing bluish swelling and the most commonly involved was the anterior maxilla. The mean follow-up was 51.1 months (range 1-408 months). Age (P ≤ .0001), location (P = .007), occurrence of lymph node metastasis (P ≤ .0001), treatment (P = .001), recurrence (P ≤ .0001), and distant metastasis (P = .0001) were independently associated with survival. Recurrence was significantly correlated with age (P = .0001), distant metastasis (P = .0001), and treatment (P = .0001). Patients older than 12 months, with lesions in the mandible, positive regional lymph node metastasis, treated with chemotherapy, recurrence, or distant metastasis presented the worst prognosis., (© 2018 Wiley Periodicals, Inc.)

Background: Odontogenic tumors (OTs) are uncommon neoplastic lesions of the maxilla and mandible, which present difficult diagnosis and therapeutics. This paper aims to determine the frequency and distribution of OTs, over a period of 22 years, at a public university in Northeastern Brazil., Material and Methods: We reviewed all cases of OTs from oral pathology laboratory of Federal University of Rio Grande do Norte (UFRN), from 1996 to 2017. The tumors were classified according to the latest (2017) World Health Organization Classification of Tumors. Data on age, gender, anatomic site, symptomatology, radiographic findings and tumor size were analyzed., Results: In the analyzed period, 247 cases of OTs were diagnosed. Epithelial tumors were more common with 127 cases (51.8%). The most common tumors were ameloblastoma (n = 112 / 45.4%), odontoma (n = 89 / 36.1%) and odontogenic myxoma (n = 17 / 6.9%). Malignant odontogenic tumors were extremely rare in the studied population with only 2 cases (0.8%) of diagnosed carcinomas. These tumors were diagnosed in a wide age range, from 5 to 81 years, being more common in the second and third decades of life. In general, the mandible was the most affected anatomic site (n = 162/66%) and the mandible:maxilla ratio was of 2:1. Ameloblastoma was the tumor with the highest number of symptomatic cases (n = 26) and with the highest mean size (cm) with 4.5cm., Conclusions: Odontogenic tumors were rare in the sample studied (2.2%), with ameloblastoma and odontoma being the most common tumors. Continuous studies that show the characteristics of these lesions are fundamental, especially after modifications in the international classification.

Background: Burkitt lymphoma (BL) is the most common childhood cancer in Ghana, where the endemic variant is the predominant subtype and historically presents as a highly chemo-sensitive jaw tumor. This study aimed to update the current epidemiological characteristics of childhood BL in our institution., Procedure: Patient data for all children diagnosed with BL and seen at Korle Bu Teaching Hospital between January 2007 and December 2012 were retrospectively analyzed., Results: BL was diagnosed in 173 children (<13 years) during the study period, with the abdomen as the most common tumor site (46%) followed by the jaw (31%). Abdominal tumors were associated with advanced/disseminated disease (P = 0.002), and were more likely to occur in females irrespective of tumor stage (relative risk = 1.56 [95% CI; 1.1-12.3]). Twenty-five percent (43/173) of the study cohort died and mortality was influenced by increasing age (P = 0.02) and advanced disease (P = 0.03). Treatment delay was experienced by nine in ten patients primarily due to familial financial constraint (75%). Treatment abandonment was observed as a first event in 94% of patients and two thirds of children in the study were eventually lost to follow-up., Conclusion: The predominance of primary abdominal tumors in our study cohort may indicate a changing epidemiological pattern of BL in Ghana. High rates of treatment delay and abandonment were evident and are likely to be contributing factors to the poor childhood cancer survival outcomes seen in resource-limited countries in Africa., (© 2018 Wiley Periodicals, Inc.)

Background: Orofacial malignancy is a growing health issue common in developing regions of the world. Presentation patterns are myriad with geographic variations. Advanced stage owing to late presentation constitutes a significant public health burden. The site and type of the lesions are valuable in diagnosis and patient management., Aim: This study aims to review cases of primary orofacial malignancies at the OAUTHC Dental Hospital., Objectives: The objective of the study was to determine the prevalence of histologically diagnosed orofacial malignancies, the relative frequencies, types and site of distribution., Materials and Methods: Records of patients with orofacial malignancies at the OAUTHC, Dental Hospital over a period of 10 years (January 2008-December 2017) were reviewed, demographic data (age, gender and site), history of tobacco use were retrieved and entered into a pro forma. The data obtained were analysed with STATA 11. Statistical significance was set at P < 0.05., Results: Of 375, 109 cases of neoplasms seen were primary malignant tumours, with prevalence rate of 29.1%. There were 71 (65.1%) males and 38 (34.9%) females (male:female ratio of 1.87:1), mean age (48.7 ± 19.3 years) and range (4-94 years). Affected sites were mandible (41, 37.6%), maxilla (39, 35.8%), palate (17, 15.6%) and others. Lesions were mainly squamous cell carcinomas (SCC: 46, 42.2%), salivary gland adenocarcinomas (SGAs, 25, 22.9%) including 8 (32%) cases of adenoid cystic carcinoma (ACC). Others were odontogenic carcinoma (18, 16.5%) and lymphoma (8, 7.3%). Most specimen analysed were hard tissues (n = 63, 57.8%). Thirty-four (73.9%) cases of SCC and 66 (60.6%) cases of primary malignancies were in the 5 th -9 th decades of life. This was statistically significant at P = 0.000., Conclusion: SCC was more prevalent than salivary and odontogenic carcinomas. ACC and mucoepidermoid carcinoma were two most common SGAs. Metastatic tumours to the jaws are rare., Competing Interests: None

