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.

When molecular outsmarts morphology: Malignant ossifying fibromyxoid tumors masquerading as osteosarcomas, including a novel CREBZF::PHF1 fusion.

We present two cases of malignant ossifying fibromyxoid tumor (OFMT) which eluded diagnosis due to compelling clinicopathologic mimicry, compounded by similarly elusive underlying molecular drivers. The first is of a clavicle mass in a 69 year-old female, which histologically showed an infiltrative nested and trabeculated proliferation of monomorphic cells giving rise to scattered spicules of immature woven bone. Excepting SATB2 positivity, the lesion showed an inconclusive immunoprofile which along with negative PHF1 FISH led to an initial diagnosis of high-grade osteosarcoma. Next generation sequencing (NGS) revealed a particularly rare CREBBP::BCORL1 fusion. The second illustrates the peculiar presentation of a dural-based mass in a 52 year-old female who presented with neurologic dyscrasias. Sections showed a sheeted monotonous proliferation of ovoid to spindle cells, but in contrast to Case #1, the tumor contained an exuberance of reticular osteoid and woven bone deposition mimicking malignant osteogenic differentiation. NGS showed a novel CREBZF::PHF1 fusion. Both tumors recurred locally less than 1 year post-operatively. As such we reiterate that careful morphologic examination is axiomatic to any diagnosis in our discipline, but this paradigm must shift to recognize that molecular diagnostics can provide closure where traditional tools have notable limitations.

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.

[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.

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.

Tumor-induced Osteomalacia in a Boy with Maxillary Ossifying Fibroma

Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic disorder of hypophosphatemia associated with elevated tumor-produced fibroblast growth factor 23 (FGF23). Maxillofacial tumors are rarely involved in TIO, especially maxillary TIO in children. We present a 14-year-old boy with osteomalacia and high serum levels of FGF23, a hormone associated with decreased phosphate resorption, due to a maxillary tumor. The patient was treated with oral phosphorus and calcitriol, and surgical removal of the tumor was performed. After 21 months follow-up, he was pain free and had returned to full activity. We review the reported pediatric cases of TIO in the maxillofacial and oral region and discuss the management of these patients considering the published evidence.

Ossifying Fibroma of Mandible - A Case Report.

INTRODUCTION: Ossifying fibromas are rare, non-aggressive benign tumours of the bone, commonly involving the posterior mandible in middle-aged individuals with a female predilection. FINDINGS: Clinical manifestations include asymptomatic expansion of the mandible with infrequent maxillary lesions, pain, malocclusion, and compromised quality of life including aesthetic perception. Owing to multiplicity of features, tendency of recurrence, and possibility of malignant transformation, the diagnosis, treatment, and post-operative management of ossifying fibroma are always a challenge. TAKEAWAY LESSONS: Study aims to report a clinical case of extensive swelling involving the coronoid process and condyle on the right side to crossing the mid-line of the mandible with compromised functions and aesthetics. The article describes the clinical, histopathological, and radiological features of the case. The possible treatment and challenges encountered are discussed.

Extraosseous cemento-ossifying fibroma beneath the left buccal mucosa: a case report.

Cemento-ossifying fibroma (COF) is a benign mesenchymal odontogenic tumor that commonly occurs in the tooth-bearing areas of the maxilla and mandible. This study reports a COF case located under the left buccal mucosa. The classification and differential diagnosis of this COF case were discussed based on the diagnosis and treatment of this case and previous literature.

Fibroma ossificante juvenil trabecular: relato de caso / Juvenile trabecular ossifying fibroma: case report / Fibroma osificante trabecular juvenil: reporte de un caso

O fibroma ossificante juvenil trabecular (FOJTr) é uma lesão fibro-óssea benigna rara de comportamento agressivo, alto potencial de recorrência, e acometimento no esqueleto craniofacial de crianças e adolescentes. Uma paciente do gênero feminino, 8 anos de idade, compareceu ao ambulatório de Patologia Oral e Maxilofacial da Universidade de Gurupi ­ UNIRG para avaliação clínica de um aumento de volume na região de corpo da mandíbula do lado esquerdo. Não havia sintomatologia dolorosa e sequer desconforto. Nos exames de imagem (radiografia panorâmica e tomografia computadorizada) foram observados uma extensa área radiolúcida que se estendia desde o primeiro molar permanente com rizogênese incompleta até o incisivo central do lado oposto. Após a realização da biópsia incisional e laudos histopatológicos realizou-se a remoção completa da lesão incluindo os remanescentes decíduos sobrejacentes ao fibroma. Nas imagens de controle pós-operatório aos 90 dias (radiografia panorâmica e tomografia computadorizada), notou-se sinais de neoformação óssea com espessamento basilar e os germes dos dentes permanentes em franco desenvolvimento. Diante disso, ressalta-se a importância do conhecimento dos aspectos clínicos, radiográficos e histopatológicos para a realização de um correto diagnóstico e tratamento adequado afim de reduzir as altas taxas de recidivas... (AU)

