Displasia Cemento Ósea Florida: Reporte de un caso / Florid Cemento-Osseous Dysplasia: A case report

La Displasia Cemento Ósea Florida corresponde a un tipo de lesión fibro ósea caracterizada por presentar un hueso trabecular con apariencia de tejido conectivo fibroso con áreas similares al cemento radicular. Afecta principalmente a mandíbula, a mujeres y a personas de etnia africana entre la cuarta a la quinta década. Posee crecimiento limitado, presentación de forma simétrica, bilateral y capacidad de afectar de uno a más cuadrantes. Tiene tres etapas de desarrollo, que se presentan con aspecto radiográfico diferente. En la primera etapa se aprecia un área radiolúcida, en la segunda etapa se visualizan radiopacidades circunscritas al área radiolúcida; y en la tercera etapa se observa una clara radiopacidad alrededor de la lesión. Para su correcto diagnóstico se necesita una cuidadosa correlación de los hallazgos clínicos, imagenológicos, de laboratorio e histopatológicos. Se presenta el caso de una paciente de género femenino de 70 años diagnosticada con Displasia Cemento Ósea Florida.

Florid Cemento-Osseous Dysplasia is a type of fibro-osseous lesion characterized by a trabecular bone with the appearance of fibrous connective tissue with areas similar to root cement. It mainly affects the jaw, women and people of African ethnicity between the fourth and fifth decades. It has limited growth, a symmetrical, bilateral presentation and the ability to affect one or more quadrants. It has three stages of development, with different radiographic appearance. The first stage shows a radiolucent area, the second stage shows radiopacities circumscribed to the radiolucent area; and in the third stage a clear radiopacity is observed around the lesion. For its correct diagnosis, a careful correlation of clinical, imaging, laboratory and histopathological findings is needed. The case of a 70-year-old female patient diagnosed with Florid Cemento-Osseous Dysplasia is presented.

Lesión Fibro-ósea de Mandíbula: Un Hallazgo Radiográfico / Fibro-osseous Lesion of the Mandible: A Radiographic Finding

Las lesiones fibro-óseas son consideradas benignas y componen un grupo de patologías de desórdenes que se caracterizan por el reemplazo de un hueso normal por un tejido compuesto de fibras colágenas, fibroblastos y tejido mineralizado. Presentamos un hallazgo radiográfico obtenido de un paciente de sexo masculino de 41 años de edad que asiste a un centro de radiología para realizarse una radiografía panorámica, el examen revela un interesante hallazgo radiográfico en la hemi mandíbula izquierda, donde se observa una lesión fibro-ósea con expansión ósea a nivel de reborde marginal y cortical basal mandibular, desplazamiento de canal mandibular, desplazamiento dentario, compromiso de cortical alveolar y rizálisis en diferentes niveles en los dientes adyacentes a la lesión. De acuerdo a los antecedentes anteriores se establece una hipótesis diagnóstica de Fibroma Osificante de larga data debido a su radiopacidad. La Organización Mundial de la Salud lo clasifica como una neoplasia ósea benigna con afección al esqueleto craneofacial, de mayor incidencia en mandíbula, se presenta generalmente entre la 3º y 4º década de vida. Concluimos que las lesiones fibro-óseas pueden ser detectadas como un hallazgo radiográfico, esto es relevante para un tratamiento precoz, sin embargo, el diagnóstico debe realizarse complementando los antecedentes clínicos e histopatológicos de la lesión, poniendo especial atención en el diagnóstico diferencial.

Fibro-osseous lesions are considered benign and make up a group of disorder pathologies that are characterized by the replacement of normal bone by tissue composed of collagen fibers, fibroblasts, and mineralized tissue. We present a radiographic finding obtained from a 41-year-old male patient who attended a radiology center for a panoramic radiograph. The examination revealed an interesting radiographic finding in the left hemi-mandible, where a fibro-osseous lesion was observed. with bone expansion at the level of the marginal ridge and basal mandibular cortex, displacement of the mandibular canal, dental displacement, compromise of the alveolar cortex and rizalysis at different levels in the teeth adjacent to the lesion. According to the previous antecedents, a long-standing diagnostic hypothesis of Ossifying Fibroma is established due to its radiopacity. The World Health Organization classifies it as a benign bone neoplasm affecting the craniofacial skeleton, with the highest incidence in the jaw, generally presenting between the 3rd and 4th decade of life. We conclude that fibro-osseous lesions can be detected as a radiographic finding, this is relevant for early treatment, however the diagnosis must be made by complementing the clinical and histopathological history of the lesion, paying special attention to the differential diagnosis.

Quantitative proteomic study reveals differential expression of matricellular proteins between fibrous dysplasia and cemento-ossifying fibroma pathogenesis.

