Recurrent cementoblastoma with multifocal growth and cellular atypia: a case report.

BACKGROUND: Cementoblastoma is a rare odontogenic tumor characterized by the formation of osteocementum-like tissue on a tooth root directly by neoplastic cementoblasts. Although it is categorized as benign, it has a high potential for growth with a certain degree of recurrence risk. However, there are only a few studies describing the features of recurrent cementoblastoma. The diagnosis of recurrent cementoblastoma is challenging not only due to its cytological atypia but also because of its large size and multicentric growth pattern. These characteristics suggest a potential for malignancy. CASE PRESENTATION: A 29-year-old woman was transferred to our university dental hospital complaining of swelling of the right mandible. She had a history of enucleation of cementoblastoma associated with the third molar of the right mandible. Five years after the initial treatment, imaging demonstrated well-circumscribed multicentric radiopaque lesions in the same area. Histologically, the lesion consisted of osteocementum-like tissue rimmed with polygonal or plump tumor cells. Several cells were large epithelioid cells with bizarre nucleoli, which may be reminiscent of malignant tumors. Otherwise, there were no apparent malignant findings, including proliferative activity or atypical mitotic figure. Besides, tumor cells were positive for c-FOS, a marker of osteoblastoma and cementoblastoma. Eventually, the patient was diagnosed with recurrent cementoblastoma. CONCLUSIONS: Pathological analyses of this case suggested that the recurrent event in the cementoblastoma altered its growth pattern and tumor cell shape. Moreover, in the case of enucleation surgery, long-term follow-up is important because there is some recurrent risk of cementoblastoma, although it is not high.

Cementoblastoma Mimicking Pathology of Endodontic Origin: A Case Report.

Interdisciplinary care in the management of complex pathology is critical to ensure patients receive predictable, evidence-based treatment. The authors report the unique case of a healthy 38-year-old female patient who presented to a private endodontics practice with a radiopaque lesion associated with the root of tooth No. 20. The patient experienced mild and occasional palpation tenderness associated with the tooth. Extraoral examination and intraoral soft tissues presented without any abnormalities. Intraoral radiographs revealed a radiopacity overlying the apex of tooth No. 20. A cone-beam computed tomography scan of the region revealed a well-delineated bulbous radiopaque lesion attached to the lingual aspect of the apical third of the root of tooth No. 20. The lesion could not be distinguished from the root outline and presented with a radiolucent halo along its periphery. The differential diagnosis at the time consisted of cementoblastoma, condensing osteitis, hypercementosis, and periapical cemento-osseous dysplasia. The patient was referred to an oral and maxillofacial surgeon for evaluation and treatment. Tooth No. 20 was subsequently treated with an excisional biopsy and concurrent extraction of the tooth. Histologic examination of the lesion supported the diagnosis of cementoblastoma.

Radiological follow-up of cemento-osseous dysplasia on cone-beam computed tomography.

This study investigated the natural course of cemento-osseous dysplasia (COD) on cone-beam computed tomography (CBCT). Retrospectively, 104 CBCT scans from 36 patients (mean age, 44.5 years; 33 female and three male) with mandibular COD (10 florid, seven focal, 19 periapical) were included, based upon clinico-radiological features, without complications such as infection and related surgery. Changes in maximum diameter and morphology (lytic, mixed lytic-sclerotic, sclerotic) were evaluated in 83 lesions, with a mean follow-up of 28.3 months. The occurrence of a diameter increase was assessed by time-to-event analysis; interreader agreement for diameter and morphological evaluation by intraclass correlation coefficient and weighted κ statistics, respectively. Fifteen of 83 (18.1%) lesions (eight florid, one focal, six periapical) in 10 patients increased in diameter; 12 of 83 (14.5%) lesions (five florid, seven periapical) in 11 patients changed morphologically. The median period until a diameter increase was longest (120 months) for periapical COD, and shortest (66 months) for florid COD (p = 0.023). There was high reader agreement (ICC = 0.891; weighted κ = 0.901). In conclusion, CBCT is an effective tool with which to follow-up COD. If any, the natural progress in uncomplicated COD is prolonged, which underlines its non-surgical character and aids in its long-term management.

