Comprehensive Genomic Analysis of Cemento-Ossifying Fibroma.

Cemento-ossifying fibroma (COF) of the jaws is currently classified as a benign mesenchymal odontogenic tumor, and only targeted approaches have been used to assess its genetic alterations. A minimal proportion of COFs harbor CDC73 somatic mutations, and copy number alterations (CNAs) involving chromosomes 7 and 12 have recently been reported in a small proportion of cases. However, the genetic background of COFs remains obscure. We used a combination of whole-exome sequencing and RNA sequencing to assess somatic mutations, fusion transcripts, and CNAs in a cohort of 12 freshly collected COFs. No recurrent fusions have been identified among the 5 cases successfully analyzed by RNA sequencing, with in-frame fusions being detected in 2 cases (MARS1::GOLT1B and PARG::BMS1 in one case and NCLN::FZR1 and NFIC::SAMD1 in the other case) and no candidate fusions identified for the remaining 3 cases. No recurrent pathogenic mutations were detected in the 11 cases that had undergone whole-exome sequencing. A KRAS p.L19F missense variant was detected in one case, and 2 CDC73 deletions were detected in another case. The other variants were of uncertain significance and included variants in PC, ACTB, DOK6, HACE1, and COL1A2 and previously unreported variants in PTPN14, ATP5F1C, APOBEC1, HDAC5, ATF7IP, PARP2, and ACTR3B. The affected genes do not clearly converge on any signaling pathway. CNAs were detected in 5/11 cases (45%), with copy gains involving chromosome 12 occurring in 3/11 cases (27%). In conclusion, no recurrent fusions or pathogenic variants have been detected in the present COF cohort, with copy gains involving chromosome 12 occurring in 27% of cases.

Synchronous cemento-ossifying fibromas: a systematic review.

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

Role of collagen and immunostaining for TGF-ß in the clinical and microscopic findings of pyogenic granuloma and peripheral ossifying fibroma.

BACKGROUND: Collagen is a component of Pyogenic Granuloma (PG) and Peripheral Ossifying Fibroma (POF) and performs different functions in these lesions. The objective of this study is to evaluate the role of collagen and immunostaining for Transforming Growth Factor beta (TGF-ß) in the clinical and microscopic findings of PG and POF. MATERIAL AND METHODS: PG (n=20) and POF (n=20) were selected for clinical evaluation (sex, age, localization, size and evolution time) and microscopic analysis (picrosirius red staining for collagen analysis and immunohistochemistry for TGF-ß) performed in the superficial and deep areas of the two lesions. ANOVA/Bonferroni and t-test, Pearson correlation and χ2 were used to compare the sites and parameters analyzed (p<0.05, GraphPad Prism 5.0). RESULTS: The depth of PG presented the highest amount of collagen (p<0.001), and its surface showed the lowest amount of type 1 collagen (yellow-red strong birefringence). Type 1 collagen gradually increased in depth of PG, surface and depth of POF (p<0.001). The number of TGF-ß+ cells was lower on the surface of PG compared with the depth of PG and the two areas of POF (p<0.001). Sex and localization did not affect these parameters, but the profile of collagen and immunostaining for TGF-ß suffered from modifications by the time of evolution and the size of the lesion. CONCLUSIONS: Although PG and POF are reactive gingival lesions, the expression of TGF-ß and its role in collagen showed different biological behaviors in these lesions, suggesting different biological origins for its components.

Spontaneous Regression of Ossifying Fibroma in the Oral and Maxillofacial Region: A Rare Case With 4-Year Follow-Up.

