Juvenile trabecular ossifying fibroma of the inferior turbinate.

Nasal obstruction is a commonly reported issue in the Otorhinolaryngology Outpatient Department. In this case, an early adolescent boy with a long-standing problem of right-sided nasal obstruction since childhood sought consultation. Diagnostic nasal endoscopy revealed a deviation of the nasal septum to the left, coupled with right inferior turbinate hypertrophy, all overlying healthy mucosa. A CT scan of the nose and paranasal sinuses further identified a bony hyperdense lesion with ground glass attenuation, confined to the right inferior turbinate. Subsequent biopsy confirmed juvenile trabecular ossifying fibroma (JTOF). The patient underwent endoscopic right medial maxillectomy, and the final histology affirmed the diagnosis of JTOF.

Psammomatoid ossifying fibroma in the frontal sinus: An intriguing clinical encounter-A detailed case report.

BACKGROUND: Psammomatoid ossifying fibroma (POF) is a rare craniofacial neoplasm, primarily affecting the maxillofacial region, and typically observed in adolescents and young adults. This case report presents a unique occurrence of POF in a 50-year-old male, defying the conventional age range and exhibiting an unusual anatomical location within the frontal sinus. CASE: A 50-year-old male with a prior history of cecal adenocarcinoma and colectomy presented with left eye proptosis and new-onset headaches. Imaging revealed a well-defined calcified mass in the left frontal sinus, leading to a diagnosis of POF. Open surgical resection was performed to remove the tumor, and histopathological evaluation confirmed its diagnosis as psammomatoid ossifying fibroma. The patient exhibited no postoperative complications or signs of recurrence. CONCLUSION: This case underscores the diverse clinical presentations and diagnostic challenges associated with POF, emphasizing the importance of accurate diagnosis and multidisciplinary collaboration. Further research is needed to explore the genetic underpinnings and optimal management strategies for this intriguing condition.

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 of Fibro-osseous Lesions and Other Bone Conditions of the Jaws.

This review directs the focus on the imaging features of various fibro-osseous lesions and other bone lesions that can be of similar presentation. Broad diagnosis of "fibrous osseous lesion" may culminate in improper treatment and management. Radiographic discriminating factors between these entities are highlighted and summarized to improve the diagnostic process when encountering these lesions.

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.

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

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

Giant fronto-spheno-orbitary juvenile psammomatoid ossifying fibroma: Case report and literature review.

Juvenile psammomatoid ossifying fibroma (JPOF) is an osteofibrous neoplasm that originates in the craniofacial skeleton typically during the first three decades of life. JPOFs usually involve the orbit, paranasal sinuses or the jaws. Extensive involvement of the anterior cranial base with compromised visual function is a rare phenomenon. In such clinical context, a definite diagnosis can only be made on the basis of histopathological findings, given the absence of pathognomonic radiological features. Despite being considered a benign entity, JPOFs present a locally aggressive behavior. Therefore, these neoplasms must be included in the differential diagnosis in every patient harboring a skull base osteofibrous lesion, and, once diagnosed, gross total surgical removal should be attempted. In this study, we present our experience in the diagnosis and treatment of a patient diagnosed with a giant JPOF involving the cranial base.

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.

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.

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.

Benign bony lesions of paranasal sinuses and skull base: from osteoma to fibrous dysplasia.