Ossifying fibroma mimiking jaw tumour: A radiographic dilema.

Fibro-osseous lesions (FOLs) of the craniomaxillofacial region comprise a group of developmental, dysplastic, and neoplastic alterations. FOLs include ossifying fibromas (OF), cemento-ossifying fibroma (COF), familial gigantiform cementoma (FGC), fibrous dysplasia (FD), and cemento-osseous dysplasia (COD). Evidence suggests that some FOL, especially FD and OF may have a risk of spontaneous malignant transformation. This report documents a rare case of malignant transformation of ossifying fibromas of the jaw and the probable cause for same. Although it is rare, the clinician should have a complete follow up to observe such changes among the patients having FOLs.

Imaging Characteristics of Embedded Tooth-Associated Cemento-Osseous Dysplasia by Retrospective Study.

BACKGROUND: Since there are many differential diagnoses for cemento-osseous dysplasia (COD), it is very difficult for dentists to avoid misdiagnosis. In particular, if COD is related to an embedded tooth, differential diagnosis is difficult. However, there have been no reports on the characteristics of the imaging findings of COD associated with embedded teeth. The aim of the present study was to investigate the occurrence and imaging characteristics of cemento-osseous dysplasia (COD) associated with embedded teeth, in order to appropriately diagnose COD with embedded teeth. METHODS: The radiographs with or without histological findings of 225 patients with COD were retrospectively analyzed. A retrospective search through the picture archiving and communication system (PACS) of the Division of Oral and Maxillofacial Radiology of Kyushu Dental University Hospital was performed to identify patients with COD between 2011 and 2022. RESULTS: Fifteen COD-associated embedded mandibular third molars were identified in 13 patients. All 13 patients were asymptomatic. On imaging, COD associated with embedded mandibular third molars appeared as masses that included calcifications around the apex of the tooth. On panoramic tomography, COD showed inconspicuous internal calcification similar to that of odontogenic cysts or simple bone cysts, especially in patients with COD only around the mandibular third molar region. Those with prominent calcification resembled cemento-ossifying fibroma, calcifying epithelial odontogenic tumor, calcifying odontogenic cyst, adenomatoid odontogenic tumor, and so on, as categories of masses that include calcifications on panoramic tomography and computed tomography. CONCLUSIONS: The current investigation is the first to report and analyze the imaging characteristics of COD associated with embedded teeth. It is important to consider the differences between COD and other cystic lesions on panoramic tomography, and the differences between COD and masses that include calcifications on CT.

Misdiagnosed long-standing unilateral nasal obstruction: ossifying fibroma of the inferior turbinate.

A man in his 20s presented with complaints of unilateral nasal obstruction for the past 6 years that progressively worsened leading to irrational use of over-the-counter nasal decongestants. With the worsening of symptoms, a non-contrast CT was done. It showed a dense expansile sclerotic lesion of the right inferior turbinate, which was excised endoscopically. Cemento-ossifying fibromas of the inferior turbinate are rare and require assessment and surgical excision to relieve the symptom of nasal obstruction. It derives its name from the variable proportions of fibrous and mineralised tissue present in it and exclusively develops in the craniofacial region. It can be surgically managed by an endoscopic, an endonasal non-endoscopic (with a speculum) or an open approach (lateral rhinotomy, sublabial approach or mid-facial degloving). Here, we present how such a case was detected and managed surgically by the endoscopic approach, which is a minimally invasive option with shorter hospital stay and early recovery.

Cemento-ossifying fibroma transforming to osteosarcoma.

Ossifying fibroma is a type of fibro-osseous lesion categorised into cemento-ossifying fibroma and juvenile ossifying fibroma. Malignant transformation of fibro-osseous lesions is documented especially for fibrous dysplasia, but scarcity is seen when we search for malignant transformation of ossifying fibroma. Thus, we are presenting an extremely rare case of cemento-ossifying fibroma transforming into osteosarcoma with long sequential radiographic details.

