Distinguishing the indistinct: a comparative clinicopathologic and radiologic analysis of cemento-ossifying fibroma, psammomatoid ossifying fibroma, and juvenile trabecular ossifying fibroma".

Classification of Fibro-Osseous Tumors in the Craniofacial Bones Using DNA Methylation and Copy Number Alterations.

Ossifying fibroma and juvenile ossifying fibroma: A systematic review on clinical and radiological parameters, treatment modalities and recurrence

Ossifying fibromas of the head and neck region are classified as cemento-ossifying fibroma (COF) (odontogenic origin), and two types of juvenile ossifying fibromas: juvenile trabecular ossifying fibroma (JTOF), and juvenile psammomatous ossifying fibroma (JPOF). The potential for recurrence in JTOF and JPOF and the discovery of newer molecular signatures necessitates accurate histological classification. Over 12years (2005-2017), a total of 45 patients with 51 tumours were retrieved and reviewed for clinic-pathological features from the archives of a tertiary care oncology centre. Of 45 cases, COF, JTOF and JPOF comprised 13 (28.9%), 11 (24.4%) and 18 (40%) cases respectively. Three cases were unclassifiable. M: F ratio was 1:3.3, 1.1:1, 2:1 for COF, JTOF and JPOF respectively with an age range of 6-66years (mean: 24.6, median; 18.1years). The most common site for COF was mandible, for JTOF was maxilla, and for JPOF was ethmoid sinus. One case of mixed JTOF and JPOF histology was seen. Aneurysmal bone cyst-like areas were seen in 26.6% of cases, most commonly in JPOF. Follow up was available in 23 cases, and ranged from 4 to 207months. Three cases of JPOF had a recurrence and one patient with JTOF had residual disease after surgery. One case of COF demonstrated increased parathyroid hormone levels. COF, JTOF, and JPOF are clinically, radiologically and histologically distinct entities. Surgical resection is the mainstay of treatment. JPOF has a higher incidence of recurrence as compared to JTOF and COF and hence needs a more aggressive follow-up.

The World Health Organization's (WHO) 2022 update on the classification of odontogenic and maxillofacial bone tumors has revolutionized diagnostic and treatment paradigms by integrating novel molecular insights. Fibro-osseous lesions of the maxillo-facial bones constitute a heterogeneous group encompassing fibrous dysplasia, Psammomatoid Ossifying Fibroma (PSOF), Juvenile Trabecular Ossifying Fibroma (JTOF), and other variants. Despite histological similarities, their distinct clinical manifestations and prognostic implications mandate precise differentiation. The intricacies of diagnosing fibro-osseous lesions pose challenges for pathologists, maxillofacial surgeons, dentists and oral surgeons, underscoring the importance of a systematic approach to ensure optimal patient management. Herein, we present two cases, fibrous dysplasia and Cemento-Ossifying Fibroma, detailing their clinical encounters and management strategies. Both patients provided informed consent for publishing their data and images, adhering to ethical guidelines.

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.

Journal of Diagnostic Pathology is a peer reviewed journal published biannually by the College of Pathologists of Sri Lanka. The journal publishes manuscripts dealing with all aspects of pathology. The aim of the journal is to update knowledge and encourage original research in the field of pathology.

To assess the sclerostin, β-catenin, and tryptase expression in fibro-osseous lesions (FOL) of the jaws. Immunohistochemistry analysis was performed for these proteins on FOL and non-lesional bone. The sclerostin-positive cells were scored from 0 (no expression) to 3 (high expression). We analyzed 46 FOL biopsies and selected 38 patients. Categorization showed 15 fibrous dysplasia (FD), eight juvenile trabecular ossifying fibroma (JTOF), two psammomatoid ossifying fibroma (PsOF), and 13 FOL. We found more sclerostin-positive cells in fibrous tissue than in bone, showing a phenotype like mast cells with strong dot-cytoplasmic positivity. The analysis of sclerostin-positive cell lesions (scored as 2 and 3) showed also tryptase positivity in 80.9% of 21 biopsies. β-catenin was diffusely expressed on the fibrous component, mostly with cytosol staining. Non-lesional bone showed sclerostin expression in medullary spaces and a few osteocytes. Sclerostin-positive cells are mostly found in the fibrous tissue of FOL, and the tryptase mast cell marker was present in most of the lesions that were positive for sclerostin.