The case presented in this study is a rare instance of an ossifying fibroma (OF), a type of benign fibro-osseous lesions, spontaneously regressed without surgical intervention. A 9-year-old boy with an intraosseous lesion in the left maxilla was diagnosed as OF. The surgeon suggested surgical excision, but due to personal reasons, the patient and his parents deferred the surgery and opted for regular follow-up. During a 4-year follow-up, the surgeon found that the lesion had significantly decreased in size and the facial deformity had remitted. At the latest follow-up, the deformity of his left face became almost unnoticeable, and the lesion seemed to be subtle on cone-beam computed tomography images. This case highlights the possibility of spontaneous regression of OF. This phenomenon may occur due to the teeth eruption, which can lead to the formation of periodontal ligaments and engage the regression of OF.

Rapport de cas Ossifying fibroma in the cervical vertebra of a dog.

The objective of this study was to describe the clinical signs, neurologic examination findings, diagnostic imaging results, and pathologic diagnosis of ossifying fibroma in the cervical vertebra of a dog. A 3-year-old spayed female Pembroke Welsh corgi dog exhibited severe cervical pain and left-sided postural reaction deficits. Magnetic resonance imaging (MRI) revealed a lobulated contrast enhancing mass associated with the C6 cervical vertebra. Due to the lack of response to pain medications, humane euthanasia was elected, and histopathologic evaluation of the mass revealed a fibro-osseous lesion most consistent with an ossifying fibroma. This neoplasm is most commonly associated with the mandible of young horses and has not been previously reported in vertebrae in veterinary medicine. Key clinical message: This case is the first report of a fibro-osseous lesion most consistent with an ossifying fibroma affecting a vertebra in veterinary medicine.

Fibrome ossifiant dans la vertèbre cervicale d'un chien. Décrire les signes cliniques, les résultats de l'examen neurologique, les résultats de l'imagerie diagnostique et le diagnostic pathologique du fibrome ossifiant dans la vertèbre cervicale d'un chien. Une chienne Pembroke Welsh corgi femelle stérilisée âgée de 3 ans présentait de fortes douleurs cervicales et des déficits de réaction posturale du côté gauche. L'imagerie par résonance magnétique (IRM) a révélé une masse lobulée augmentant le contraste associée à la vertèbre cervicale C6. En raison de l'absence de réponse aux analgésiques, l'euthanasie a été choisie et l'évaluation histopathologique de la masse a révélé une lésion fibro-osseuse plus compatible avec un fibrome ossifiant. Ce néoplasme est le plus souvent associé à la mandibule des jeunes chevaux et n'a jamais été signalé auparavant dans les vertèbres en médecine vétérinaire.Message clinique clé :Ce cas est le premier rapport d'une lésion fibro-osseuse plus compatible avec un fibrome ossifiant touchant une vertèbre en médecine vétérinaire.(Traduit par Dr Serge Messier).

A large-cohort study of 2971 cases of epulis: focusing on risk factors associated with recurrence.

BACKGROUND: To analyze the clinicopathological features of different histological subtypes of epulis, and evaluate the risk factors associated with recurrence. MATERIALS AND METHODS: A retrospective study including 2971 patients was performed. The patients' sex, age, location, size, histological subtypes, recurrence information, oral hygiene habits, periodontitis symptoms and smoking history were retrieved from the patient medical records and follow-up information. RESULTS: Among the 2971 cases, focal fibrous hyperplasia (FFH) was the most common lesion (60.92%), followed by peripheral ossifying fibroma (POF) (29.32%), pyogenic granuloma (PG) (8.08%) and peripheral giant cell granuloma (PGCG) (1.68%). The peak incidence of epulis was in the third and fourth decade of life, with a mean age of 45.55 years. Female predominance was found in all types of lesions with a female to male ratio of 1.71:1. PG had the highest recurrence rate (17.18%), followed by POF (12.98%), FFH (9.55%) and PGCG (8.82%). Histological subtypes were significantly correlated with the recurrence of epulis (P = 0.013). Regular supportive periodontal therapy (P = 0.050) had a negative correlation with recurrence, whereas symptoms of periodontitis (P < 0.001) had a positive correlation with the recurrence of epulis. CONCLUSIONS: Controlling the periodontal inflammation and regular supportive periodontal therapy might help reduce the recurrence of epulis.

