A Case Series and Literature Review of Craniofacial Fibrous Dysplasia.

Craniofacial fibro-osseous lesions (CFOLs) are a diverse group of relatively rare entities whose etiology ranges from reactive to dysplastic with a potential for malignant transformation. It is distinguished by the replacement of bone with fibrous tissue, that subsequently develops different degrees of calcification. Fibrous dysplasia (FD) is a component of the fibro-osseous lesion spectrum. The clinical spectrum of FD is wide, ranging from minor monostotic lesions affecting a single bone to devastating polyostotic disease involving the entire skeleton. FD produces asymmetry, which impairs face aesthetics. FD leads to bone differentiation, disintegration, and disorganization. It depicts a cellular collagenous stroma lacking mitotic figures and pleomorphism. Blood capillaries are evenly distributed, as are elongated trabeculae of woven or lamellar bone with uneven curves (often referred to as the Chinese letters pattern). Three types of FD patterns can be identified by computed tomography (CT) imaging: a cystic pattern, a homogeneously dense pattern, and a ground-glass pattern. The cornerstone of treatment is surgery, although the method varies depending on the location, size, and symptoms of the lesion. As an alternative to surgery, the use of bisphosphonates to reduce osteoclastic activity is under consideration. In this case series, we present three cases of FD involving the maxilla and mandible. We aim to correlate the clinical presentation, histological features, and radiographic findings, to promote early diagnosis, treatment, and better prognosis of the patient.

Exploring a rare case of juvenile psammomatoid ossifying fibroma in the ethmoid: a case study and review.

Juvenile ossifying fibroma (JOF) and its variants, including juvenile psammomatoid ossifying fibroma (JPOF), represent rare yet clinically significant benign fibro-osseous lesions that primarily occur in children and young adolescents. They can be found in diverse anatomical sites such as the jaw, nasal cavity, paranasal sinuses, and orbit. JOF exhibits an aggressive nature, necessitating early radiological detection and surgical intervention. Similarly, JPOF, with a locally malignant potential, requires surgical removal, typically conducted through endoscopic approaches. We report a case of a 5-year-old girl with JPOF arising in the ethmoid, revealed by recurrent epistaxis and proptosis. The text emphasizes the importance of early diagnosis through histopathology as a diagnostic tool and underscores the need for appropriate management.

Lesions Misdiagnosed as Endodontic Pathosis: A Narrative Review.

In daily practice, clinicians come across certain radiographic abnormalities which may or may not be asymptomatic. This abstract discusses radiographic abnormalities encountered by clinicians in daily practice, some of which resemble endodontic lesions. Prompt attention is crucial as these lesions can be benign or malignant. The article emphasizes the importance of differential diagnosis for accurate identification of periapical pathosis.

Cherubism Unmasked: A Case Report of Clinical and Histopathological Presentation.

Cherubism, an uncommon genetic disorder, manifests as painless swelling in both jaws. A 20-year-old male presented with symmetrical swelling in both the mandible and maxilla. The jaws exhibited bilateral expansion, typical of this condition. Dentofacial abnormalities associated with cherubism stem from mutations in the SH3BP2 gene, which plays a crucial role in regulating osteoblasts and osteoclasts. In summary, cherubism is a genetic disorder characterized by non-cancerous jaw bone lesions. Surgical intervention may be necessary for functional or aesthetic concerns.

Displasia Cemento Ósea Florida: Reporte de un caso

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.

Juvenile trabecular ossifying fibroma: A case of extensive lesion of the maxilla.

INTRODUCTION AND IMPORTANCE: Juvenile trabecular ossifying fibroma is a rare benign tumor of childhood affecting the facial bones rarely described in literature. Its aggressive growth and high tendency of recurrence make it a real challenge for diagnosis and care. CASE PRESENTATION: This article presents a case of an extensive juvenile trabecular ossifying fibroma of the maxilla in a 14-year-old boy, which required surgical intervention with immediate bone reconstruction using an autogenous graft (iliac crest). No recurrence has been found after two and a half years of follow-up. CLINICAL DISCUSSION: Common symptoms of juvenile trabecular ossifying fibroma include painless swelling, facial asymmetry, jaw deformity and teeth displacement. Differential diagnosis should consider other forms like psammomatoid ossifying fibroma, osteofibrous dysplasia, fibrous dysplasia, ameloblastoma, odontoma, or even poorly differentiated carcinoma. CONCLUSION: Currently, there is no recommendation or consensus for the treatment of juvenile trabecular ossifying fibroma. The objective of treatment is also to preserve growth and development and conserve the nervous structure and the function, as mastication, vision. Early diagnosis and appropriate care are essential to prevent morphological and functional defects in young patients. Regular and long-term follow-up is essential due to the high recurrence rate.