Familial gigantiform cementoma with recurrent ANO5 p.Cys356Tyr mutations: Clinicopathological and genetic study with literature review.

BACKGROUND: Familial gigantiform cementoma (FGC) is a rare tumor characterized by the early onset of multi-quadrant fibro-osseous lesions in the jaws, causing severe maxillofacial deformities. Its clinicopathological features overlap with those of other benign fibro-osseous lesions. FGC eventually exhibits progressively rapid growth, but no suspected causative gene has been identified. METHODS: In this study, three patients with FGC were recruited, and genomic DNA from the tumor tissue and peripheral blood was extracted for whole-exome sequencing. RESULTS: Results showed that all three patients harbored the heterozygous mutation c.1067G > A (p.Cys356Tyr) in the ANO5 gene. Furthermore, autosomal dominant mutations in ANO5 at this locus have been identified in patients with gnathodiaphyseal dysplasia (GDD) and are considered a potential causative agent, suggesting a genetic association between FGC and GDD. In addition, multifocal fibrous bone lesions with similar clinical presentations were detected, including five cases of florid cemento-osseous dysplasia, five cases of polyostotic fibrous dysplasia, and eight cases of juvenile ossifying fibromas; however, none of them harbored mutations in the ANO5 gene. CONCLUSION: Our findings indicate that FGC may be an atypical variant of GDD, providing evidence for the feasibility of ANO5 gene testing as an auxiliary diagnostic method for complex cases with multiple quadrants.

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.

High-grade osteosarcoma arising in DCIA flap reconstruction after a prior resection of maxillar cemento-ossifying fibroma: A case report.

Cemento-ossifying fibroma is a rare benign odontogenic tumour of the tooth-bearing jaws. Its concomitant occurrence with osteosarcoma, a malignant maxillofacial bone tumour, has never been described before. We present an uncommon case of a 43-year-old woman in whom a cemento-ossifying fibroma in the right maxilla was treated by resection and reconstruction using a deep circumflex iliac artery flap. During surgical prosthetic rehabilitation one-year post-operative, an osteosarcoma extending from the contralateral maxilla was coincidentally discovered in the deep circumflex iliac artery flap. The aim of this case report is to raise awareness on the extremely rare but possible simultaneous and independent occurrence of a cemento-ossifying fibroma and an osteosarcoma.

Correção de sorriso gengival em paciente com displasia cemento óssea florida / Correction of gingival smile in patient with cemento bone dysplasia florida

Com os avanços tecnológicos na odontologia o planejamento digital se tornou algo mais presente na prática clinica. O objetivo desse trabalho é relatar uma experiência vivida em clínica sobre harmonização do sorriso através de uma gengivoplastia e remodelação óssea em paciente com displasia cemento óssea florida. Paciente 42 anos, autodeclarada do sexo feminino, não relatou nenhuma alteração sistêmica, uso de medicamento ou tabagismo, entretanto relatou ter displasia cemento óssea florida com diagnóstico de periodonto saudável. Compareceu a clínica escola de uma instituição de Ensino Superior para realização de tratamento odontológico queixando-se da aparência de seus dentes. Neste caso, foi utilizado escaneamento digital, tomografia computadorizada para tecidos moles, Digital Smile Design (DSD) e Perioguide (guia cirúrgico) para a realização do procedimento de harmonização do sorriso através da cirurgia periodontal. Em suma, apesar da displasia óssea ser um fator limitante ao sucesso ideal do procedimento, o resultado final alcançado foi satisfatório sob ótica do paciente e profissional não havendo recidiva(AU)