Gingival and alveolar mucosal reactive hyperplastic lesions: a retrospective clinical and histological study of 996 cases.

BACKGROUND: gingival/alveolar mucosal reactive hyperplastic lesions (GRHL), including fibrous hyperplasia (FH), pyogenic granuloma (PG), peripheral ossifying fibroma (POF) and peripheral giant cell lesion (PGCL), are a common group of oral diseases. The aim of the present study was to access the frequency and distribution of the clinical and histological features of these disorders in a Brazilian population. MATERIAL AND METHODS: all specimens diagnosed as GRHL in three Oral Pathology laboratories were selected for the study. Clinical information was retrieved from the laboratory biopsy forms and hematoxylin and eosin stained histological slides were reviewed for analysis of the histological characteristics. RESULTS: final sample was composed of 996 specimens, including 463 FH (47%), 280 PG (28%), 183 POF (18%) and 70 PGCL (7%). Females were more affected by FH, PG, and POF, and most cases affected adults with mean ages ranging from 40 to 53 years. FH, PG, and POF were more common in the upper gingiva/alveolar mucosa. Most PG, POF and PGCL were pedunculated, in contrast with FH (p<0.001). PG, FH and POF were mostly red or normal mucosal in color, while PGCL were mostly red/purple (p<0.001). PGCL were larger, followed by POF, FH and PG (p<0.001). Some histological features were characteristically found in some conditions, but they were also encountered in other lesions with variable frequencies. CONCLUSIONS: Oral medicine specialists, oral pathologists and periodontists are usually the professionals in contact with patients presenting GRHL and it is of upmost relevance that they should be familiarized with their clinical and histological profile.

Synchronous Reconstruction of the Jaw Using a "One and a Half"-Barrel Fibular Bone Graft for Ossifying Fibroma Patients.

BACKGROUND: Ossifying fibroma is one of the common benign tumors that affect the appearance and functions of the jaw. Ossifying fibroma may exhibit a wide range of biological behaviors, leading to deformities involving the jaw and other secondary facial deformities. Hence, to improve the function of the jaw and the patient's general facial appearance (bearing in mind each patient's facial shape and, or appearance), the authors thus, however, used a ''one and a half"-barrel fibular bone graft to achieve the ideal height and radian of the bone graft. CASE PRESENTATION: Between July 2017 and January 2021, the authors retrospectively collected and analyzed clinical and surgical data from 39 patients who had undergone operations in our hospital. Twenty patients were operated on using our new surgical method, whereas 19 patients received conventional or debulking operation. Clinical factors associated with the operation were assessed, including classification of the jaw defects, perioperative complications, and postoperative outcomes. RESULTS: All the flaps ultimately survived. According to the postoperative satisfaction survey, patients who underwent reconstruction were satisfied with their postoperative facial appearance, with an average of 8.5 out of 10. Based on the preoperative clinical data, 26 patients had suitable bone grafts for dentures to improve their oral function. CONCLUSIONS: A ''one and a half''-barrel fibular bone graft effectively improves the facial appearance of patients and as well as provides an appropriate height and radian for the bone graft.

Peripheral ossifying fibroma: A 20-year retrospective study with focus on clinical and morphological features.