Trabecular juvenile ossifying fibroma (TrJOF) is a rare benign fibro-osseous lesion, with aggressive behavior, high recurrence potential, which affects the craniofacial skeleton of children and adolescents. This paper aims to describe a clinical case in a female patient, 8 years old, who attended the Oral and Maxillofacial Pathology outpatient clinic ­ Faculty of Dentistry ­ University of Gurupi - UNIRG, city of Gurupi - TOCANTINS - BRAZIL for clinical evaluation of an increased in volume in the region of the mandible body, on the left side. There was no painful symptomatology or even discomfort. Imaging examinations (panoramic radiography and computed tomography (CT) showed an extensive radiolucent area that extended from the first permanent molar with incomplete root formation to the central incisor on the opposite side. After performing an incisional biopsy and histopathological examination, the lesion was completely removed included the remainder deciduous teeth overlying the tumor. In the postoperative control images at 90 days (panoramic radiography and CT), signs of bone neoformation with basilar thickening and the germs of the permanent teeth in full development were noted. In view, this importance of knowledge of clinical, radiographic and histopathological aspects is emphasized for the realization of a correct diagnosis and adequate treatment in order to reduce the high rates of relapses... (AU)

El fibroma osificante trabecular juvenil (TRFOJ) es una lesión fibroósea benigna rara con comportamiento agresivo, alto potencial de recurrencia y afectación del esqueleto craneofacial de niños y adolescentes. Paciente femenina de 8 años de edad que acude al ambulatorio de Patología Oral y Maxilofacial de la Universidad de Gurupi - UNIRG para evaluación clínica de aumento de volumen en la región del cuerpo mandibular del lado izquierdo. No presentaba sintomatología dolorosa ni molestias. Los exámenes de imagen (radiografía panorámica y tomografía computarizada) mostraron una extensa área radiolúcida que se extendía desde el primer molar permanente con formación radicular incompleta hasta el incisivo central del lado opuesto. Tras realizar la biopsia incisional y los informes histopatológicos, se procedió a la extirpación total de la lesión, incluidos los remanentes caducos que recubrían el fibroma. En las imágenes de control postoperatorio a los 90 días (radiografía panorámica y tomografía computarizada), se observaron signos de neoformación ósea con engrosamiento basilar y los gérmenes de los dientes permanentes en pleno desarrollo. Por tanto, es importante conocer los aspectos clínicos, radiográficos e histopatológicos para la realización de un diagnóstico correcto y un tratamiento adecuado con el fin de reducir las altas tasas de recaídas... (AU)

Clínica, epidemiología e imagenología del fibroma osificante y fibroma cemento osificante: revisión de casos reportados en la literatura / Clinic, epidemiology and imagenology of the ossifying fibroma and cemento ossifying fibroma: a review of cases reported in the literature

Introducción: El fibroma osificante (FO) y el fibroma cemento osificante (FCO) son patologías distintas, que hasta el 2017 se consideraban como una. A la fecha no se han comparado las características del FO y el FCO. La presente revisión tuvo como objetivo analizar características clínicas, epidemiológicas e imagenológicas del FO y FCO en los casos publicados. Materiales y métodos: Se realizó una búsqueda de casos clínicos de FO y FCOpublicados desde el año 2015 en PubMed, Scopus y Web of Science, mediante la estrategia de búsqueda ("Ossifying Fibroma" OR "Cemento Ossifying Fibroma") AND (Craniofacial OR Jaws). Los casos debían presentar información clínica, imagenológica e histológica suficientes para confirmar su diagnóstico, patrón histológico y comparar sus características. Revisión: Se incluyeron 32 artículos, con 32 casos y 34 lesiones, siendo 6 FCO y 28 FO. Los FO y FCO se diferencian por su edad y ubicación: mientras los FCO se presentan en edades adultas, exclusivamente en los maxilares y en relación con tejidos dentarios, los FO lo hacen principalmente en niños y jóvenes, y en cualquier hueso. Los FO y FCO tienen las mismas características imagenológicas: son lesiones uniloculares o multiloculares, con distintos grados de radiodensidad, límites definidos y una radiolucidez periférica. Sin embargo, los casos de FO trabecular pueden no presentar esta radiolucidez periférica. Conclusión: Los FO y FCO son patologías, clínica y epidemiológicamente similares, y con las mismas características imagenológicas. Por lo que establecer diferencias histológicas es esencial para un correcto diagnóstico. (AU)