With technological advances in dentistry, digital planning has become something more present in clinical practice. The objective of this work is to report a clinical experience on smile harmonization through gingivoplasty and bone remodeling in a patient with florid cemento-osseous dysplasia. Patient 42 years old, self-reported female, did not report any systemic changes, use of medication or smoking, however she reported having florid cemento-osseous dysplasia with a diagnosis of healthy periodontium. He attended the school clinic of a Higher Education institution for dental treatment, complaining about the appearance of his teeth. In this case, digital scanning, soft tissue computed tomography, Digital Smile Design (DSD) and Perioguide (surgical guide) were used to perform the smile harmonization procedure through periodontal surgery. In short, despite bone dysplasia being a limiting factor for the ideal success of the procedure, the final result achieved was satisfactory from the perspective of the patient and professional, with no recurrence(AU)

Immunohistochemical analysis of osteoclastic and osteoblastic activity in ossifying fibroma and juvenile ossifying fibroma: A comparative study.

Cemento-ossifying fibroma (COF) and juvenile ossifying fibroma (JOF) have been considered distinct entities within the category of fibro-osseous lesions. This study aimed to assess osteoblast and osteoclast activity in COF and JOF by investigating bone resorption markers, specifically receptor activator of nuclear factor-kB (RANK), RANK ligand (RANKL), and its inhibitor osteoprotegerin (OPG). A comparative analysis of these markers was performed on all lesions. Immunohistochemistry was employed to evaluate and quantify the expression of these biomarkers in a sample of 20 cases of cemento-ossifying fibroma (COF), 15 cases of psammomatoid juvenile ossifying fibroma (PsJOF), and 10 cases of trabecular juvenile ossifying fibroma (TrJOF). The expression of osteoprotegerin was significantly higher in cemento-ossifying fibroma (33.9±13.0) compared to trabecular juvenile ossifying fibroma (27.3±9.2) and psammatoid ossifying fibroma (25.2±14.9), with the COF showing the highest expression followed by the latter two (p=0.037). There was a higher percentage (80%) of stromal fibroblast cells that showed positive expression of RANKL in cemento-ossifying fibroma (COF) compared to psammomatoid juvenile ossifying fibroma (PsJOF) (33.3%) and trabecular juvenile ossifying fibroma (TrJOF) (30.0%) when considering a positive expression score of 3 (p=0.024). Cemento-ossifying fibroma demonstrated the highest expression of osteoprotegerin and RANKL-positive stromal fibroblast cells, followed by psammomatoid juvenile ossifying fibroma and trabecular juvenile ossifying fibroma. These findings provide valuable insights into the pathogenesis of these lesions.

Clínica, epidemiología e imagenología del fibroma osificante y la displasia fibrosa del territorio cráneo-maxilofacial: revisión narrativa / Clinical, epidemiology and imaging of ossifiying fibroma and fibrous dysplasia of the cranio-maxillofacial territory: narrative review

Introducción: El fibroma osificante (FO) y la displasia fibrosa (DF) tienen características imagenológicas e histopatológicas similares que dificultan el diagnóstico diferencial. El propósito de la presente revisión narrativa es analizar las características clínicas, epidemiológicas e imagenológicas del FO y la DF, y evaluar la relación entre las características imagenológicas y las variantes histopatológicas del FO, en reportes y series de casos publicados.Materiales y métodos:Se realizó una búsqueda de reportes y series de casos de FO y DF entre 2017-2021 en PubMed, Scopus y Web of Science. Los casos debían tener suficiente información clínica, epidemiológica, histopatológica e imagenológica.Resultados:Se incluyeron 23 artículos con 25 lesiones: 17 FO y 8 DF. El tiempo de evolución de DF es más prolongado que FO. FO se ubica con mayor tendencia en complejo osteomeatal en comparación con DF. Sólo FO presentó alteraciones en piel, radiolucidez/hipodensidad periférica, perforación ósea y rizalisis externa, además, tuvo mayor tendencia al avance a espacios anatómicos adyacentes y a generar asimetría facial y/o craneal. FO psammomatoide y FO trabecular comparten patrones imagenológicos y tienen límites definidos corticalizados. FO convencional tuvo mayor tendencia a perforar corticales. DF puede tener límites mal definidos y definidos corticalizados. El FO mostró características imagenológicas de mayor agresividad que la DF.Conclusiones:La histología e imagenología por sí solas no son decisivas en el diagnóstico de FO y DF. Es imprescindible considerar conjuntamente clínica, imagenología e histopatología, enfatizando en las características que orienten el diagnóstico diferencial.(AU)