BACKGROUND: Peripheral Ossifying Fibroma (POF) is a reactive hyperplastic lesion that exclusively occurs in the gingiva and is characterized by the deposition of dystrophic calcification, cementum-like tissue, and immature and mature bone within the connective tissue. The objective of the present study was to perform a retrospective analysis of clinicopathologic features of POF. MATERIAL AND METHODS: Clinical and histopathological data were obtained from biopsy records and histopathological reports from a Brazilian reference service in Oral Pathology (1999 - 2020). Morphological analysis was performed to evaluate features related to the mesenchymal component, inflammatory infiltrate, ulceration, and mineralized tissue. RESULTS: A total of 270 POFs were diagnosed during the study period. A higher frequency was observed in females (71.9%) between the third (22.9%) and fourth (23.3%) decades of life. The anterior upper gingiva (29.1%) was the most affected region. Mature (86.7%) and immature (52.6%) bone tissue were the most frequent. There was a significant association between immature bone deposition and lesions with size ≤ 1.7 cm (p = 0.041); immature bone and cement-like tissue deposition with an evolution time ≤ 16 months (p < 0.001); deposition of immature bone and mesenchymal hypercellularization (p < 0.001); deposition of dystrophic calcification and the presence of ulceration (p < 0.001). CONCLUSIONS: The clinical characteristics corroborate the findings in the literature. The heterogeneous distribution and quantity of mineralized tissues found in the analyzed cases support the theory that the different mineralized tissues constitute a spectrum of clinical maturation of POF.

A systematic review of the clinical and radiographic features of hybrid central giant cell granuloma lesions of the jaws.

OBJECTIVE: Central giant cell granuloma (CGCG) can coexist with other benign lesions of the jaw. These hybrid lesions are diagnostically challenging to both oral pathologists and radiologists. This work systematically reviews the clinical and radiographic features of hybrid-CGCG lesions in the jaws. MATERIALS AND METHODS: Three reviewers conducted an electronic search of five databases for histologically diagnosed hybrid-CGCG lesions in human jaws. RESULTS: Thirty-four of 1224 articles met the inclusion criteria. Of 39 hybrid-CGCG lesions, 14 (35.9%) were central odontogenic fibroma, 11 (28.2%) were central ossifying fibroma, seven (17.9%) were fibrous dysplasia, and seven (17.9%) were other bone conditions. There were 22 females and 17 males with a mean age of 30.5 ± 19.9 years. 89.5% of hybrid-CGCG lesions were well defined, 57.9% were non-corticated, 60.5% were radiolucent, and 66.7% were in the posterior mandible. Most hybrid lesions affected the cortical plates by thinning, expansion, or perforation (93.1%), displaced, or resorbed teeth (60%). CONCLUSION: The radiographic features of hybrid-CGCG lesions vary according to the concurrent bony lesion. Hybrid-CGCG lesions altered the radiographic appearance with the following entities: fibrous dysplasia, melorheostosis, and Paget's disease. Optimal imaging modalities are crucial to detail radiographic features and direct representative biopsy of suspicious sites that may host a CGCG hybridisation.

Fibro-Osseous Lesions Of The Craniofacial Complex In Horses: 30 Cases (2001-2019).

OBJECTIVE: To describe the presentation, diagnosis, treatment, and outcome for horses with fibro-osseous lesions of the craniofacial complex. STUDY DESIGN: Retrospective multicenter case series. ANIMALS: Thirty horses evaluated for fibro-osseous lesions of the skull from January 1, 2001 through December 31, 2019 in four centers. METHODS: Medical records were reviewed for signalment, clinical presentation, histological and diagnostic imaging findings, treatment instituted, and outcome. Long-term outcome information was obtained by owner questionnaire or the medical record. RESULTS: Diagnoses included ossifying fibroma in 20 of 30 horses, osteoma in eight of 30 horses, and fibrous dysplasia in two of 30 horses. Twelve of 30 lesions were diagnosed in horses <1 year old, and 20 of 30 lesions originated from the rostral mandible. The most common treatment was rostral mandibulectomy. Recurrence was not reported after complete excision. Incomplete excision was confirmed in eight horses (four ossifying fibromas, three osteomas, and one fibrous dysplasia), and follow-up information was available for seven horses. Recurrence occurred in one horse, while six horses had long-term resolution of clinical signs. Prognosis for survival and return to use was excellent in 23 horses with long-term follow-up. CONCLUSION: Fibro-osseous lesions were uncommon in this multicenter study; they were most commonly diagnosed in young animals and most frequently affected the rostral mandible. Long-term survival was excellent. CLINICAL SIGNIFICANCE: The definitive diagnosis of fibro-osseous lesions of the craniofacial complex in horses is made from results of histopathology and cannot be determined on the basis of clinical presentation alone. Surgical excision is indicated, and prognosis can be favorable even when complete surgical margins are not obtained.

