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Purpose: Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant odontogenic tumor that originates from odontogenic epithelial remnants. It is often difficult to diagnose PIOSCC definitively; hence, extraction or surgical treatment is performed before the initial diagnosis in most cases. The present study examined new insights into and prognostic factors of patients with PIOSCC admitted to the authors' department., Materials and Methods: An extensive record review was conducted of patients who underwent radical surgery for PIOSCC from January 2001 through December 2014., Results: Of all cases of OSCC, the frequency of PIOSCC was 1.45%. The 2-year relapse-free survival (RFS) and overall survival (OS) rates were 50.0 and 41.6% in all cases, respectively. Three patients underwent surgery or tooth extraction before the initial diagnosis; in fact, intervention before initial diagnosis was found to be an important poor prognostic factor for RFS and OS. In contrast, patients who were not treated before the initial diagnosis was made did not exhibit any locoregional recurrence., Conclusions: The treatment of PIOSCC should be similar to that for oral cancer with at least clinical stage T3N0 in the National Comprehensive Cancer Network clinical practice guidelines. In addition, cases of PIOSCC that are not treated before the initial diagnosis are more likely to obtain a good prognosis., (Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Primordial odontogenic tumor (POT) was first described in 2014. It typically presents in the posterior mandible of a child or adolescent as a "dentigerous cyst-like" well-circumscribed radiolucency associated with an unerupted molar. POT consists of an ellipsoidal mass of dental papilla-like myxoid connective tissue entirely enveloped in a delicate membrane of ameloblastic epithelium. It shows features of a developing tooth with a huge dental papilla, and because it is devoid of dental hard tissue, it could be regarded a soft tissue odontoma. The lesion histologically mimics early (primordial) stages of tooth development. This report describes a case of POT and POT-like proliferations in an unrelated complex odontoma., (Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: Dentinogenic ghost cell tumor (DGCT) is a very rare odontogenic lesion, with most knowledge based on single case reports. Therefore, a comprehensive analysis was performed of the clinical and radiologic features of reported cases of DGCT with an emphasis on treatment modalities., Material and Methods: This is a case series of DGCTs collected from the literature after a systematic search of Medline's PubMed and Google Scholar. Three additional cases were included from the authors' files. Demographic data of the patients, lesion site and size, and radiologic features were analyzed. Treatment approach and events of recurrence were recorded., Results: Forty-five cases (42 from the literature) were included. The mean age of patients was 39.7 ± 19.3 years (range, 12 to 79 yr) and the male-to-female ratio was 1.8:1. The mandible-to-maxilla ratio was 1.14:1, with the posterior region of the jaws being the most commonly involved site. Radiographically, 78% lesions were unilocular, 67% were mixed radiolucent and radiopaque, and 68% had well-defined borders. The mean lesion size was 4.0 cm (range, 1.8 to 13.0 cm). The primary treatment for 21 patients was conservative surgery consisting of enucleation or curettage. Follow-up information for longer than 1 year (mean, 6.2 ± 8.3 yr; range, 1 to 31 yr) was known for 15 patients, of whom 11 (73%) had recurrences. The primary treatment in 19 patients was radical surgery consisting of marginal or segmental resection. Follow-up information for longer than 1 year (mean, 3.3 ± 2.6 yr; range, 1 to 10 yr) was known for 12 patients, of whom 4 (33%) had recurrences., Conclusions: This study highlighted the potentially aggressive biological behavior of DGCTs that demands extensive surgery and long follow-up. However, owing to the rarity of DGCT, more well-documented cases with long follow-up periods are needed to further define the optimal treatment modalities and prognosis., (Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Currently, central cemento-ossifying fibroma is classified by the World Health Organization as a primary bone-forming tumor of the jaws. However, histopathologically, it is often indistinguishable from cemento-osseous dysplasias in that it forms osteoid and cementicles (cementum droplets) in varying proportions. It is believed that pluripotent cells within the periodontal membrane can be stimulated to produce either osteoid or woven bone and cementicles when stimulated. If this is true, cemento-ossifying fibroma would be better classified as a primary odontogenic neoplasm arising from the periodontal ligament. Cemento-ossifying fibromas also do not occur in the long bones. The present report compares several entities that fall within the diagnostic realm of benign fibro-osseous lesions and reviews the evidence for reclassifying central cemento-ossifying fibroma as a primary odontogenic neoplasm., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Ameloblastic fibrosarcoma is a mixed odontogenic tumor that can originate de novo or from a transformed ameloblastic fibroma. This report describes the case of a 34-year-old woman with a recurrent, rapidly growing, debilitating lesion. This lesion appeared as a large painful mandibular swelling that filled the oral cavity and extended to the infratemporal fossa. The lesion had been previously misdiagnosed as ameloblastoma. Twenty months after final surgery and postoperative chemotherapy, lung metastases were diagnosed after she reported respiratory signs and symptoms., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Odontogenic sarcomas and their subtypes are very rare tumors. The authors' objectives were to report an additional case of ameloblastic fibrosarcoma, review the clinicopathologic features, discuss their treatment approach, and complete a thorough review of the literature., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

