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Neuroblastoma is the most common extracranial solid cancer of infancy, occurring mainly in the adrenal gland, with high metastatic potential. However, involvement of the head and neck region is rare. Here, we present two cases of metastatic neuroblastoma of childhood, in which a mandibular swelling was the first sign of disseminated disease. Case 1 describes a 4-year-old boy with a 2-week history of painful swelling in the left mandibular region, body soreness and weakness. Panoramic radiography and computed tomography showed a destructive lesion in the left mandibular ramus. Case 2 describes a 3-year-old boy with a 1-month history of swelling in the right mandibular area. Panoramic radiograph and cone-beam computed tomography showed a destructive lesion in the right body and ramus of the mandible, displacing tooth germs, with the destruction of vestibular and lingual bone cortices. In both cases, microscopic analyses revealed a diffuse proliferation of small, round, and blue cells with hyperchromatic nuclei and scant cytoplasm. While Case 1 was more undifferentiated, Case 2 presented eosinophilic areas suggestive of neuropil. A large immunohistochemical panel was performed, showing expression of neural markers such as CD56, neuron-specific enolase (in Case 2), chromogranin, and synaptophysin. Both lesions presented a high proliferation index (Ki67 > 70% and 80%, respectively). Positron emission tomography-computed tomography revealed ipsilateral adrenal primary lesions in both cases, with multiple bone metastatic lesions. Besides the mandible, multiple sites of the axial and appendicular skeleton were affected. Treatment consisted of induction chemotherapy, adrenalectomy, consolidation chemoradiotherapy, and post-consolidation therapy., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC part of Springer Nature.)

Background: To evaluate the specific growth rate (SGR) of ameloblastoma., Material and Methods: cases of ameloblastoma initially underdiagnosed (e.g. cases overlooked or diagnosed as reactive lesions) which had adequate radiographic documentation to evaluate their progression were retrospectively selected. Two panoramic radiographs were analyzed to determine the specific growth rate (SGR) of each tumor, defined as the logarithm of the ratio of final tumor area (when the diagnosis of ameloblastoma was made) to the initial tumor area (when the lesion was underdiagnosed), divided by the time interval between the radiographic images. The tumor area was measured using the software ImageJ., Results: Twelve patients with mandibular ameloblastomas were selected, including 5 males and 7 females, with a mean age of 24.9 years (range: 14-61 years). In four cases, the lesion was associated with the crown of an impacted third molar. In three cases, it was initially diagnosed as a periapical lesion. Three cases were extrafollicular and were not noticed in the initial radiographs. Two cases were initially diagnosed as ameloblastoma, but the surgery was delayed for personal reasons. The mean interval of time between the two radiographic images was 4.3 years (range: 0.4-9 years). Based on our analysis, ameloblastoma grows in average 40.4% per year (range: 14.9-88.7%)., Conclusions: Ameloblastoma is a progressively growing tumor, but its growth rate seems to be smaller than initially reported in the literature. Better understanding the radiographic progression of ameloblastoma might improve its early diagnosis, management, and prognosis.