Metastatic tumours to the jaw bones are rare, and usually develop during the final stages of cancer. Some, such as those of lung, breast, and kidney, are more likely to metastasise to the jaw. We have therefore analysed the clinical and epidemiological characteristics of patients with metastatic tumours. We retrieved the notes of 4 478 patients with metastatic tumours to the jawbones who were treated in the Clinical Hospital Centre Dubrava in Zagreb, Croatia, during the 15 years 2002-17 and made a retrospective analysis of patients' age, sex, site of primary tumour, site and clinical presentation of the metastases, time interval since diagnosis of the primary tumour and oral metastases, and time interval from diagnosis of oral metastases to death. Of the 10 who were diagnosed with metastases to the jaw, there were four male and six female patients (mean age 57 (range 51-84) years) and the most common primary tumours were kidney (n=5), lung (n=2), breast (n=1), colon (n=1) and unknown (n=1). The mandible was more often affected (n=7) than the maxilla (n=3), and the most common histological type was adenocarcinoma (n=6). The primary tumour in most of the patients (n=7) was diagnosed before the oral metastatic lesion. A metastasis in the jaw was the first sign of metastatic tumour in three patients, and in one case the metastasis and the primary tumour were diagnosed at the same time. Most of the patients had some oral problems. The time intervals from diagnosis of an oral metastasis to death varied from one month - five years. Because of the rarity of the presentation, the diagnosis of an oral metastatic lesion remains challenging, so metastases in the jaw should be suspected in every patient with such cancers and lesions in the jaw., (Copyright © 2018 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Unicystic ameloblastoma (UA) is a variant of ameloblastoma that has a relatively benign biologic behavior and mostly occurs in a younger age group. The entire cystic lining of unicystic ameloblastoma may not always be uniformly characteristic and may partly consist of non-specific epithelium or dentigerous cyst-like lining. The variability seen reinforces the advice that multiple biopsies should be taken from large cystic lesions to represent the entire lesion., Methods: All cases were retrieved from the archives of our unit from 1986 to 2016. Demographic data such as age, gender, and primary site were recorded. Histologically, all the cases were subcategorized according to the WHO 2017 classification., Results: UA accounts for 31.1% out of all different subtypes of ameloblastoma, and male-to-female ratio is 1.08:1. Age ranged from 4 to 88 years with the mean age of 30.25 years. Peak incidence of UA was found in the range of 11-20 years, and 89% of them occurred in the mandible and 55.3% in the canine-to-first molar region. The right side was frequent in both upper and lower jaws. Of the total sample, 233 (63%) cases were luminal and 137 (37%) cases were mural, and 13 cases recurred (3.5%)., Conclusion: The present report analyzes the largest UA sample in a single center. There is a clear need for further large case-controlled retrospective or prospective studies of the management of UA with careful and follow-up studies to draw conclusions on the correct method of treatment of these lesions., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: Our objective was to describe the clinicopathologic and immunohistochemical features of acute lymphoblastic leukemia/lymphoma (ALL/LBL) of the oral and maxillofacial region (OMF)., Study Design: Cases diagnosed as ALL/LBL of the OMF region were retrieved from the files of 2 Brazilian and 1 Guatemalan oral pathology services from 2005 to 2017. Microscopic and immunohistochemical features of each case were reviewed and fully described, and clinical data were retrieved from the pathology reports., Results: During the period considered, 6 cases were identified. Male patients were the most affected (4:2), with a mean age of 19 years old. The mandible was involved in 2 cases, the maxilla in 2, the cheek mucosa in 1, and the parotid gland in 1. Painful swelling was the most common presentation, and 3 patients also had systemic complaints. Microscopically, tumors revealed solid infiltrations of small to medium-sized immature cells. "Puzzle-like" and "starry-sky" patterns were observed, and "single lane" growth was also identified. Immunohistochemically, 2 cases were diagnosed as T-cell ALL/LBL with the leukocyte common antigen (LCA) + /cCD3 + /CD79 a + focal/CD20 - /PAX5 - /CD99 + /CD34 - /CD10 + /terminal deoxynucleotidyl transferase (TdT) + phenotype and 4 as B-cell ALL/LBL with the LCA + /CD3 - /CD20 - /CD79 a + /CD10 + /CD34 variable/TdT + predominant phenotype. The Ki67 index ranged from 80% to 99%., Conclusion: OMF ALL/LBL is rare, but its microscopic features and immunohistochemical profiles CD3 + or CD79 a + /CD10 + /CD34 + variable/CD99 + /TdT + contribute to the correct diagnosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Several skeletal aberrations of the skull have been described for the tumor predisposition syndrome neurofibromatosis type 1 (NF1). Recently, periapical cemental/cemento-osseous dysplasia (COD) has been described in females affected with NF1. This reactive lesion of the hard tissues in tooth-bearing areas of the jaw has been proposed to represent a gender-specific radiological feature of NF1. The aim of this study was to investigate the prevalence of COD in patients with NF1., Patients and Methods: The orthopantomograms (OPGs) of 179 patients with a confirmed diagnosis of NF1 were analyzed for COD. The results were compared to radiographic findings obtained in OPGs of age- and sex-matched controls. The NF1 patient group was further differentiated according to the evidence of facial plexiform neurofibroma., Results: COD was a very rare finding in both groups. The extension of the diagnostic criteria including radiologically-healthy teeth and a widened periodontal gap in the periapical area only marginally increased the number of considered cases. Although there was a somewhat more common occurrence of such changes in the patient group compared to the control group and the number of affected women was greater than the number of men, none of these differences reached statistical significance. Furthermore, COD or widening of the periradicular periodontal space was not found to be associated with facial tumor type in NF1., Conclusion: The investigation revealed that COD is not a diagnostic feature of NF1. There is no clear association of the rare finding of COD with gender. These studies should be compared with patient groups of other ethnic backgrounds., (Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Children and adolescents who present with neuroendocrine tumors are at extremely high likelihood of having an underlying germline predisposition for the multiple endocrine neoplasia (MEN) syndromes, including MEN1, MEN2A and MEN2B, MEN4, and hyperparathyroid-jaw tumor (HPT-JT) syndromes. Each of these autosomal dominant syndromes results from a specific germline mutation in unique genes: MEN1 is due to pathogenic MEN1 variants (11q13), MEN2A and MEN2B are due to pathogenic RET variants (10q11.21), MEN4 is due to pathogenic CDKN1B variants (12p13.1), and the HPT-JT syndrome is due to pathogenic CDC73 variants (1q25). Although each of these genetic syndromes share the presence of neuroendocrine tumors, each syndrome has a slightly different tumor spectrum with specific surveillance recommendations based upon tumor penetrance, including the age and location for which specific tumor types most commonly present. Although the recommended surveillance strategies for each syndrome contain similar approaches, important differences do exist among them. Therefore, it is important for caregivers of children and adolescents with these syndromes to become familiar with the unique diagnostic criteria for each syndrome, and also to be aware of the specific tumor screening and prophylactic surgery recommendations for each syndrome. Clin Cancer Res; 23(13); e123-e32. ©2017 AACR See all articles in the online-only CCR Pediatric Oncology Series ., (©2017 American Association for Cancer Research.)