Surgical approach for a rare case of bilateral trabecular juvenile ossifying fibroma in the mandible

Juvenile trabecular ossifying fibroma (JTOF) is a rare variant of ossifying fibroma, a benign fibro-osseous lesion. This entity is characterized by aggressive clinical behavior, predominantly affecting young individuals aged 8–12 years. A slight male predominance has been observed, and the lesion demonstrates a marked propensity for recurrence. Central giant cell granuloma (CGCG) is classified by the World Health Organization as a benign nonodontogenic lesion of the jaw, with unknown etiology. It is an uncommon, benign but aggressive osteolytic neoplasm of the craniomaxillofacial region. We report a case of a 6-year-old female child who presented with a painless, slow-growing swelling over her left cheek for 3 months. A large expansile lesion of neoplastic etiology of the left maxilla was suspected clinically and on radiological examination. A thorough histological examination revealed it to be a hybrid lesion of CGCG with JTOF, which in combination is extremely rare.

An obturator prosthesis for a 4-year-old child following treatment of a juvenile trabecular ossifying fibroma with a maxillary resection

Benign fibro-osseous lesions within the maxillofacial region represent a heterogeneous group of benign entities with overlapping histologic features. Ossifying fibroma, the rarest of these entities, represents a true neoplasm. Juvenile ossifying fibroma (JOF) is considered an aggressive rapidly growing sub-type. It tends to occur in the first or second decades of life. Based on histological and clinical features it can further be classified into two variants, namely juvenile trabecular ossifying fibroma (JTOF) and juvenile psammomatoid ossifying fibroma (JPOF). JTOF features a proliferation of cellular fibroblastic tissue admixed with woven bone trabeculae with varying histologic presentations. Correlation with clinical and radiographic features is essential to differentiate it from other fibro-osseous lesions. A case of JTOF of the mandible is exemplified in this Sine Qua Non Radiology-Pathology article.

Ossifying fibroma (OF) is an uncommon benign fibro-osseous lesion. Based on its clinical, morphological, and radiological features, OF is further divided into cemento-ossifying fibroma (COF), juvenile psammomatoid ossifying fibroma (JPOF), and juvenile trabecular ossifying fibroma (JTOF). JPOF rarely involves the cranial base, with limited reports published on spheno-orbital JPOF. In this paper, we report a case of JPOF of the greater wing of the sphenoid bone and lateral orbital wall in an 11-year-old child and show a surgical video. Although rare, JPOF should be considered in the differential diagnosis of fibro-osseous lesions of the spheno-orbital region.

Psammomatoid ossifying fibroma of the paranasal sinuses. An extragnathic variant of cemento-ossifying fibroma: Report of three cases

Ossifying fibromas of the jaws and craniofacial bones

Fibrous dysplasia (FD) and cemento-ossifying fibroma (COF) are benign fibro-osseous lesions that are generally considered to be separate entities, distinguished by histologic and radiographic features. In our experience, some lesions lack the classic clinical, radiographic, or pathologic features of FD or COF and rather have overlapping features of both entities. Consequently, these cases are frequently diagnosed nonspecifically as fibro-osseous lesions. We examined 56 gnathic and extragnathic fibro-osseous lesions of bone morphologically, clinically, and radiographically to determine whether they can be reliably distinguished and whether their distinction has any clinical or prognostic significance. The lesions exhibited a broad morphologic spectrum of patterns ranging from pure FD (24 cases) to pure COF (10 cases). Twenty-two lesions contained a mixture of both patterns; 11 lesions with a predominant FD pattern contained calcified spherules histologically indistinguishable from those characteristically seen in COF. The remaining 11 lesions contained areas of typical FD adjacent to areas of COF. The lesions examined also demonstrated considerable radiographic overlap, and FD could not be reliably distinguished from COF. The recurrence rate was low for all lesions regardless of the histologic pattern. Because of histologic and radiographic overlap and similar low recurrence rate of FD and COF, we consider them to be related lesions, and COF is probably an opposing end of a morphologic spectrum of FD.