Computed tomographic characteristics of frontal sinus ossifying fibroma in a dog.

A 10-year-old female spayed Kelpie cross was presented to The Austin Vet Specialists for further investigation of a mineralized, lobulated frontal sinus mass that had previously been detected radiographically. Computed tomography (CT) revealed a large, expansile, well-defined, heterogeneously mineral attenuating mass invading both frontal sinuses. The mass was surgically debulked via a frontal sinusotomy approach. Histopathology was consistent with ossifying fibroma. This  is the first published report to describe frontal sinus ossifying fibroma in a dog, and the second to describe CT features of ossifying fibroma involving the cranium in a dog.

Fibrous Dysplasia of the Jaw: Advances in Imaging and Treatment.

A total of 7% of all benign bone lesions are diagnosed as fibrous dysplasia (FD). The symptoms of FD of the jaw range from asymptomatic to dental anomalies, pain and facial asymmetry. Due to its resemblance to other fibro-osseous bone lesions, misdiagnosis often occurs and can lead to inadequate treatment. Particularly in the jaw, this lesion does not become quiescent during puberty, making fundamental knowledge about the diagnosis and treatment of FD crucial. Mutational analysis and nonsurgical approaches offer new diagnostic and therapeutic options. In this review, we examine the advances and the difficulties of the diagnosis and the various treatment modalities of FD of the jaw in order to capture the current scientific knowledge on this bone disease.

A Rare Case of Cemento-Ossifying Fibroma: A Case Report.

Benign fibro-osseous lesions are a group of pathological conditions characterized by the replacement of normal bone with cellular fibrous connective tissue that undergoes mineralization. The most common types of benign fibro-osseous lesions include fibrous dysplasia, ossifying fibroma, and osseous dysplasia. However, diagnosing these lesions can be challenging due to their overlapping clinical, radiological, and histological features, which can cause a diagnostic dilemma for surgeons, radiologists, and pathologists. One rare type of benign fibro-osseous lesion is the cemento-ossifying fibroma (COF), which is a definitive form of a benign fibro-osseous tumor that affects the craniofacial region, particularly the jaws (70%). Here, we present a case of COF in a 61-year-old female patient in the maxillary anterior region. Due to a clear distinction between the lesion and healthy bone, the lesion was treated with conservative surgical excision followed by curettage and primary closure. However, differential diagnosis of COF can be highly challenging for clinicians due to its overlapping features with other fibro-osseous lesions like Paget's disease and fibrous dysplasia. Ossifying fibroma and fibrous dysplasia often present a histopathological, clinical, and radiological overlap. The post-operative follow-up after eight months was unpredictable, with a radiological picture showing the increased thickness of the frontal bone, parietal bone, and maxilla with obliteration of marrow spaces, alteration of the trabecular pattern with a cotton wool/ground glass appearance, and reduced maxillary sinus space. Proper evaluation and diagnosis of fibro-osseous lesions are necessary before arriving at a final conclusion. Cemento-ossifying fibroma in the maxillofacial skeleton is uncommon, and after eight months, the recurrence rate is rare. This case highlights the importance of considering COF as a differential diagnosis for fibro-osseous lesions in the maxillofacial region and the necessity for proper evaluation and diagnosis to determine the appropriate treatment plan and prognosis. In summary, the diagnosis of benign fibro-osseous lesions can be challenging due to their overlapping features, but early diagnosis and proper evaluation are essential for successful treatment outcomes. COF is a rare type of benign fibro-osseous lesion where other fibro-osseous lesions in the maxillofacial region should be considered as a differential diagnosis, and the necessary steps should be taken to confirm the diagnosis before arriving at a final conclusion.

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.

Psammomatoid Juvenile Ossifying Fibroma of the Maxilla Misdiagnosed as Fibrous Dysplasia: A Clinicopathologic Case Report.