Epidemiological studies on odontogenic tumours conducted in different parts of the world emphasised variation in incidence and distributional pattern. Such epidemiological studies are obscured in Southern state of Andhra Pradesh in India. Present study was conducted at an institutional setup in South Indian population to assess the demographic data of odontogenic tumours. The retrospective study, which included all the odontogenic tumours from the archives of department of oral pathology, Dental teaching and Research Institution in southern part of India. Cases were selected based on the classification of WHO 2005 histopathological typing for odontogenic tumours and the assessment year considered was from 2002 to 2014. Demographic data was analysed for these tumours. Results were analysed using Chi-Square Test. Incidence of the odontogenic tumours was found to be 2.17%. Peak age incidence was recorded highest in third and fourth decade of life. Males were commonly involved [59%] with the male to female ratio of 1.43:1. Posterior mandible [53.4%] was the chief anatomical location involved with the tumours. Considering the individual lesions, Ameloblastoma [49%] was found to be more frequent, followed by Keratinizing cystic odontogenic tumour [32%], Odontome [6.2%], Adenomatoid odontogenic tumour [5.5%], Odontogenic myxoma [2.4%], Ameloblastic fibroma [0.6%], Calcifying epithelial odontogenic tumour [1.8%] and Squamous odontogenic tumour [1.2%]. The total frequency of odontogenic tumours was 2.17%. Ameloblastoma and Keratinizing cystic odontogenic tumours were the predominant tumours, demonstrating significant regional and geographic variation., (Copyright © 2016 King Saud Bin Abdulaziz University for Health Sciences. Published by Elsevier Ltd. All rights reserved.)

Background: Ameloblastoma is a benign slow growing tumor of odontogenic origin composed of epithelial cells that resemble enamel forming cells namely the ameloblasts however these lesional cells do not differentiate to produce enamel. They are locally aggressive and can cause severe abnormalities of the face and jaw., Aim: This study aimed to correlate the incidence and recurrence rates of ameloblastoma to the different clinical and histopathological parameters., Methods: A study on ameloblastomas in the Goan population for 16 years (1999-2014) was carried out. Archived documented data of surgically treated cases of ameloblastomas were used in this study., Results: It was revealed that out of 52 cases of ameloblastomas, a male preponderance was seen. Age predisposition was seen to favor the third-fourth decade, the most common site was the posterior aspect of lower jaw for new and recurrent cases., Conclusion: The study highlights, the possible etiological role of location influencing the incidence and recurrence rates of ameloblastomas.

Background: Odontogenic tumors (OTs) are rare lesions, exclusive of the jaws, that are derived from epithelial and/or ectomesenchymal elements of the tooth-forming apparatus. Their biological behavior is heterogeneous, including hamartomatous tissue proliferation, benign nonaggressive and aggressive neoplasms, and malignant tumors with metastatic capacity. The aim of this study was to describe the relative frequency of odontogenic tumors in a Brazilian population. In addition, a review of the literature identified studies on odontogenic tumors that follow the 2005 World Health Organization., Material and Methods: A total of 376 cases of odontogenic tumors from an oral pathology service were reviewed about age, gender, anatomic site and histologic diagnosis., Results: Keratocystic odontogenic tumors (31.6%) were the most common, followed by ameloblastoma (28.5%), and odontoma (22.6%). The mean age was 32.2 years, and more than half the patients (52.1%) were in the second and third decades of life. The male to female ratio was 1:1.37, with a maxilla to mandible ratio of 1:2.08., Conclusions: The variation in relative frequency of tumors observed among the several series, including the present study, is probably due in part to cultural differences between geographic areas but also to the study design.