BACKGROUND: Fibrous dysplasia (FD) and cemento-ossifying fibroma (COF) are the most common gnathic fibro-osseous lesions. These diseases exhibit remarkable overlap of several clinicopathological aspects, and differential diagnosis depends on the combination of histopathological, radiographic, and clinical aspects. Their molecular landscape remains poorly characterized, and herein, we assessed their proteomic and phosphoproteomic profiles. METHODS: The quantitative differences in protein profile of FD and COF were assessed by proteomic and phosphoproteomic analyses of formalin-fixed paraffin-embedded tissue samples. Pathway enrichment analyses with differentially regulated proteins were performed. RESULTS: FD and COF exhibited differential regulation of pathways related to extracellular matrix organization, cell adhesion, and platelet and erythrocytes activities. Additionally, these lesions demonstrated distinct abundance of proteins involved in osteoblastic differentiation and tumorigenesis and differential abundance of phosphorylation of Ser61 of Yes-associated protein 1 (YAP1). CONCLUSIONS: In summary, despite the morphological similarity between these diseases, our results demonstrated that COF and DF present numerous quantitative differences in their proteomic profiles. These findings suggest that these fibro-osseous lesions trigger distinct molecular mechanisms during their pathogenesis. Moreover, some proteins identified in our analysis could serve as potential biomarkers for differential diagnosis of these diseases after further validation.

Fibroma ossificante trabecular juvenil: um relato de caso / Juvenile ossifying fibroma: a case report

Objetivo: o fibroma ossificante trabecular juvenil (FOJT) é uma lesão fibro-óssea incomum, benigna, porém de comportamento agressivo. Ocorre, frequentemente, em crianças e adolescentes, com maior acometimento em maxila e mandíbula. O presente relato aborda os aspectos clínicos, imaginológicos, histopatológicos, imuno-histoquímicos e o tratamento de um caso de FOTJ. Além de discutir suas similaridades com o osteossarcoma de baixo grau e as alternativas para o seu diagnóstico. Relato de caso: paciente do sexo masculino, com 12 anos de idade, apresentando aumento de volume em mandíbula no lado esquerdo, perda de função mastigatória e de fala, com tempo de evolução de seis meses. Radiograficamente, a lesão apresentava aspecto unilocular, com limites definidos e de padrão misto. O tratamento consistiu em cirurgia de enucleação total da lesão, seguida de curetagem sob anestesia geral. A histopatologia e a ausência de marcação para MDM2 e CDK4 na imuno-histoquímica confirmaram o diagnóstico de FOTJ e excluíram o de osteossarcoma de baixo grau. No controle pós-operatório de 20 dias, o paciente relatou melhora da parestesia, da função mastigatória e da fala e ausência de dor. Não se observou recidiva no acompanhamento de 1 ano após a cirurgia. Considerações finais: a similaridade dos aspectos clínico, imaginológico e microscópico entre FOJT e osteossarcoma de baixo grau pressupõe o estudo imuno-histoquímico com os arcadores MDM2 e CDK4, minimizando equívocos no diagnóstico e orientando de forma mais eficiente o plano de tratamento adotado.(AU)

Objective: juvenile Trabecular Ossifying Fibromas (JTOF) are uncommon benign fibro-osseous lesions with a local aggressive behavior. They frequently occur in children and adolescents involving the jaw bones. This report addresses the clinical, imaging, histopathological, and immunohistochemical aspects and the treatment of a case of JTOF. In addition, it discusses similarities between JTOF and low-grade osteosarcomas and alternatives for their diagnosis. Case report: male patient, 12 years old, with a tumor in his left mandible, loss of masticatory function and speech, with a 6-month time course. Radiographically, the lesion had an unilocular aspect, with defined borders and a mixed image pattern. Treatment consisted surgical enucleation, followed by bone curettage, under general anesthesia. Histopathology presented fibrous stroma interspaced with bone trabeculae without signs of cellular malignancy. MDM2 and CDK4 markers in immunohistochemistry, confirmed the diagnosis of JTOF and excluded low-grade osteosarcoma. At the 20-day postoperative follow-up, the patient reported improvement in paresthesia, masticatory function and speech, and the absence of pain. Evaluation 1 year after surgery showed no signs of local recurrence. Final considerations: the similarity of clinical, imaging, and microscopic aspects between JTOF and low-grade osteosarcoma prompts an immunohistochemical study including MDM2 and CDK4 markers in order to improve final diagnosis and guide the treatment plan more efficiently.(AU)

A distinct fibro-osseous lesion of the jaws affecting the maxilla.

The differential diagnosis of fibro-osseous lesions (FOL) presents oral clinician stomatologists with a challenging task because of their similar clinical, radiographic and histopathological behavior that will also hinder the therapeutic planning. Presentation of case to describe the clinical, radiographic and histopathological features of an unusual case of FOL presented by the patient, a Black woman, and the results obtained with the conservative surgical treatment and 3-month follow-up. The presence of cementum in the histopathological analysis of cemento-osseous dysplasias, according to the current literature, is an important factor for the diagnosis of this lesion. Considering the characteristics presented by this particular case, we could suggest another entity that could be named a benign cemento-osseous lesion with adult onset. Some occurrences, as in the present case, do not fit properly into the current World Health Organization classification, thereby generating some doubts concerning the correct management of these patients. Conservative surgical treatment is the first choice in the management of these lesions due to their self-limiting nature, which show a behavior of clinical involution.