Concomitant bilateral mandibular cemento-ossifying fibroma and cementoblastoma: case report of an extremely rare occurrence.

BACKGROUND: Cemento-ossifying fibroma (COF) and cementoblastoma (CB) are rare benign odontogenic tumors with a predilection for the mandible. Cemento-ossifying fibroma is a fibro-osseous lesion that originates in the tooth bearing areas of jaw and shows cementum-like tissue in a fibrotic stroma. Cementoblastoma is classically related to roots of teeth with the presence of calcified cementum-like material. To date, only a single case of concomitant unilateral COF and CB has been reported in the literature. CASE PRESENTATION: We present an unusual case of a 37-year-old female who presented with two discrete bilateral swellings in the right and left mandible for 10 years. The larger tumor involved the left posterior mandible with extension anteriorly to the left and right anterior mandibles, and the smaller tumor was present in right posterior mandible. Radiology revealed two distinct lesions involving both sides of mandible. Histopathological examination showed characteristic features of cemento-ossifying fibroma in sections of the larger tumor and cementoblastoma in sections of smaller tumor. CONCLUSION: This case shows the very unique bilateral co-existence of COF and CB, the second case reported in literature to date.

Juvenile ossifying fibroma of the maxillofacial region: analysis of clinico-pathological features and management.

BACKGROUND: The diagnosis and management of juvenile ossifying fibroma (JOF) remains a highly debated topic with paucity of studies with long-term follow-up, hence the aim of this study was to report on the clinico-pathological features and management of these neoplasms. MATERIAL AND METHODS: A retrospective analysis was performed on all histopathologically confirmed JOF presenting at two tertiary hospitals in Cape Town, South Africa over a period of 39 years. Clinical, demographic, histopathological and radiological features were analyzed. Surgical methods were documented and a minimum post-operative follow-up of 12 months was a prerequisite. RESULTS: Seventeen patients met the inclusion criteria and were included in this study. Overall, the ages of patients ranged from 3-31 years (mean= 13 years) with male to female ratio of 1.8:1. The ages of patients diagnosed with Trabecular JOF were significantly younger than patients with Psammomatoid JOF (P = 0.01). The majority of patients presented with marked swelling (88.2%). Interestingly, most neoplasms occurred in the mandible (76.5%) with all Psammomatoid JOF uncharacteristically occurring in the mandible. There was only one case of Trabecular JOF occurring in the sinonasal area. Most neoplasms appeared as unilocular (76.5%) and well-defined (82.4%) with mixed radio-density (70.6%) on radiographs and computed tomography. Curettage with peripheral ostectomy was shown to be the least invasive method with an acceptable recurrence rate (10%). Six lesions underwent resection without any recurrences however caused high morbidity to these young patients. CONCLUSIONS: The high number of lesions occurring in the mandible for both variants of JOF demonstrates that site should not be a major determining factor in the diagnosis of JOF. Moreover, curettage with peripheral ostectomy should be used as the first line of management to minimize morbidity to the patient and that resection should be reserved for large and recurrent lesions.

Use of the Cover-Lifting Technique in Mandibular Cemento-Ossifying Fibroma Excision to Preserve the Inferior Alveolar Nerve.