Purpose: The aim of this study was to evaluate the impact of the reclassification of odontogenic keratocyst (OKC) as a tumor on the prevalence profile of odontogenic cysts (OCs) and odontogenic tumors (OTs)., Study Design: Two referral Oral and Maxillofacial Pathology services in Brazil were evaluated. All cases diagnosed as OCs or OTs were selected and classified according to the 1992 WHO-classification (cases before 2005 WHO classification of tumors excluding OKC) and the 2005 WHO classification of tumors, going forward including cases of odontogenic keratocyst tumor (KCOT). The frequency and prevalence of OCs and OTs were compared before and after the reclassification., Results: Among 27,854 oral biopsies, 4920 (17.66%) were OCs and 992 (3.56%) were OTs. The prevalence of OTs before 2005 WHO classification of tumors was 2.04%, while the prevalence after 2005 WHO classification was 11.51% (p < 0.0001). Before 2006, the most frequent tumor diagnosed was odontoma with 194 cases (39.67%), and after 2005 WHO classification of tumors the KCOT was the most frequent with 207 cases (41.07%)., Conclusions: The increase in the prevalence of OTs after 2005 WHO is related to the improvement of pathology services and to the inclusion of KCOT in the OTs group., (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Purpose: To investigate and compare the probability of recurrence of keratocystic odontogenic tumors (KCOTs) for different variables and treatment protocols., Materials and Methods: An electronic search was undertaken in April 2016 that included clinical series of KCOTs reporting recurrences. Untransformed proportions and meta-analyses were performed to estimate the probability/risk of recurrence, according to several variables., Results: A total of 94 publications were included (6427 KCOTs, 1464 recurrences). Probability of recurrence: all lesions, 21.1%; nevoid basal cell carcinoma syndrome, 35.4%; males, 20.3%; females, 19.3%; maxilla, 15.3%; mandible, 21.5%; unilocular, 14.7%; multilocular, 24.4%; marsupialization/decompression, 28.7%; decompression + enucleation ± additional therapy, 18.6%; enucleation/curettage, 22.5%; enucleation + peripheral ostectomy, 18.6%; enucleation + Carnoy's solution, 5.3%; enucleation + cryotherapy, 20.9%; marginal/segmental resection, 2.2%. The recurrence was not statistically significantly affected by lesion location (maxilla vs. mandible, risk ratio [RR] 0.92, P = 0.32) or patient's sex (male vs. female, RR 0.94, P = 0.44), but by locularity (unilocular vs. multilocular, RR 0.67, P = 0.007). Recurrence risk for surgical managements: marsupialization vs. enucleation (RR 1.65, P = 0.0006), marsupialization vs. resection (RR 3.17, P = 0.009), enucleation alone vs. enucleation + peripheral ostectomy (RR 1.66, P = 0.05), enucleation alone vs. enucleation + Carnoy's solution (RR 1.94, P = 0.03), enucleation alone vs. enucleation + cryotherapy (RR 0.88, P = 0.56)., Conclusions: KCOTs have a considerable rate of recurrence, which varies significantly according to some clinical, radiographic, and histopathological features, as well as surgical management., (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Introduction: Primary intraosseous carcinoma (PIOC) of the jaws is a rare epidermoid carcinoma from epithelial origin and initially strictly localized within the bone. Histologically, type 3 PIOC (PIOC3) is a de novo primary intraosseous carcinoma. Because of the rarity of this illness, we propose an analysis of a personal case and a revue of the literature., Material and Methods: Two search engines (Pubmed ® , Sciencedirect ® ) were questioned over the period 1976-February 2016 by using following keywords carcinoma, intraosseous, jaws, squamous cell carcinoma. Articles reporting proven PIOC3 and mentioning a precise treatment were selected., Results: Thirty articles concerning 54 patients (sex ratio: 2.4; mean age: 56.8; extreme: 24-78) met the inclusion criterions. The most common symptoms were swelling (53%), pain (44.9%) and infra-alveolar nerve paresthesia (30.6%). The time to diagnosis was 13 weeks. Classification of Zwetyenga et al. showed more than 80% of T2 and T3 stages. The lesions were predominantly mandibular (85.2%) and posterior. Less than a third of patients had lymph node and 10% had distant metastasis. Treatment consisted mostly in a combination of surgery and radiotherapy. With a mean follow-up of 74.8 months, 70.8% were in remission with no evidence of recurrence. We report the case of a 58-year-old patient, with no medical history, complaining since several months about periodontitis with teeth mobility in the right mandibular area. The panoramic X-ray showed a bone lysis at the place of tooth No. 46. In the absence of alveolar healing after extraction and antibiotherapy, a biopsy was made that diagnosed a differentiated keratinizing squamous cell carcinoma. CT scan and MRI showed a mandibular cortical bone loss with involvement of adjacent structures and lymphadenopathy in the ipsilateral IB area. The patient was treated with a combination of chemotherapy and surgery. Postoperative chemo- and radiotherapy is still going on., Discussion: The PIOC3 is a rare tumor, mainly arising in males around 50. Diagnosis should be evoked in the presence of painful swelling and nervous symptoms. The time to diagnosis is long. Tumors are usually seen at late stages. Treatment classically combines surgery and radiotherapy., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Odontogenic myxoma is a benign, locally aggressive neoplasm of the jaws. Prevalence rates range between 0.5% and 17.7% of odontogenic tumours. There are few reports in the literature on this lesion in African populations, and therefore, this study aimed to report on odontogenic myxoma in a South African population over a 40-year period., Methods: The clinical records and orthopantomograms of 29 histopathologically diagnosed odontogenic myxoma were retrospectively analysed. Details of age, gender, ethnic origin and clinical, histological as well as radiological features were recorded., Results: The ages of patients ranged from 7 to 44 years with a mean of 21.3 years. The male-to-female ratio was 1:2.6 with the majority of patients being of mixed race and Africans. Clinically, 31% complained of pain while 58.6% had a history of swelling. The majority of odongenic myxomas (62.1%) were located in the mandible with the posterior region being most commonly affected. Multilocular lesions (69.2%) were more common and were significantly larger than unilocular lesions (P < 0.05). The outline of these tumours was mostly well-defined (84.6%) with different degrees of cortication. Only one tumour caused tooth resorption, while 20 cases (76.9%) caused tooth displacement. Six tumours expanded into the maxillary sinus, and 14 tumours caused expansion of the mandible., Conclusions: Odontogenic myxomas have variable clinical, radiological and histological features. Most of these features in this population were similar to other populations. It is mandatory to use conventional radiographs along with histopathological examination to aid in arriving at an accurate diagnosis., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Opinions regarding the treatment of multicystic ameloblastoma are divergent due to its benign nature and the high rate of recurrence if not adequately excised. The aim of the present study was to perform a systematic review of the literature for a qualitative and quantitative assessment of studies addressing primary multicystic ameloblastoma with regard to treatment and recurrence. Searches were conducted of the Ovid Medline and Embase databases for articles published up to January 2014. Based on predefined eligibility criteria, studies were selected in a two-stage screening process conducted by two independent reviewers. Quality assessment of the selected articles was performed using the modified criteria of the Agency for Healthcare Research and Quality. The meta-analysis was performed using Review Manager (RevMan) software. Statistical heterogeneity was investigated by performing a χ(2) test at the 5% significance level (P<0.05) and determining I(2). The relative risk of recurrence was 3.15-fold greater (95% confidence interval 1.98-5.00) when conservative treatment was performed on primary multicystic ameloblastoma in comparison to radical treatment (P<0.00001 for treatment effect; I(2)=0% and P=0.48 for heterogeneity). The findings justify the treatment of primary multicystic ameloblastoma with bone resection., (Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Morphological and functional disturbances induced by postsurgical defects and loss of tissues in the stomatognathic system due to the treatment of tumors in the maxillofacial region determine the therapeutic needs of patients. The study aimed at clinical and epidemiological evaluation of patients under prosthetic treatment in order to establish the algorithm for rehabilitation. The study group was composed of the patients after midface surgery (45.74%); surgery in a lower part of the face (47.38%); mixed postoperative losses (3.44%); loss of face tissues and surgery in other locations in the head and neck region (3.44%). The supplementary treatment was applied in 69.63% of patients. Clinical and additional examinations were performed to obtain the picture of postoperative loss, its magnitude, and location to plan the strategy of prosthetic rehabilitation. The management algorithm for prosthetic rehabilitation in patients after surgical treatment of maxillofacial neoplasms was based on its division in stages. The location and magnitude of postoperative losses, as well as the implementation of supplementary treatment of the patients after treatment of maxillofacial tumors, influence the planning of prosthetic rehabilitation that plays a key role and facilitates the patients' return to their prior living situation, occupational and family lives., Competing Interests: The authors declare that they have no competing interests.