Can non-collagenous proteins be employed for the differential diagnosis among fibrous dysplasia, cemento-osseous dysplasia and cemento-ossifying fibroma?

Differential diagnosis among fibrous dysplasias, cemento-ossifying fibromas and cemento-osseous dysplasias is difficult, since there is considerable overlap of histologic features, but also extremely important, since they differ greatly in etiology, clinical behaviour, prognosis and terapeuthic approach. There is no data about the use of immunohistochemistry, a viable and accessible technique, for this purpose. The objective of this study was to investigate, comparatively, the immunohistochemical expression of major non-collagenous proteins (osteonectin [ON], osteopontin [OP], bone sialoprotein [BSP] and osteocalcin [OC]) of mineralized tissue extracellular matrix in 22 cases of fibrous dysplasias, 16 of cemento-ossifying fibromas and 16 of cemento-osseous dysplasias. ON maintained the same expression profile in all cases; the staining for OP was negative in fusiform cells producing cementoid globules and weak, as well as heterogeneous, in high mineralized matrixes; there was negativity for BSP in cementoid globules and in the fusiform cells that produce them, differently from the strong positive expression found in the majority of bone trabeculae and their peripheral cuboidal osteoblasts; and finally, the immuno-reactivity for OC was weak, except in cuboidal osteoblasts and osteocytes. We can conclude that the nature of mineralized structure and the cellular phenotype are much more responsible for variability in immunohistochemical profile than the type of lesion (fibrous dysplasias, cemento-ossifying fibromas and cemento-osseous dysplasias) which makes difficult, at least for a while, the use of these proteins with diagnosis purpose.

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.

Fibrous dysplasia with secondary aneurysmal bone cyst-a rare case report and literature review.

INTRODUCTION: Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon non-neoplastic intraosseous lesions, and the occurrence of concomitant FD and ABC is extremely rare. CASE REPORT: An 11-year-old boy presented with gradual progressive enlargement of his right zygomatic bone over 4 years prior to presentation. Computed tomography revealed a lesion with a central bony area showing a ground-glass appearance surrounded by a well-defined expansile lesion with internal septations. An incisional biopsy was performed and suggested a fibro-osseous lesion. Resection was performed, followed by immediate reconstruction using autogenous bone graft from the iliac crest. Histopathological examination revealed irregularly shaped trabeculae comprising immature woven bone in a fibroblastic cell-rich stroma. Blood-filled sinusoidal spaces lined by fibrous septa containing scattered multinucleated giant cells were observed peripherally. These findings were compatible with concomitant FD and ABC. The patient was disease-free at the time of his 10-month follow-up. CONCLUSIONS: Based on our literature search, this report is the first to describe concomitant monostotic FD and ABC in the zygomatic bone. Accurate diagnosis requires careful investigation and examination of clinical, radiographical, and histopathological features. The treatment of choice should provide an esthetic and functional improvement in the patient.

Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical, radiological, and histopathological features.

Fibrous dysplasia is a non-neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune-Albright syndrome, Jeffe-Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.

Immunolocalization of bmp2/4, tgfβ-1 and osteonectin in fibro-osseous lesions of the jaws / Imunolocalização de bmp2/4, tgfß-1 e osteonectina em lesões-fibro-óssea benignas

Objetivo: as lesões fibro-ósseas benignas (LFOB) correspondem a um grupo diverso de patologias caracterizadas pela substituição do tecido ósseo por tecido conjuntivo e matriz extracelular mineralizada. Pouco se conhece a respeito da etiologia desse grupo de lesões. Propomo-nos a analisar por meio da técnica imunohistoquímica a expressão de 3 moléculas (osteonectina, TGFβ-1 e BMP 2/4) envolvidas no metabolismo ósseo. Métodos: Trinta e dois casos diagnosticados como osso normal (ON,8), displasia fibrosa (DF,8), displasia cemento-óssea (DCO,8) e fibroma cemento-ossificante (FCO,8) foram selecionados. Resultados: A osteonectina e a BMP2/4 foram positivas em todos os casos. O TGFβ-1 revelou positividade em 1 caso de DCO e FCO. Conclusão: Os achados imunohistoquímicos sugerem que as LFOB tem processos diferentes de produção de tecido ósseo.

Background: Benign fibro-osseous lesions (BFOL) comprise a diverse group of pathologies characterized by the replacement of normal bone by fibrous tissue and a mineralized product. Little is known about the biology of this group of lesions. We have analyzed the immunohistochemical expression of three molecules involved in bone metabolism, namely osteonectin, TGF-b1, and BMP2/4. Methods: Thirty-two cases diagnosed as normal jaw bone (NJB, 8 cases), fibrous dysplasia (FD, 8 cases), cemento-osseous dysplasia (COD, 8 cases), and cemento-ossifying fibroma (COF, 8 cases) were selected. Results: Osteonectin and BMP2/4 antibodies were positive in all cases. TGFb-1 labeling was seen in one case of COD and COF. Conclusion: The immunohistochemistry findings suggest that BFOL have different processes of osseous tissue production.