Introduction: Ossifying fibroma (OF) and cemento ossifying fibroma (COF) are different pathologies, which until 2017 where considered as one. To date, the features of OF y COF have not been compared. This aim of this review was to analyze clinical, epidemiological and imaging features of OF and COF in published case reports. Materials and methods: A search of clinical cases of OF and COF published since 2015 was performed on PubMed, Scopus and Web of Science, using the search strategy ("Ossifying Fibroma" OR "Cemento Ossifying Fibroma") AND (Craniofacial OR Jaws). The cases had to haveenough clinical, imaging and histological information to confirm their clinical diagnosis, histological pattern and compare their features. Review: 32 articles were included, with 32 cases and 34 lesions, being 28 OF and 6 COF. OF and COF differ by age and location: while COF occur in adult ages, exclusively in the jaws and in relation to dental tissues, OF occur mainly in children and young people, and in any bone. OF and COF have the same imaging characteristics: they are unilocular or multilocular lesions, with different degrees of radiodensity, defined limits and a peripheral radiolucent area. However, cases of trabecular OF may not present this peripheral radiolucent area. Conclusions: OF and COF are similar pathologies clinically and epidemiologically, with the same imaging characteristics. So, establishing histological differences is essential for an accurate diagnosis. (AU)

SATB2 Immunoexpression in Peripheral Ossifying Fibroma and Peripheral Odontogenic Fibroma.

Peripheral ossifying fibromas (POFs) and peripheral odontogenic fibromas (POdFs) appear clinically similar but of different histogenesis. The novel marker SATB2 is involved in regulation of osteoblastic differentiation and phenotype. However, SATB2 expression has not been previously explored in POFs and POdFs. Given the potential for mineralized tissue formation in POFs and POdFs, and to more clarify the phenotype of the lesional cells, this study was aimed to immunohistochemically investigate SATB2 expression in POFs and POdFs. Fourteen cases of POF and POdF (7 cases each) were selected, stained for SATB2 immunohistochemically, and scored according to the percentage of positive lesional cells (0, no staining; 1 +, < 5%; 2 +, 5-25%; 3 +, 26-50%; 4 +, 51-75%; and 5 +, 76-100%), and the intensity of staining was graded as weak, moderate, or strong. The control group included the inflammatory fibrous hyperplasia-like area present in two cases, 1 case fibroma, and 1 case giant cell fibroma. Moderate to strong, and diffuse SATB2 nuclear immunoreactivity was detected in the lesional cells of all cases of POFs and POdFs with variable scores; 3-5 + for the POFs and 3-4 + for the POdFs (P = 0.101). The distribution of staining was more prominent in those lesional cells associated with the osteoid/calcification in the cases of POFs. No staining was noted in the control group. The lesional cells in both POFs and POdFs express SATB2 and may exhibit the osteoblastic-like phenotype. SATB2 staining may be useful for diagnosis of subsets of POFs with minimal or absent calcification and some POdFs with unidentifiable odontogenic epithelium.

Fibrous and Fibro-Osseous Lesions of Bone.

Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap significantly, an interdisciplinary approach helps to consolidate the classification of these tumors. Herein, the clinical, radiologic, and pathologic features of lesions within these categories are described.

Peripheral ossifying fibroma: A case report.

Peripheral ossifying fibroma (POF) is a reactive lesion that originates in the gingiva. Morphological similarity to other epulides and similar nomenclature to neoplastic tumors give rise to obvious problems both at the stage of diagnosis and treatment of POF. Although POF is one of the less common gingival lesions, it accounts for up to 2% of all oral lesions, which means that most general dentists will encounter POF in their practice. This paper aims to present the case of a 56-year-old woman diagnosed with POF. In the described case, POF most likely resulted from chronic periodontal irritation due to the presence of prosthetic crowns. The applied treatment in the form of total excision of POF, removal of irritants and gingivoplasty brought the expected therapeutic effect, i.e. no recurrence in the period of the hitherto follow-up. Terminological difficulties, the basics of differentiating typical gingival tumors, therapeutic options with suggested management of POF, and recurrence rate with the follow-up scheme were discussed. Despite the reactive nature of POF, this lesion recurs up to 20% and can transform to giant POF. For this reason, POF should not be underestimated, and proper diagnosis, treatment, and follow-up are critical to the success of therapy.