Ameloblastoma is the most common epithelial odontogenic tumor. It may show locally invasive behavior resulting in recurrence and malignancy. Therefore, appropriate diagnosis of this tumor is necessary. The aim of this study was to evaluate clinicopathological characteristics of ameloblastomas in an Iranian population. We present a 40-year retrospective study of patients diagnosed from 1971 to 2010 in the Department of Oral and Maxillofacial Pathology, Faculty of Dentistry, Mashhad, Iran. Information gathered from patient records included age, gender, tumor location and histologic type. The frequency of odontogenic tumors among all lesions was 2.08% and ameloblastoma with 88 samples demonstrated the greatest prevalence (41.5%). Regarding gender, 60% of samples occurred in males. The mean age of studied patients was 33.02± 15.74 years with a peak of occurrence in the third decade of life. The most frequent location of tumor was the mandibles (93.2%). Eighty five (96.6%) tumors were recorded as benign and 3 (3.4%) as malignant. Of benign tumors, 62 (72.9%), 20 (23.5%) and 3 (3.6%) cases were of conventional, unicyctic and peripheral types, respectively. In contrast to most previous studies, the most common histologic subtype in the present study was plexiform. Knowledge of the incidence of ameloblastoma and its clinicopathologic features including most common location, gender and age distribution in different ethnogeographic backgrounds is necessary for accurate diagnosis and proper treatment.