Introduction: Ossifying fibroma (OF) and fibrous dysplasia (FD) have similar imaging and histopathological characteristics, which make differential diagnosis difficult. The aim of this narrative review was to analyze the clinical, epidemiological and imaging characteristics of OF and FD, as well as to evaluate the relationship between imaging characteristics and histopathologic variants of OF, in reports and case series published.Materials and methods:A search of reports and case series of OF and FD between 2017-2021 in PubMed, Scopus and Web of Science was performed. The cases had to have enough clinical, epidemiological, histopathological and imaging information.Results:23 articles with 25 lesions were included: 17 OF and 8 FD. FD had a longer time of evolution than OF. OF is more likely to be in osteomeatal complex compared to FD. Only OF had skin alterations, peripheral radiolucency/hypodensity, bone perforation and external root resorption, in addition, it had a greater tendency to advance to adjacent anatomical spaces and generate facial and/or cranial asymmetry. Psammomatoid OF and trabecular OF share imaging patterns and have defined and corticated margins. Conventional OF had a greater tendency to cortical perforation. DF can have ill-defined and defined and corticated margins. OF has imaging characteristics of greater aggressiveness than FD.Conclusion:Histology and imaging alone are not decisive in OF and FD diagnosis. It is essential to consider clinical, imaging and histopathological evaluations as a whole, emphasizing in the characteristics that guide the differential diagnosis.(AU)

Odontogenic tumours in a Chilean population: a retrospective study of 544 cases based on 2022 WHO classification

Background: Odontogenic tumours are infrequent lesions. Studies on the frequency of odontogenic tumours from Latin America are scarce. This work aimed to determine the relative frequency of odontogenic tumours in a Chilean population using the 2022 World Health Organization classification. Material and Methods: This is a case series retrospective study. We reviewed 35,530 samples from 1975 to 2022 from the Oral Pathology Referral Institute and the Pathological Anatomy Service, Faculty of Dentistry, University of Chile. We utilized the 2022 World Health Organization classification for histological typification. Results: According to 2022 World Health Organization classification, 544 odontogenic tumours were confirmed. The most frequent odontogenic tumours were: odontoma (n=241; 44.3%), ameloblastoma (n=109; 20.0%) and cemento-ossifying fibroma (n=71; 13.1%). Benign odontogenic tumours corresponded to 538 cases (98.9%) and malignant tumours were only six cases (1.1%). Conclusions: In our population, odontoma was the most frequent odontogenic tumour followed by ameloblastoma and cemento-ossifying fibroma. Malignant odontogenic tumours were very rare. The results of this study are similar to reports from America, but there are some differences concerning the data from Africa and Asia. (AU)

Odontogenic tumours in a Chilean population: a retrospective study of 544 cases based on 2022 WHO classification.

BACKGROUND: Odontogenic tumours are infrequent lesions. Studies on the frequency of odontogenic tumours from Latin America are scarce. This work aimed to determine the relative frequency of odontogenic tumours in a Chilean population using the 2022 World Health Organization classification. MATERIAL AND METHODS: This is a case series retrospective study. We reviewed 35,530 samples from 1975 to 2022 from the Oral Pathology Referral Institute and the Pathological Anatomy Service, Faculty of Dentistry, University of Chile. We utilized the 2022 World Health Organization classification for histological typification. RESULTS: According to 2022 World Health Organization classification, 544 odontogenic tumours were confirmed. The most frequent odontogenic tumours were: odontoma (n=241; 44.3%), ameloblastoma (n=109; 20.0%) and cemento-ossifying fibroma (n=71; 13.1%). Benign odontogenic tumours corresponded to 538 cases (98.9%) and malignant tumours were only six cases (1.1%). CONCLUSIONS: In our population, odontoma was the most frequent odontogenic tumour followed by ameloblastoma and cemento-ossifying fibroma. Malignant odontogenic tumours were very rare. The results of this study are similar to reports from America, but there are some differences concerning the data from Africa and Asia.