PURPOSE OF REVIEW: Benign bony lesions of the craniofacial complex are relatively common. However, their location close to critical neurovascular structures may render their treatment, if required, highly challenging.This article reviews the current literature on their pathophysiology, diagnosis, natural course and treatment, with a focus on most recent findings. RECENT FINDINGS: A new classification has been suggested concerning endoscopic resectability. The ratio of lateral frontal to interorbital distance can accurately and reliably predict the endoscopic reach to lateral frontal sinus, while orbital transposition can assist us in reaching lateral frontal sinus when anatomy is unfavorable. New and combined endoscopic transnasal and transorbital approaches are now in the surgical armamentarium. Prophylactic optic nerve decompression in fibrous dysplasia is absolutely contraindicated as it leads to worse visual outcomes. Radiotherapy of such lesions is of no benefit and may lead to a higher risk of malignant transformation. The presence of Guanine Nucleotide binding protein Alpha Stimulating (GNAS) mutation in chromosome 20 is universally present in fibrous dysplasia and can differentiate them from ossifying fibromas. SUMMARY: Diagnosis and therapeutic management of benign craniofacial bone lesions remains challenging. If surgical treatment is contemplated, the morbidity of the intervention should always be weighed against the potential benefits. Evolution of extended endoscopic endonasal and transorbital surgery means that more lesions can be reached purely endoscopically with better oncological and cosmetic results.

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.

A Periodontal Perspective on the Successful Treatment of Recurrent Benign Gingival Lesions Affecting the Anterior Dentition: Two Case Reports.

Recurrent benign gingival lesions occurring in the anterior dentition are clinical dilemmas. While complete removal of such lesions is required to prevent recurrence, this can result in a poor esthetic outcome. Relative to this conundrum, this report discusses the diagnosis, psychologic management, and clinical treatment of two patients with recurring lesions on the facial gingiva of the mandibular and maxillary incisors, respectively. Patient A, a 55-year-old woman, presented with a recurrent peripheral ossifying fibroma (POF); Patient B, a 76-year-old man, presented with a recurrent pyogenic granuloma (PG). Both patients underwent multiple procedures and were ultimately treated without lesion recurrence. The efficacious surgical treatment of recurrent gingival lesions like POF and PG requires an aggressive approach involving lesion removal of the lesion as well as a 1.0- to 2.0-mm margin of normal tissue, underlying alveolar bone, and associated periodontal ligament (PDL). The rationale for this approach stresses the potential periodontal and esthetic ramifications that were considered. In summary, when recurrent benign gingival lesions are localized to the anterior part of the mouth, the approach to their surgical removal should be modified to minimize the extent of gingival recession and other potential esthetic issues.

Preserving periodontal tissue in the treatment of a large peripheral ossifying fibroma: a case study.

Peripheral ossifying fibroma (POF) is a reactive, benign gingival enlargement. Its etiology is not fully known. It can be seen in many different sizes in the mouth. The histopathological appearance of POF is mineralized tissue and fibrous proliferation. All relevant soft and hard tissues must be removed to prevent recurrence. Periodontal tissue remaining after excision is important for tooth preservation. With large lesions, the loss of healthy periodontal tissue is also large. Periodontal surgical approaches are important to preserve the remaining periodontal tissue. The positive effects of autogenously obtained titanium-prepared platelet-rich fibrin (T-PRF) and connective tissue graft (CTG) on soft tissue are well known. A 34-year-old woman presented with a fibrous and pedunculated gingival mass in the upper left canine premolar region. The operation was performed with complete excision of the lesion down to the bone along with the surrounding healthy tissue. Periodontal treatment of the large defect created after excision of a large POF lesion was performed with laterally positioned flap, CTG and T-PRF. The periodontal tissue and defect were noted to heal in a healthy manner at the 6-month follow-up. POF is a benign lesion; however, it has a high recurrence rate. Complete elimination of the lesion is crucial to prevent recurrence. Periodontal surgical methods and biomaterials applied after surgical excision are significant to maintain the periodontal health of the remaining teeth and tissues.

Ossifying fibroma presented as an incidental [18F]FDG PET/CT uptake within the hard palate.

Incidental uptake in [18F]FDG PET/CT is not uncommon, but uptake in the oral and sinonasal regions was less frequently reported. We present a case of incidental focal [18F]FDG PET/CT uptake within the hard palate, which was later revealed to be an ossifying fibroma. We also reviewed some relevant literature and suggested that further investigation may be necessary for some patients with incidental [18F]FDG PET/CT uptake in the oral and sinonasal regions.