Objective: The goal of this study is to describe 7 cases of clear cell odontogenic carcinoma among a Brazilian population and compare these data with a systematic review of the English-language literature., Study Design: Descriptive statististics were used to compare the clinicopathologic data gathered retrospectively with those compiled from a review. Tumor sections were immunostained for Ki-67, p16, p53, and cytokeratins (CKs) 7, 8, 14, 18, and 19. Log-rank tests were performed for survival analysis., Results: Most cases occurred in the posterior mandible (5/7, 71.4%), and recurrence was diagnosed in all treated patients. Metastatic disease occurred in 2 patients (28.6%). Tumors were focally positive for CKs 7, 8, 14, and 18 and diffusely positive for CK19, p53, and p16. The mean number of Ki-67-positive cells was 35.2 cells/high-power field. Our systematic review provided evidence that tumor size (P = .046), histologic pattern (P = .034), regional metastasis (P = .001), distant metastasis (P = .001), and local recurrence (P = .05) were of significant prognostic value., Conclusions: This study has contributed to improved characterization of clear cell odontogenic carcinoma, which is an aggressive odontogenic malignant neoplasm diagnosed mainly in the posterior mandible of middle-aged women and typically at an advanced stage. Radical surgical procedures remain the gold standard treatment., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Our practice in a humanitarian (or crisis) context differs from what we experience in daily practice. There are several reasons for this. First, the diseases encountered are sometimes unfamiliar, such as sequelae of noma, or the presentation of familiar diseases may be unusual, such as facial malformations seen at a late stage. Secondly, these missions take place in developing countries, and consequently, evaluation and anticipation of possible malnutrition should be considered, especially because facial diseases themselves may be responsible for nutritional problems. Lastly, conditions are often difficult, occurring in an unusual environment, and we sometimes have to face communication and equipment problems. The goal of our work, based on a 15-year experience (in Bamako and Mopti with the Association "Santé et Développement", and in Ouagadougou with the organization "Les enfants du noma") and the analysis of literature, is to point out these features and maybe to be helpful to others., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Ameloblastoma in the paediatric age group is considered a rarity and it accounts for approximately 10-15% of all reported cases. This study assessed the clinical, radiological, and histopathological features of 39 cases of ameloblastoma in Indian children aged less than 18 years, seen over a 41-year period (1971-2011) in the Department of Oral Pathology, Nair Hospital Dental College, India. Out of 256 diagnosed cases of ameloblastoma, 39 (15.2%) occurred in patients ranging in age from 4.5 to 18 years (mean age 13.6 years; male-to-female ratio 2:1). All of the tumours were intraosseous, with a marked predilection for the mandible (97.4%), the body-angle-ramus being the most commonly involved site. Radiographically, 23 cases presented as unilocular radiolucency. Histologically, 20 cases presented as solid and 19 as unicystic ameloblastoma. The interesting finding of 10 solid ameloblastoma presenting as unilocular radiolucency and five cases of unicystic ameloblastoma manifesting as multilocular radiolucency suggests that solid ameloblastomas should be included in the differential diagnosis of unilocular radiolucency of the jaw in the paediatric age group., (Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Importance: Osteosarcoma of the jaws is rare and clinically distinct from osteosarcoma of the long bones of the body with different treatment and outcomes. The literature on these tumors is limited to case reports and small case series mostly from single institutions. We used data from the population-based national Surveillance, Epidemiology and End Results (SEER) cancer registry to determine the epidemiology and prognostic factors associated with osteosarcoma of the jaws., Objective: To investigate the epidemiologic characteristics and prognostic factors for survival in patients diagnosed with osteosarcoma of the jaws., Design, Setting, and Participants: A retrospective, population-based cohort study of 541 patients in the SEER tumor registry diagnosed with osteosarcoma of the jaws from 1973 through 2011 were reviewed., Exposures: Patients had been treated with surgery, radiation, both, or neither., Main Outcomes and Measures: Overall and disease-specific survival., Results: A total of 541 patients diagnosed with osteosarcoma of the jaws were identified (49.9% male and 50.1% female, with a mean age of 41.3 years). Kaplan-Meier analysis demonstrated an overall survival (OS) and disease-specific survival (DSS) of 53% and 62%, respectively, at 5 years and 35% and 54%, respectively, at 10 years. Multivariate Cox regression analysis revealed that independent predictors of OS and DSS included age at diagnosis (hazard ratio [HR], 1.03; 95% CI, 1.02-1.04 [P < .001] for OS; and HR, 1.03; 95% CI, 1.02-1.05 [P < .001] for DSS); stage at presentation (HR, 1.37; 95% CI, 1.10-1.71 [P = .006] for OS; and HR, 1.34; 95% CI, 1.01-1.76 [P = .04] for DSS); and surgical resection (HR, 0.31; 95% CI, 0.16-0.60 [P < .001] for OS; and HR, 0.22; 95% CI, 0.09-0.56 [P = .001] for DSS). Tumor size was not significant for OS (HR, 1.00; 95% CI, 1.00-1.01 [P = .11] but significant for DSS (HR, 1.01; 95% CI, 1.00-1.01 [P = .003])., Conclusions and Relevance: To our knowledge, this is the largest study to date investigating prognostic factors for survival in patients diagnosed with osteosarcoma of the jaws. Determinants of survival include age at diagnosis, stage at presentation, tumor size, and surgical therapy. Radiation therapy was not associated with improved survival, reflecting the controversy surrounding its use in clinical literature.

Although relatively rare, lymphomas can and do present within the oral cavity and can represent either the initial presentation or secondary involvement in the setting of systemic disease. Our objective was to conduct a retrospective search of the surgical pathology database at our institution to review all oral biopsy specimens diagnosed as either a lymphoma or plasma cell neoplasm over the past 15 years. Based on our search, we identified 47 cases. We report here the type of neoplasm, location, patient age and gender, and available pertinent clinical information.

Background: Malignant ameloblastoma, comprising metastasizing ameloblastoma and ameloblastic carcinoma, represents 1.6-2.2% of all odontogenic tumors. Due to its rare nature, malignant ameloblastoma has only been reported in the literature in small case series or case reports. Using the Surveillance, Epidemiology and End-Results (SEER) database, we have performed a population-based study to determine the incidence rate and the absolute survival of malignant ameloblastoma., Method: Using the International Classification of Diseases for Oncology (ICD-O) codes 9310/3 and 9270/3, data from the SEER database were used to calculate the incidence rate and absolute survival rate of population with malignant ameloblastoma., Results: The overall incidence rate of malignant ameloblastoma was 1.79 per 10 million person/year. The incidence rate was higher in males than females and also higher in black versus white population. The median overall survival was 17.6 years from the time of diagnosis and increasing age was associated with a statistically significant poorer survival., Conclusions: To our best knowledge, we report the largest population-based series of malignant ameloblastoma. The incidence rate was 1.79 per 10 million person/year and the overall survival was 17.6 years.