Juvenile ossifying fibroma (JOF) is a variant of the ossifying fibroma and includes two histopathological subtypes: trabecular and psammomatoid. Psammomatoid JOF (PJOF) in craniofacial structures should be distinguished from other fibro-osseous lesions, such as fibrous dysplasia (FD), considering the difference in the treatment protocols. Here, we present a rare case of PJOF that was initially misdiagnosed as a case of FD and emphasize the importance of considering JOF in the differential diagnosis of patients with craniofacial fibro-osseous lesions. A 4-year-old boy demonstrated progressive enlargement of the zygomaticomaxillary area on his left side for the last 6 months. The patient was diagnosed as a case of FD based on the clinical features and radiographic findings, and was operated considering the rapid progression. To achieve facial symmetry, contouring of the zygomatic bone and arch was performed. However, the patient demonstrated rapid enlargement at the 3-month postoperative follow-up. The decision was made to surgically remove the tumor due to visual field impairment. Intraoperatively, a rubbery mass, which was separated from the surrounding cortical bone, was identified and excised. The lesion was confirmed as PJOF by histopathological examination. The possibility of PJOF should not be ruled out in the differential diagnosis of patients with fibrous-osseous lesions. In the event of suspected PJOF, accurate diagnosis should be made through definitive biopsy.

Ossifying Fibroma in the Maxilla and Mandible: A Case Report With a Brief Literature Review.

Fibro-osseous lesions in the jaw bones include fibrous dysplasia, ossifying fibroma (OF), cemento-ossifying fibroma, florid osseous dysplasia, and focal osseous dysplasia. OF is the most common fibro-osseous tumor that presents as a slow-growing well-encapsulated benign neoplasm composed of varying amounts of bone or cement-like tissue in a fibrous stroma well-demarcated from the adjacent normal bone. OF is most common in the jaw bones, with a predilection for the mandible. OF usually occur as solitary lesions and rarely as multiple lesions in a patient. We present clinical and radiologic features, histopathology, and surgical management of a rare case with large synchronous OFs in the mandible and maxilla and a brief review of the literature.

Juvenile psammomatoid ossifying fibroma with aneurysmal bone cyst in the mandible: A report of a rare case.

Aneurysmal bone cysts (ABCs) are rare benign lesions seen as locally destructive, rapidly expansile, and mostly affecting the long bones and vertebrae. The association of ABCs with juvenile psammomatoid ossifying fibroma (PsJOF) is predominantly seen in the extra gnathic region, and it is extremely rare with only a few cases reported so far in the mandible. Here, we report one such case of a hybrid lesion in a 30-year-old male, who presented with a solitary swelling of the right mandible showing partial obliteration of lingual vestibular sulcus, which was histologically confirmed as PsJOF as a preexisting lesion, transforming into an ABC. Such hybrid lesions are usually misdiagnosed and have been sparsely reported in the literature.

Focal Cemento Osseous Dysplasia: A Case Report.

Focal cemento osseous dysplasia (FCOD) is a subgroup of benign fibro osseous lesions, which occur in a single site of tooth-bearing areas of jaws. It is usually asymptomatic and noticed accidentally through routine radiological exams. There is often no need for treatment of such lesions. This case was a 28-year-old male patient with a mixed radiolucent-radiopaque lesion in posterior part of his mandible. Based on radiographic examination, cemento ossifying fibroma (COF) was the first differential diagnosis. After the surgical removal of the lesion, histopathologic evaluation was made and the case was diagnosed as FCOD. Fortunately, after a few months of operation, there was no complication and complete bone formation was occurred.

An Uncommon Presentation of Ossifying Fibroma in the Maxilla.

Ossifying fibromas are rare, benign, nonaggressive fibro-osseous lesions that manifest in the craniofacial region. Ossifying fibromas are benign tumors of bone, commonly involving the posterior dentate mandible in middle-aged individuals with a female predilection. Clinical manifestations are an asymptomatic expansion of the dentate mandible, with infrequent maxillary lesions. Benign fibro-osseous lesions of the maxilla exhibit similar histopathological findings. Cone-beam computed tomography scan plays an important role in diagnosing and understanding the invasiveness of this lesion. This case report describes an uncommon presentation of ossifying fibroma in the posterior maxilla in an adult male patient. Treatment consisted of surgical excision, and wound healing was uneventful during the one-year postoperative follow-up period.

Expression of Beta-Catenin, Cadherins and P-Runx2 in Fibro-Osseous Lesions of the Jaw: Tissue Microarray Study.