Cemento-ossifying fibroma (also known as ossifying fibroma or cementifying fibroma) is a benign osteogenic neoplasm. Pain and paresthesia are rarely associated with cemento-ossifying fibroma; thus, nerves must be preserved during excision. With the advent of computer-aided techniques, the use of virtual surgical planning and a customized template can improve the precision of resection and reconstruction, reduce operating time, and improve postoperative outcomes. In this report, we describe a case of cemento-ossifying fibroma in a female patient who underwent segmental mandibulectomy and reconstruction with an iliac bone graft. Additionally, we describe a simple and effective way to preserve the inferior alveolar nerve.

A Clinical Audit of Histopathologically Diagnosed Ossifying Fibroma of the Jaws in a Nigerian Population.

BACKGROUND: Ossifying fibroma (OF) is a benign tumor of the jaws, which belongs to the group of fibro-osseous lesions, and exhibits slowly progressive growth leading to jaw expansion with well-defined borders. OBJECTIVE: To evaluate the clinical and radiological characteristics of OFs and their treatment. MATERIALS AND METHODS: A 5-year retrospective study was done. The case notes of subjects were retrieved, analyzed, and evaluated. Relevant information about patients was categorized into bio-data, clinical, and radiological. The diagnosis of lesion was made histopathologically by the oral pathologist. RESULTS: Forty- five patients' case notes were retrieved and analyzed and the patients' age ranged from 2 to 70 years (mean 29.4 ± 16.9). Majority of the patients (55.5%; P = 0.23) were 10-29 years. There were 16 (35.6%) males and 29 (64.4%) females giving a male: female ratio of 1:1.8 (P = 0.01). The cross-sectional dimension of the tumors measured vertically and horizontally ranges from 1 cm2 to 420 cm2 (mean = 54.7 cm2 ± 82.9). There were 60% in the maxilla and 40% in the mandible (P = 0.014). Most of the lesions (68.9%; P = 0.01) showed patchy opacity and cotton wool appearances. Majority of the lesions were extirpated by enucleation alone (P = 0.01). CONCLUSION: The prevalence of OF among the fibro-osseous lesions was 51.0% while against other benign lesions, it was 16.0%. In conclusion, contrary to most other reports across the globe, this lesion in southeast Nigeria occurred more frequently in the maxilla than mandible.

Intraoral Approach for Surgical Treatment of Psammomatoid Juvenile Ossifying Fibroma.

Psammomatoid juvenile ossifying fibroma (PJOF) is a rare benign tumor that usually affects the paranasal sinuses, orbit, and skull. In most cases, extensive incisions are necessary for full access to the tumor site. The aim of this paper is to report a case of extensive PJOF in which an intraoral surgical approach was performed with complete excision of the tumor. A female patient, 18-year old had a deforming volume increase in the region of the left facial middle third with an approximate evolution time of 2 years. She complained of headache, epiphora in the left eye, and total obstruction of the left nostril. Extraoral examination showed facial asymmetry with dystopia, ocular proptosis, and considerable sclera exposition of the left eye. Tomography examination showed a mixed aspect lesion on the left side of the face, well delimited. The intraoral surgical approach was chosen for the excision of the lesion. Under general anesthesia and nasotracheal intubation, total resection was performed, followed by exodontia of the directly involved teeth. In the definitive histopathological examination, the diagnosis was confirmed. The patient is currently with 2 years and 6 months of postoperative follow-up and has good healing of intraoral surgical wounds and stable occlusion. Extraoral examination showed harmonic projection of the facial middle third, but still with excessive exposure of the sclera of the left eye due to the defect in the orbital floor. The surgical treatment of PJOF was possible by intraoral approach, even taking into account the limitations of the access and the complexity of the anatomy of the facial bones involved.

[Comparison of radiological and clinicopathological features of craniofacial fibro-osseous lesions].