Aim: The goal of our study is investigate the frequency of metastasis to oro-maxillo-facial region to understand if they are really so rare., Material of Study: In this eight year's retrospective study (2004-2012) we collected 15 cases of metastasis localized in the maxilla-facial region from distant primary tumor., Results: Our results show breast and kidney as the most frequent primary site (40% and 20% respectively), adenocarcinoma as most common histological type (60%). Bone involvement has found to be much frequent than the soft tissue one (53.3%). The mandible (5/15 cases) is more affected than the maxilla, and most common interested subsites are molar and retromolar region. In our study we found only one case of unknown primary tumor, it was a mandibular bone metastasis from a renal clear cell carcinoma., Conclusion: Finally, according to our results and considering the increase of survival in cancer disease, even if metastases to oro- maxilla- facial region from distant sites are not frequent, it is important to suspect secondary lesions both in patients that was referred a tumor in their medical history and in those that present a head and neck lesion.

Purpose: The aim of this work is to report a review of the literature concerning epidemiology, clinical and radiographic features as well as treatment of odontogenic myxofibroma (MF)., Methods: The PubMed database was searched using the following keywords: "odontogenic myxofibroma", "odontogenic fibromyxoma", "myxofibroma of the jaw" and "fibromyxoma of the jaw"., Results: Fifteen articles reporting the experience with 24 patients were identified. Male/female ratio was 1:1.4 and the average age 29.5 years. The most frequent location was the mandible. In 66.7% of the cases the radiographic appearance was a multilocular radiolucency. Swelling was observed in 13 patients (92.86%), varying degrees of pain in 5 (35.71%) and paresthesia in only one patient (7.14%). Six out of 24 patients (26.09%) were treated with radical surgery and 17 out of 24 (73.91%) with a conservative approach. In two out of 21 cases (9.52%) a recurrence was reported., Conclusions: MF is an extremely rare tumour and no agreement exist on the causes of its development. According to the present review, the choice of treatment should depend on variables such as localization, presence of a primary or of a recurrent lesion, age, general medical conditions and aesthetic needs of the patient.

Objectives: To provide epidemiological data of ameloblastomas of the jaws in the Netherlands over a 25-year time period (1985-2010) and to compare these data with data from other parts of the world., Material and Methods: The data of all patients diagnosed with a primary ameloblastoma of the jaws in the Netherlands in the period 1985-2010 have been retrieved from the nationwide histopathology and cytopathology network and registry in the Netherlands (PALGA). The pathology reports were screened and only those cases were included in which a distinct diagnosis of primary, histopathologically benign, intraosseous ameloblastoma was rendered. The average population in The Netherlands during this period amounted approximately 15 million people., Results: An annual incidence rate was approximately 1,5 per million population, the male-female ratio being 1.4: 1. The age at the time of diagnosis was 44.1 years. The average age in males was 46.3 years compared to an average age in females of 41.3 years, the difference being significant (p≤ 0.05). The results were compared with those available in only a small number of publications worldwide., Conclusions: There is no strong evidence for significant differences of the true incidence of ameloblastomas worldwide, neither for a gender predilection. The diagnosis is generally made at a somewhat lower age in women; this phenomenon is even much stronger in the Black population, irrespective of gender. No proper explanation for this finding can be provided.

Osteosarcomas of the jaws are rare mesenchymal tumors frequently diagnosed in the fourth decade of life which account for 6 % of all osteosarcomas. This study evaluated the efficacy on the patients outcome of multimodality treatment consisting of surgery, chemotherapy and radiotherapy. The records of 22 patients affected by jaw osteosarcoma treated with a combination of surgery, poly-chemotherapy and adjuvant radiotherapy in selected cases were reviewed. Response rate, progression-free survival and overall survival were evaluated. Neoadjuvant chemotherapy resulted in an overall response rate of 83.3 %, necrosis of grade I or II was obtained, respectively, in 44.4 and 55.6 % of the patients, and surgery was radical in all patients. At a median follow-up of 60 months, the 5-year progression-free survival and overall survival were 73.5 and 77.4 %, respectively. These outcome parameters significantly correlated with age at diagnosis and grade of chemotherapy-induced necrosis. A complex multimodality approach including chemotherapy and radiotherapy, along with radical surgery, can maximize the outcome of patients affected by osteosarcoma of the jaws.

Objective: This study aims at contributing to the definitive diagnosis of ossifying fibroma (OF) based on histomorphological features. The study also aims to determine some demographic features of OF, common sites of occurrence and to determine whether behavior correlates well with the histomorphological variations seen in the lesion., Materials and Methods: A total of 80 patients who were diagnosed either as cementifying fibroma CF, OF and cemento-ossifying fibroma (COF) of the jaws from the files of the Oral Pathology Department of the University of the Witwatersrand Dental School were retrieved and the histology slides of each case were reviewed with the most recent diagnostic criteria for OF and the authors additional criteria. A total of 56 cases that met the set criteria were analyzed., Results: The patients were clustered within the third and fourth decades of life ( n = 39, 69.6%). Majority of the patients were black (83.93%), whereas the rest were whites (12.50%) and Asians (3.57%). There were 17 males (30.4%) and 39 females (69.6%), giving a male to female ratio of 1:2.3. Most of the lesions (70.3%) occurred in the mandible, involving the premolar molar region (56.7%). Scanty fibrous tissues in highly cellular lesions were found in 36 (64.3%) of the cases. There were globular, dystrophic or granular calcifications mixed with irregularly shaped trabeculae of lamellar or cellular woven bone or osteoid were found, in 36 (64.3%) cases., Conclusion: Demographic data, clinicoradiologic features, combined with histopathology will continue to be relevant in the definitive diagnosis of OF and in predicting its behavior. Highly aggressive lesions with shorter duration in people below 15 years were called juvenile OF and treated as such, while OF applies to other conventional ones.