[Clinical and pathological features and differential diagnosis of fibro-osseous tumors and dysplasias].

Fibro-osseous lesions is a class of diseases with obvious similarities in clinical manifestations and pathological features, which has been attracting the attention of clinicians and pathologists. The latest WHO 2022 Classification (5th edition) included six of these diseases (cemento-osseous dysplasia, segmental odontomaxillary dysplasia, fibrous dysplasia, juvenile trabecular ossifying fibroma, psammomatoid ossifying fibroma and familial gigantiform cementoma) in the " fibro-osseous tumours and dysplasias ", and put forward new ideas on the diagnosis and treatment of these diseases. According to the latest WHO 2022 Classification (5th edition), the clinical and pathological features, diagnosis and differential diagnosis of these six diseases were described.

The True History of Cementoblastoma.

REVIEW OF THE LITERATURE: Cementoblastoma (CB) is unique among odontogenic tumors because its gross pathological anatomy is pathognomonic in most cases, i.e., a rounded calcified growth that is fused to the root of a tooth and completely encapsulated by fibrous tissue. The resulting radiographic appearance is a well-defined, globular mixed radiopaque/lucent or completely radiopaque mass obliterating some details of the root, with a thin radiolucent zone surrounding the central opacity. Although hundreds of publications have covered the clinicopathologic features of CB, almost nothing is known about its true history. Also it seems there is little understanding about how the term "CB" was originally introduced as a pathologic entity. This report covers some overlooked papers on CB dating back to the 19th century, including the first complete description in 1888 and the first radiographic presentation in 1906.

A Clinical, Radiological and Histopathological Review of 74 Ossifying Fibromas.

BACKGROUND: Ossifying fibroma (OF) is a fibro-osseous lesion of the jaws and craniofacial bones. Accurate diagnosis can be challenging due to significant overlap of clinicopathological features. This study aimed to evaluate the clinical, radiological and histological features that can aid in diagnosis and identify characteristics that allow categorisation into the three subtypes: juvenile trabecular, psammomatoid and cemento-ossifying OF. METHODS: A total of 74 cases of OF were systematically reviewed for their principle features. Of these, 46 cases were evaluated for their radiographic features including size, location and relationship to the teeth. Histological assessment and stereological point counting were performed in 69 cases to assess the pattern, type and proportion of calcification, the nature of the stroma, the border of the lesion and the presence of secondary changes. Fisher's exact test and Chi-squared tests were used to determine associations between clinicopathological parameters and maxillary, mandibular, odontogenic, non-odontogenic and psammomatoid or trabecular lesions. RESULTS: OF showed a female predilection (F: M; 2:1) and a slight bimodal age distribution with peaks in the second (23%) and fourth decades (27%) (Mean age: 32.4 years). 83% of cases presented as an intra-oral swelling, with the mandible being the most common site (73%). Histologically, a range of morphological patterns were seen, with 50% of cases showing mixed trabecular and psammomatoid features. However, there were no significant differences between the variants of OF in terms of age, gender or histological features. CONCLUSION: Histological features of OF cannot be used to differentiate between the subtypes.

An alternative surgical approach to cemento-ossifying fibroma of the maxilla

Objective@#To report an alternative combined gingivobuccal and endoscopic endonasal approach to treating ossifying fibroma of the left maxilla.@*Methods@#Design: Case Report. Setting: Tertiary Private University Hospital. Patient: A 19-year-old girl with a progressively enlarging left cheek mass for 3 years.@*Results@#Our patient underwent left medial maxillectomy via a combined gingivobuccal and endoscopic endonasal approach with complete resection and significant improvement of symptoms, with good functional outcome.@*Conclusion@#Large ossifying fibromas of the maxilla can be completely and successfully excised via a combined gingivobuccal and endoscopic endonasal approach.