The purpose of this study was to perform a systematic review of the literature concerning all documented cases of malignant transformation of craniomaxillofacial fibro-osseous lesions (FOLs). Three electronic databases were searched. Data were evaluated descriptively. Kaplan-Meier survival curves were constructed and compared using the log-rank test. A critical appraisal of included articles was performed through the Joanna Briggs Institute tool. A total of 19 studies including 27 patients were selected for data extraction. Twenty-six cases were initially diagnosed as fibrous dysplasia and one as ossifying fibroma. The mean age at the time of malignant transformation was 38.11 years, and the average time from initial diagnosis to malignant transformation was 18.2 years. The male:female ratio was 1:1.2, and the maxilla:mandible ratio was 1.5:1. The histological type of the malignant tumor was predominantly osteosarcoma. Follow-up was available for 21 patients. The 3-year overall survival rate was 51%. Mandible tumors and diagnoses other than osteosarcoma tended to have poor survival rates, but no significant difference was identified. We concluded that between all FOLs, only fibrous dysplasia seems to have a considerable increased risk of malignant transformation. Thus, a regular and long follow-up period is advised., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Ameloblastic carcinoma is rare, accounting for 1.5%-2.0% of all odontogenic tumors. Few small series are available, but data on its clinicopathologic characteristics derive mainly from single case reports; therefore, descriptions of new cases may help to better understand the biological characteristics of this rare odontogenic malignancy. In the current report we describe an ameloblastic carcinoma affecting a 27-year-old female patient who had a previous diagnosis of ameloblastoma 7 years before. The carcinoma featured extensive areas of squamous differentiation resembling a primary intraosseous squamous cell carcinoma, as well as areas of dedifferentiation, a poorly documented histologic characteristic of ameloblastic carcinoma. This case provides new insights on the microscopic spectrum of ameloblastic carcinoma, permitting a critical discussion of the current World Health Organization classification of this odontogenic tumor., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Non-Hodgkin lymphomas are a group of disorders involving malignant monoclonal proliferation of lymphoid cells, which appear at extranodal sites in approximately 40% of the cases, particularly in the gastrointestinal tract. Intraosseous lymphomas of the head and neck region are extremely rare and can mimic other diseases such as periodontitis or periapical pathologies. This report presents an additional case of intraosseous lymphoma that was previously misdiagnosed as periapical disease. In addition, a literature review was made based on PubMed, and all cases of periapical lymphoma were analyzed. After the diagnosis of lymphoma, the current patient was treated with 6 cycles of chemotherapy and showed satisfactory outcome. The literature review displayed 29 cases of lymphoma affecting the periapical region, and in 51.7% of them endodontic treatment was performed previously to the diagnosis of lymphoma. Although lymphoma is uncommon in the oral cavity, some symptoms can assist the dentist to suspect malignant conditions, mainly in cases presenting numb chin syndrome., (Copyright © 2015 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.)

Introduction: The purpose of this study was to describe a series of unicystic ameloblastomas (UAs) that presented clinical and radiographic similarities to apical periodontitis in order to call the attention of clinicians to the fact that several different diseases are able to mimic endodontic periapical lesions., Methods: A retrospective clinicopathological analysis of 5 patients who had periapical lesions in the mandible initially diagnosed as apical periodontitis but with a final histologic diagnosis of UAs is presented., Results: Three men and 2 women with a mean age of 26.4 years presented lesions involving the posterior mandible. No patient reported pain, and 3 patients showed pulp vitality of the involved teeth. Radiographically, all lesions presented periapical well-defined radiolucencies, ranging from 2 to 3 cm in diameter, and 3 cases presented root resorption. Endodontic therapy was applied in 2 patients who were referred to our clinics because of a lack of involution of the periapical lesions. All patients were submitted to periapical surgical curettage and histologically diagnosed as UA. After a mean follow-up of 45.6 months, all patients are well without signs of recurrence., Conclusions: UA may involve the periapical area of teeth, mimicking an inflammatory lesion of endodontic origin. The diagnosis of periapical lesions should be determined based on clinical and radiographic aspects. Recalcitrant lesions should be surgically explored and submitted to histologic evaluation. The early diagnosis of nonendodontic lesions mimicking apical periodontitis such as UA may prevent unnecessary endodontic treatment and wide surgical resections., (Copyright © 2013 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.)