The present study is a case report of a 3-year-old girl who was referred to our clinic with the clinical features of cherubism. A locally aggressive tumor was diffusely infiltrating the maxilla and mandible. At 4 years after resection, our patient has not demonstrated any signs of recurrence, which might point to a role for adjunctive chemotherapy, in this case imatinib (Gleevec), for odontogenic myxoma., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to report the histopathologic findings after postdecompression definitive treatment of odontogenic cystlike lesions and determine whether the diagnosis was consistent with the pretreatment diagnosis, thereby answering the clinical question: does decompression change the histologic diagnosis?, Materials and Methods: The authors implemented a retrospective cohort study from a sample of patients diagnosed with a benign odontogenic cystlike lesion and who underwent decompression followed by definitive surgery as part of their treatment. The predictor variable was treatment by decompression and the dependent variable was change in histologic diagnosis. Age, gender, and lesion location were included as variables. The χ(2) test was used for statistical analysis of the categorical data and P values less than .05 were considered statistically significant., Results: Twenty-five cysts and cystlike lesions in 25 patients were treated with decompression followed by enucleation and curettage. The mean age was 34 years (range, 13 to 80 yr) and 56% (14) were male patients. Lesions were located in the mandible in 76% (19 of 25) of patients. Postdecompression histologic examination at the time of definitive surgical treatment was consistent with the preoperative biopsy diagnosis in 91% (10 of 11) of keratocystic odontogenic tumors, 67% (2 of 3) of glandular odontogenic cysts, 75% (3 of 4) of dentigerous cysts, and 100% (7 of 7) of cystic ameloblastomas., Conclusions: The histologic diagnosis at time of definitive treatment by enucleation and curettage is consistent with the predecompression diagnosis. Therefore, all lesions should be definitively treated after decompression based on the initial lesion diagnosis, with all patients placed on appropriate follow-up protocols., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. All rights reserved.)

Clear cell odontogenic carcinoma (CCOC) is an extremely rare neoplasm, with only 74 cases in the English-language literature. It displays a propensity for the mandible, most commonly presenting in the fifth to seventh decades. Histopathologically, CCOC is characterized by sheets and islands of vacuolated and clear cells. The aggressive nature of CCOC was noted in its first description in 1985, although it was not formally classified as malignant by the World Health Organization until 2005. This report describes a case of CCOC presenting atypically in a young patient and at an uncommon site. The authors review the details of this case, outlining management strategies referencing their experience and that described in the other limited cases in the literature., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Twenty-four reports describing 39 cases of primary intraosseous carcinoma (PIOC) were reviewed and the clinicopathologic features were summarized. The mean age of the patients at the time of diagnosis was 51.0 years, and the male: female ratio was 2:3. The posterior mandible was the predominant site; in no patient was a lesion observed in the posterior maxilla. Twenty-five of 31 patients (80.6%) showed swelling of the oral mucosa. However, ulcer formation was observed in only 3 of 36 patients. Pain, sensory disturbances, and metastasis to regional lymph nodes were observed in 25 of 34 patients (73.5%), 9 of 15 patients (60%), and 13 of 33 patients (39.4%), respectively. Radiographically, most lesions produced bone resorption with ill-defined margins (51.6%) or with well-defined margins (19.4%). The diagnostic criteria proposed for PIOC are absence of ulcer formation, except when caused by other factors; histologic evidence of squamous cell carcinoma without a cystic component or other odontogenic tumor cells; and absence of another primary tumor on chest radiographs obtained at the time of diagnosis and during a follow-up period of more than 6 months.

The dental follicle is radiographically and histologically observed in association with unerupted or impacted teeth. However, this normal tissue structure is often confused with odontogenic tumors by pathologists with limited experience in evaluating jaw lesions. This study was designed to evaluate the incidence and possible reasons for incorrect interpretation of dental follicles. From 1970 to 1988, 847 dental follicles and/or dental papillas from 663 patients were submitted to the Armed Forces Institute of Pathology (AFIP) by medical pathologists seeking diagnostic consultation. Nearly 84% of patients were in the second and third decades of life. The male to female ratio was 1.4:1.0. Over 70% of specimens were obtained from around impacted third molar teeth. Fifty-three percent of specimens were correctly interpreted by the contributing pathologists. Only a descriptive interpretation was given for 17%, no diagnosis was made in 10%, and 20% were incorrectly diagnosed. In descending order, the most frequent incorrect diagnoses were odontogenic cyst, odontogenic myxoma, odontogenic fibroma, ameloblastic fibroma, odontoma, and ameloblastoma. Dental papillas were most frequently misdiagnosed as odontogenic myxomas. The histologic features and diagnostic pitfalls are discussed, as well as the need to consider the clinical, radiographic, and microscopic features in arriving at a final diagnosis.

A retrospective review of experience with jaw tumors in 48 children over a ten-year period was made. These were predominantly benign, nonodontogenic, mesenchymal tumors. Clinical behavior was variable and often did not correspond to the histologic patterns. The clinically aggressive variants displayed rapid growth usually with associated pain. Resection rather than curettage is appropriate therapy when there has been earlier treatment failure by local tumor removal or rapid tumor expansion, or when tumor extensively invades the jaw. When resection is carried out, facial growth is not adversely affected if early reconstruction with autogenous bone is performed.