Benign fibro-osseous lesion (BFOL) is a distinct group of jaw entities composed of fibrocellular tissue and mineralized materials. In this study, we examined the epidemiological, clinical, and pathological features of patients with BFOL. Records and microslides of 207 BFOLs submitted to pathology service were retrospectively reviewed. Overall, fibrous dysplasia (FD) was the most prevalent (36.7%), followed by ossifying fibroma (OF; 32.4%), osseous dysplasia (OD; 24.6%), and juvenile ossifying fibroma (JOF; 6.3%). Female predilection was noted. FD and JOF were common in maxilla, whereas most OF and OD affected the mandible. Most patients with FD and OF presented with painless swelling, while patients with OD were symptomless. The majority of FD specimens showed woven bone, while a mixture of woven bone and cementum-like materials was often noted in OF and OD. Our data show variations in the clinicopathologic features of BFOLs. A thorough examination of all aspects of BFOL patients is imperative for accurate diagnosis., (© The Author(s) 2013.)

Renaming of the Odontogenic Keratocyst as the Keratocystic Odontogenic Tumor by the World Health Organization (WHO) is based on the aggressive nature of this lesion. Satellite cysts founded in the walls of the original cysts may give rise to a new lesion formation. The aim of this retrospecitve study was to identify the existence of specific features according incidence of satellite cysts and the pallisading of the basal layer of the epithelium and to establish their mutual correlation. The histopathologic data of Keratocystic Odontogenic Tumor on the basis of new WHO's classification (2005) were analized. Prominent palisade basal cell layer was found in 415 (94.75%) and partially absent palisade basal cell layer in 23 (5.25%) cases. Satellite cysts were presented in prominent palisade basal cell layer in 85 specimens (20.5%) and in cases with partial absent of the palisade basal layer in 3 spicemens (13%). The higher the frequency of pallisading was the higher the frequency of satellite cysts was (p > 0.05).

Intraosseous lipoma is a benign tumor of the bone. It is mostly seen in the metaphyses of the long bones and calcaneus. There are few documented cases of intraosseous lipomas in the jaw. Clinically, the lesion is usually silent and radiologically it appears as a radiolucent area rarely including some radio-opacities. Diagnosis is based on clinical, radiological and histopathological features. Surgical removal of the lesion is the recommended treatment., (Copyright © 2014. Published by Elsevier Masson SAS.)

Odontogenic tumors (OTs) are lesions that develop exclusively on maxillary bones, and form a heterogeneous group. They vary from hamartomatous lesions to benign and malign tumors. Although they are rarely observed in dentistry clinics, it is extremely important for the dentist to be aware of them. The aim of this study was to investigate the incidence of odontogenic tumors diagnosed in the population of Florianópolis, Santa Catarina, Brazil. Cases of odontogenic tumors were selected from the anatomopathological diagnostic services at Federal University of Santa Catarina from 1998 to 2011. Clinical data on these cases were collected from biopsy reports and patient files. Seventy-eight cases of odontogenic tumors were surveyed. Of these diagnoses, 51% were keratocystic odontogenic tumors (KCOTs); the remaining cases were mainly ameloblastomas and odontomas. The most frequently observed lesion in this retrospective study was KCOT (more than half of cases). Thus, this study shows that modifying the classification of the OTs altered the frequency of the lesions, possibly making KCOT the most common lesion observed in diagnostic services worldwide.

In the article information is resulted about morbidity by malignant new formations of maxillofacial localization in region of Poltava for 20 years of supervisions, which diminished from 4 to 2,5% to 2011 year, but increased to 3,3% in the structure of general oncologic morbidity in 2012; about the change of structure and grade places of oncostomatology's morbidity; about establishment of reasons of late diagnostics. It is suggested to continue the study of monitoring of malignant new formations of maxillofacial localization of population and level of motivation of providing of the specialized help a population with the obligatory analysis of information; to count the indexes of timely diagnostics of shrine one of basic criteria of estimation of activity of stomatological service; to oblige doctors--necessarily to pass stomatologies courses of the in-plant training on oncology 1 time in 5 years.

Introduction: Fibro-osseous lesions, a diverse group of bone disorders including developmental, reactive or dysplastic diseases and neoplasms, share overlapping clinical, radiographic and histopathologic features and demonstrate a wide range of biological behaviour., Aim: To evaluate the characteristics, treatment and outcome of benign fibro-osseous lesions of the jaws in children., Patients and Method: All patients with fibro-osseous lesions of the jaws treated at the department of Oral and Maxillofacial Surgery of the «A & P Kyriakou» Children's Hospital of Athens from 2000 to 2011 were included in this study. Data were retrieved from patients' files and their present situation was registered., Results: Sixteen males and 10 females (mean age 8.5 years) were treated. Fibrous dysplasia was most often encountered (26.9%), and the mandible was the most frequent location (76.9%). All cases were surgically treated and histopathologically confirmed. Marginal ostectomy was performed in 7 cases, partial ostectomy in 4, enucleation and curettage in 10 and trimming-remodelling in 5 cases. Mean follow-up was of 5.5 years with no recurrence, except in one case of fibrous dysplasia., Conclusions: Fibro-osseous lesions, although sharing similar microscopic features, exhibit a variety of clinical behaviour rendering their treatment highly individualized., (Copyright © 2012 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

We conducted a retrospective study of 36 cases of ameloblastoma from the files of Mittaphap Hospital in Vientiane Capital, Laos. Clinical findings showed an average patient age of 31.0 ± 3.8 years, with a slight male preponderance. Radiographically, all of the lesions showed multicystic radiotranslucency. Twenty-four patients underwent tumor resection and 12 underwent tumor enucleation. Of those patients, four underwent additional surgery because of tumor recurrence. This is the first report in the English literature of ameloblastoma cases from Laos.