Objective: The aim of this study was to describe the clinicopathologic and immunohistochemical characteristics of 14 cases of central odontogenic fibroma (COF), and the ultrastructural features of 2 of them., Study Design: Collaborative retrospective study based on the records of 4 oral pathology diagnostic services in Latin America based on the current World Health Organization classification., Results: There were 7 male and 7 female patients (mean age 31.8 years). Eight tumors occurred in the maxilla and 6 in the mandible. Thirteen cases were epithelium-rich and 1 epithelium-poor COF. Three were classified as hybrid COF with giant cell lesion. Mean size of the hybrid lesions were larger than pure COF (3.8 vs. 2.4 cm). Odontogenic epithelial islands were immunoreactive for cytokeratin (CK) AE1/AE3, CK5, CK14, CK19, and 34BE12 and negative for CK1 and CK18. Langerhans cells positive for S-100 and CD1a were found within the epithelial islands in 6/6 tested cases. CD68 was expressed in the giant cells of the hybrid lesions and in a few mononuclear cells of 2 cases of COF. Ki-67 index was <1% in all cases. In 6 tumors (42.8%), there were small globular eosinophilic droplets within the epithelial islands, which were positive for collagen type IV, and 9/13 cases (69.2%) were focally positive for smooth muscle actin. In addition to fibroblasts, myofibroblastic differentiation was found in the 2 cases studied ultrastructurally., Conclusions: Immunohistochemistry was useful to confirm the presence of epithelium and to exclude other central fibrous tumors. COF also contains a variable number of mast cells, Langerhans cells, and myofibroblasts, and further studies are needed to better understand the participation of these cells in COF histogenesis., (Copyright © 2011 Mosby, Inc. All rights reserved.)

The aim of this study was to analyze the clinico-pathological and immunohistochemical features of 62 cases of odontogenic myxoma (OM) diagnosed in three Oral Pathology Diagnostic Services in Latin America, as well as to describe the ultrastructural features of three of these cases. OM showed a wide age range (9-71 years), with a mean of 27.97 yr (SD: 11.01) and a male to female ratio of 1:2.2. Mandible was affected in 37 cases (59.6%) and maxilla in 25 (40.4%), with 61.3% located in the posterior region. Thirty-nine cases (62.9%) were multilocular and 23 (37.1%) unilocular. Size ranged from 1 to 13 cm, (mean: 5.2 cm). Thirty-seven multilocular (54.8%) and 6 unilocular lesions (26%) were larger than 4 cm (p<0.05). Epithelial islands were identified in 5 cases (8%) on H&E stained sections, but AE1/AE3 and CK14 disclosed these structures in 15 cases each (24.2%); CK5 was positive in 8 (12.9%); CK7 in 2 (3.2%) and CK19 in only 3 cases (4.8%). All cases were negative for CKs 8 and 18, S-100 protein, NSE and CD68, and showed a low index of expression of Bcl2 and ki-67 proteins (<1%). Mast cell antibodies showed these cells in 45 cases (72.6%). Myofibroblastic differentiation evidenced by myofilaments and fibronexi was found in one case out of the three studied by TEM and 29 cases (46.7%) were positive by immunohistochemistry for alpha actin. In conclusion, only a minority of OM had epithelial islands, and only 3 cases expressed CK 19, indicating an odontogenic epithelium origin. Immunohistochemical and ultrastructural findings suggest that OM is a mesenchymal neoplasm in which several factors may contribute to its pathogenesis, including myofibroblastic differentiation and the participation of mast cell products. However, further investigations are needed to better understand the participation of these elements in this particular neoplasm.

A 42-year-old woman presented a large, nontender, quickly progressive mass in the left mandible. Radiograph showed extensive destruction of the angle, posterior body, and ramus of the left side of the mandible. The patient was surgically treated by hemimandibulectomy. Microscopically, the tumor was composed of large epithelioid cells, many of them showing polarized nuclei, and evident eosinophilic cytoplasm. The predominant pattern was trabecular, and rosette-like structures were also observed. Typical osteoid-containing cells surrounded by malignant cells were found in a few areas. Immunohistochemistry for a large panel of antibodies showed positivity for osteocalcin, osteonectin, osteopontin, VS38c, and S-100. CD34 saliented the hemangiopericytoma-like distribution of the blood vessels. Collagen I was focally positive for the extracellular matrix and malignant osteoid. All other markers were negative, including vimentin and cytokeratins. To the best of our knowledge, this is the first case of epithelioid osteosarcoma affecting the mandible and the second case affecting the jaws that has been reported in the literature.