A PHF1-TFE3 fusion atypical ossifying fibromyxoid tumor with prominent collagenous rosettes: Case report with a brief review.

Ossifying fibromyxoid tumor (OFMT) is a rare mesenchymal neoplasm of uncertain line of differentiation that can be subdivided into typical, atypical, and malignant tumors. Cytogenetically, OFMT is characterized by recurrent gene rearrangement involving PHF1 in up to 85% of cases. The most common PHF1 fusion partner is EP400, present in approximately half of cases. Most recently, a novel fusion of PHF1-TFE3 was identified in about 10% of PHF1-rearranged OFMTs. Herein, we report a unique case of PHF1-TFE3 fusion atypical OFMT with prominent collagenous rosettes. A 50-year-old male patient presented with a slowly growing, painless mass in the right foot for 4 years. Gross examination showed a 3.5-cm, subcutaneous well-circumscribed, lobulated mass. Microscopic examination revealed a well-demarcated but un-encapsulated tumor without a peripheral bony shell. The neoplasm was composed of mildly atypical spindle to ovoid cells with increased mitosis (2 mitoses per 10 high-power fields) arranged in a multinodular manner within a fibromyxoid stroma, which contained numerous small, irregular collagenous rosettes surrounded by radiating growth of tumor cells. The neoplastic cells were diffusely positive for TFE3 and CD10. RNA sequencing revealed an in-frame fusion between PHF1 exon 12 and TFE3 exon 7. Subsequent Fluorescence in-situ hybridization analyses demonstrated positive for rearrangements of both the PHF1 and TFE3 loci. The patient was free of disease at 63 months' follow-up. Our case exhibits atypical features and prominent collagenous rosettes, expanding the morphological spectrum of OFMT with PHF1-TFE3 fusion.

Juvenile ossifying fibroma of the maxillofacial region: analysis of clinico-pathological features and management / Fibroma osificante juvenil de la región maxilofacial: Análisis de características y manejo clínico-patológico

Background: The diagnosis and management of juvenile ossifying fibroma (JOF) remains a highly debated topicwith paucity of studies with long-term follow-up, hence the aim of this study was to report on the clinico-patho-logical features and management of these neoplasms.Material and Methods: A retrospective analysis was performed on all histopathologically confirmed JOF pre-senting 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 minimumpost-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 patientsranged from 3–31 years (mean= 13 years) with male to female ratio of 1.8:1. The ages of patients diagnosed withTrabecular JOF were significantly younger than patients with Psammomatoid JOF (P = 0.01). The majority ofpatients 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 Trabecu-lar 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 ostectomywas shown to be the least invasive method with an acceptable recurrence rate (10%). Six lesions underwent resec-tion 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 siteshould not be a major determining factor in the diagnosis of JOF. Moreover, curettage with peripheral ostectomy...(AU)

Similar appearance of different multifocal carpal bone destructing disease entities in 3 patients: A case report.

RATIONALE: Several diseases feature tumors, or tumor-mimicking lesions, that further invade the bone and surrounding joints of the wrist region. Here, we describe 3 rare cases of multiple destructed carpal bones and adjacent joints in different disease entities confirmed via pathologic diagnosis. PATIENT CONCERNS: All 3 cases were examined between January 2016 and December 2019. Three patients presented with similar clinical manifestations and radiographic features, with multiple osteolytic lesions in the carpal bones and metacarpal bone base. DIAGNOSES: The 3 cases were diagnosed as diffuse type tenosynovial giant cell tumor, calcifying aponeurotic fibroma, and rheumatoid arthritis. INTERVENTIONS: Separate, experienced radiologist and pathologist took part in the interpretation and compartmentalization of radiographs and pathological findings, respectively. Even magnetic resonance imaging could not achieve a diagnosis; surgical excision was therefore required, with subsequent pathological assessment for treatment and final diagnosis. OUTCOMES: functional outcomes also differed among patients, poorest in rheumatoid arthritis patient. LESSONS: We report 3 rare disease entities, presenting with multifocal osteolytic lesions in the wrist. They all presented with similar clinical manifestations, and the final diagnoses were made via pathological evaluation. Compared with tenosynovial giant cell tumor and calcifying aponeurotic fibroma, rheumatoid arthritis had the poorest outcome.