Fibrous dysplasia (FD) and hyperparathyroidism-jaw tumor syndrome (HPT-JT) are well-characterized benign bone fibro-osseous lesions. The intracellular mechanism leading to excessive deposition of fibrous tissue and alteration of differentiation processes leading to osteomalacia have not yet been fully clarified. Tissue Microarray (TMA)-based immunohistochemical expression of ß-catenin, CK-AE1/AE3, Ki-67, cadherins and P-Runx2 were analyzed in archival samples from nine patients affected by FD and HPT-JT and in seven controls, with the aim of elucidating the contribution of these molecules (ß-catenin, cadherins and P-Runx2) in the osteoblast differentiation pathway. ß-catenin was strongly upregulated in FD, showing a hyper-cellulated pattern, while it was faintly expressed in bone tumors associated with HPT-JT. Furthermore, the loss of expression of OB-cadherin in osteoblast lineage in FD was accompanied by N-cadherin and P-cadherin upregulation (p < 0.05), while E-cadherin showed a minor role in these pathological processes. P-Runx2 showed over-expression in six out of eight cases of FD and stained moderately positive in the rimming lining osteoblasts in HPT-JT syndrome. ß-catenin plays a central role in fibrous tissue proliferation and accompanies the lack of differentiation of osteoblast precursors in mature osteoblasts in FD. The study showed that the combined evaluation of the histological characteristics and the histochemical and immunohistochemical profile of key molecules involved in osteoblast differentiation are useful in the diagnosis, classification and therapeutic management of fibrous-osseous lesions.

Clinical, Radiological, and Pathological Diagnosis of Fibro-Osseous Lesions of the Oral and Maxillofacial Region: A Retrospective Study.

BACKGROUND: Fibro-osseous lesions (FOL) of the jaw represent a rare, benign group of lesions that share similar clinical, radiological, and histopathological features and are characterized by progressive, variable replacement of healthy bone tissue by fibrous connective tissue. METHODS: This retrospective study aimed to evaluate the incidence of fibro-osseous lesions and to reassess the efficacy of case-specific treatment management from a clinical, radiological, and histopathological perspective based on 14 years of data. RESULTS: Forty-four patients with a radiological and/or histopathological diagnosis of benign FOLs were identified and re-evaluated. Cemento-osseous dysplasia was the most common group of FOLs present in our patient cohort (45%), followed by ossifying fibroma (39%) and fibrous dysplasia (16%). The diagnostic imaging technique of choice was CBCT (68%), followed by PAN (18%), with most patients (95 %) additionally undergoing biopsy. The mean age of the patients at the time of diagnosis was 40.54 ± 13.7 years, with most lesions being located in the mandible (86%), with females being predominantly affected (73%). CONCLUSION: An interdisciplinary approach that analyzes all case-specific factors, including demographic data, medical history, intraoperative findings, and, most importantly, histopathological and radiological features, is essential for an accurate diagnosis and key to avoiding inappropriate treatment.

Role of MDM2, CDK4, BCL2, Parafibromin and Galectin 1 in Differentiating Osteosarcoma from its Benign Fibro-osseous Lesions.

Benign fibro-osseous lesions (BFOLs) are a diverse group of lesions showing considerable degree of overlap with low grade osteosarcoma (LGOS). Further, de-differentiated osteosarcoma (DOS) is usually indistinguishable from conventional high-grade OS (COS) if LGOS foci are not identified. Thus, there is a need for adjunctive immunohistochemical markers to differentiate OS from benign FOLs as well as DOS from COS. This study evaluated the role of immunohistochemical expression of MDM2, CDK4, parafibromin, BCL-2 and Galectin-1 (Gal-1) in accurate characterization of benign FOLs and in differentiating them from OS. From our archives, we retrieved 101 tissue samples which were diagnosed as osteosarcoma (OS) /ossifying fibroma (OF) / fibrous dysplasia (FD) or fibrous hyperplasia (FH) and examined their immunohistochemical staining pattern with the aforementioned antibodies. MDM2 showed 100% specificity for diagnosing OS. CDK4 and Gal-1 showed linear increase in immunoexpression from benign BFOLs to OS. BCL-2 showed equivocal immunopositivity in OF and OS, but the positivity was higher than that observed in FD. The highest immunoexpression for parafibromin was seen in FD followed by OF and OS cases. Thus, MDM2 is most specific, and Gal-1 is most sensitive of all the markers studied in differentiating OS from benign mimics. Combination of these two markers can be used as an adjunct to conventional imaging and microscopy in accurate characterization of these lesions. Further MDM2 overexpression can differentiate DOS and COS.

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.