Adenocarcinoma of the lung that metastasizes to the mandible is very uncommon; only a few cases have been described in the English-language literature. This article presents a metastasis from adenocarcinoma of the lung affecting the mandible of a 64-year-old woman, in which the first discovered metastatic lesion was detected before the primary tumor. The immunoreactivity for human thyroid transcription factor-1 (TTF-1) in the oral lesion was essential for determining the site and type of the primary tumor, as the patient showed no clinical or radiographic evidence of a tumor in the thyroid gland. After the primary tumor in the lung was diagnosed, radiotherapy and chemotherapy were initiated; unfortunately, the patient died two months after the start of treatment. This article emphasizes the importance of a well-conducted examination for diagnosing metastatic oral lesions.

Fibrosarcoma is a malignant mesenchymal neoplasm of fibroblasts that rarely affects the oral cavity and can cause local recurrences or metastasis. In this article we describe a case of primary fibrosarcoma in the mandible in a 16-year-old girl. Microscopically the tumor showed an intense proliferation of spindle-shaped cells, varying little in size and shape and arranged in parallel bands, partly crossing each other, the mitotic activity was increased and there was nuclear pleomorphism. Immunohistochemically the cells only showed immunoreactivity for vimentin and negativity for S-100 protein, CD 68, cytokeratin cocktail, HMB-45, CD34, pan actin HHF 35, desmin, smooth muscle actin and epithelial membrane antigen (EMA). Based on clinical, radiological, histological and immunohistochemical findings the final diagnosis was high-grade intra-osseous fibrosarcoma. The treatment choice was radical surgery with mandibular reconstruction. After one year and nine months of the treatment the patient displayed multiple metastases. Radiation therapy and chemotherapy were used as adjuvant treatment. Unfortunately, the girl died two years after initial diagnosis.

We report two cases of an uncommon odontogenic lesion, previously described as adenomatoid dentinoma. They were well-circumscribed unilocular radiolucent lesions exhibiting discrete radiopacities, located in the left mandibular third molar region. Microscopically they were composed of odontogenic hard and soft tissues, similar to a dental germ. Dental papilla and dentin were easily identified. Odontogenic epithelium formed adenomatoid-like structures, and by scanning electron microscopy a layer of enamel was seen in contact with the dentin. Based on these clinical, radiographic, histological and electron microscopical features we proposed the diagnosis of adenomatoid odontogenic hamartoma. Treatment consisted of surgical removal, and no recurrence was observed. In our opinion all similar cases previously reported pertain to the same spectrum of this lesion and thus should be named as suggested above. Moreover, ultrastructural observations using 5 microm sections can be useful to better characterize the presence of hard tissues.

Adenomatoid Odontogenic Tumour (AOT) is a relatively uncommon odontogenic lesion and few studies describing its cytokeratin profile have been reported in the English-language literature. Thirty-nine cases of AOT from three Oral Diagnosis services (Brazil, Mexico and Guatemala) were studied, considering their clinical, radiographic, and histological features and immunohistochemical expression of cytokeratins (AE1/AE3, 34betaE12, CK1, CK5, CK6, CK7, CK8, CK10, CK13, CK14, CK16, CK18, and CK19), vimentin and Ki-67. Sixty five percent of cases affected women, anterior maxilla was the preferred site and radiographically most cases showed unilocular radiolucency with well defined sclerotic borders. Calcifying epithelial odontogenic tumour (CEOT)-like areas were found in 36 out of 39 cases, and 10 cases showed positivity for Congo red in polarized light. All cases were positive for AE1/AE3, 34betaE12, CK5, CK14 and CK19. CEOT-like areas were negative for CK 19. Vimentin was also expressed in 27 cases and this profile may indicate the existence of a variable phenotype in certain areas of the tumour. There were no recurrences after surgical treatment, and this can be related to the low proliferative activity observed in all cases with Ki-67 marker.