Objective: To investigate the clinicopathological and radiological features of benign fibro-osseous lesion (BFOL). Methods: Sixty-five cases of craniofacial BFOL, eight cases of peripheral ossifying fibroma (POF) and one case of low-grade central osteosarcoma diagnosed at Sichuan Provincial People's Hospital between January 2010 and March 2019 were collected. The clinicopathologic features, hematoxylin-eosin and immunohistochemical (IHC) staining and radiographic features were analyzed. MDM2 gene amplification was detected by FISH in difficult borderline cases. Results: This cohort of BFOLs included 50 cases of fibrous dysplasia (FD), 12 cases of ossifying fibroma (OF), and three cases of juvenile psammomatoid ossifying fibroma (JPOF). The average ages of patients with FD,OF and JPOF were 31.7, 39.2 and 26.0 years respectively. The male to female ratio was 1.0∶1.8.The average age of POF was 47.0 years, with male to female ratio of 1∶7. Patient of low-grade central osteosarcoma was a 48-year-old man. Twenty-seven cases of FD were located in the jaw, and 23 cases were in other craniofacial bones. Nine cases of OF were located in the jaw, and three cases were in the nasal cavity. Two cases of JPOF were in the nasal sinus, and one was in the jaw. All POF were located in the gingiva, and low-grade central osteosarcoma was located in the mandible. The imaging features of FD were luffa-like or ground-glass like signal shadows with poorly defined borders with expansion. OF had clear borders or sclerosing margins. Both JOF and low-grade central osteosarcoma were expansile intraosseously and with focally invasive nodular masses with ground-glass like signal shadows; and POF showed soft tissue mass with bone formation. Histological features of BFOLs showed mixed fibrous and irregular osteoid lesions. FD had no clear relationship with the host bone and no osteoblasts surrounded the bone trabeculae. Osteoblasts rimming was found in OF, and the boundaries of the host bone were clear. JPOF and low-grade central osteosarcoma infiltrated the host bone focally, and the latter showed mild cellular atypia. MDM2 amplification was detected in low-grade central osteosarcoma. Conclusions: BFOLs are a group of fibro-osseous lesions with similar morphology in the head and neck and face, but their clinical features and prognosis are different; and their imaging and histological characteristics are also slightly different. Attentions should be given to the combination of clinical, imaging and pathologic features of BFOLs, especially the differential diagnosis between BFOLs and low-grade central osteosarcoma. Molecular detection could be used to assist the diagnosis in difficult cases.

Malignant transformation of craniomaxillofacial fibro-osseous lesions: A systematic review.

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.

The use of 3D printing in shared decision making for a juvenile aggressive ossifying fibroma in a pediatric patient.

Juvenile aggressive ossifying fibromas (JAOF) are rare, typically benign pediatric tumors that are locally aggressive and have high recurrence rates. A 7-year old male presented with a palatal mass and a 3D printed model was created and used as a visual aide to highlight the importance of management in terms of functional, cosmetic, and disease-free outcomes with the family. The patient ultimately underwent successful enucleation with final pathology consistent with JAOF. To our knowledge, this is the first description of the use of 3D printing to help in the shared decision-making process for the treatment of this aggressive tumor.

Reconstruction and Mandibular Rehabilitation After Resection of Juvenile Aggressive Ossifying Fibroma Using Undifferentiated Mesenchymal Cells and Osseointegrated Implants: A Case Report.

PURPOSE: The aggressive juvenile ossifying fibroma is a benign fibro-osseous neoplasm that affects the jaws of young patients. The treatment can range from careful curettage to complete bone resection. Early dental rehabilitation through osseointegrated implants is indicated for functional recovery and esthetics. MATERIALS AND METHODS: This article reports the case of a 20-year-old man submitted to osseous resection, installation of a titanium plate associated with bone graft and osteogenic substances with subsequent installation of osseointegrated dental implants. RESULTS: After 28 months of clinical and radiographic follow-up, there were no signs of tumor recurrence. As the patient showed good graft integration, 3 dental implants could be installed and are now waiting to receive a prosthetic work. CONCLUSIONS: The use of a graft with morphogenetic proteins proved to be stable and efficient as it allowed the rehabilitation with dental implants.