Juvenile Psammomatoid Ossifying Fibroma Research Articles

Diagnosis and Surgical Management of Juvenile Psammomatoid Ossifying Fibroma: A Case Report

Diagnosis and Surgical Management of Juvenile Psammomatoid Ossifying Fibroma: A Case Report

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.

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.

Juvenile psammomatoid ossifying fibroma: A case report and a review of the literature

Abstract Juvenile psammomatoid ossifying fibroma (OF) is a rare histological variant of OF with aggressive behavior. It is a benign fibro-osseous lesion with a high rate of recurrence. It occurs mainly in male children with paranasal sinuses and orbits the most common sites. The diagnosis depends on clinical, radiologic, and pathologic correlation. This study aims to present a case report of juvenile psammomatoid OF (JPOF) in a 9-year-old boy and to also review the relevant literature. A case of a 9-year-old boy who presented with a 1-year history of the left maxillary swelling was presented. There was no associated history of pain, ulceration, bleeding, or teeth mobility. There was no history of previous trauma to the site or teeth extraction. The patient had no difficulty in chewing, speaking, nasal blockage, or discharge. Examination revealed an ovoid left maxillary buccal mass measuring about 4 cm × 3 cm, which was hard, nontender with no teeth mobility. The diagnosis of JPOF made was confirmed through a combination of clinical, radiological, and histopathological findings. The patient had enucleation of the lesion under general anesthesia. Follow-up was satisfactory with no recurrence. JPOF is a rare histological variant of OF that is known to exhibit a high rate of recurrence. Although clinical and radiological features contribute to its diagnosis, histopathological examination has been the main diagnostic modality. Early detection and treatment are essential.

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.

Massive Juvenile Psammomatoid Ossifying Fibroma in the Mandible With a Complex Clinical Course: Case Report and Literature Review

The juvenile ossifying fibroma is a rare subset of the benign ossifying fibroma. These tumors have an aggressive behavior and often cause severe destruction of bone and surrounding tissue. JOF can be separated into the juvenile trabecular ossifying fibroma and juvenile psammomatoid ossifying fibroma. In this article, we present a case of an 18 year old male who presented with a rapidly growing massive tumor originating in the right mandible that caused severe local destruction. Due to the size of the tumor, the patient had impairment in many activities of daily living. Pre-operative management was further complicated due to the vascular nature of the tumor which resulted in spontaneous bleeding. The tumor was successfully resected and final pathology revealed a diagnosis of juvenile psammomatoid ossifying fibroma. In this article, we describe treatment of a debilitating mandibular tumor and provide recommendations for other clinicians who encounter similar cases. Additionally, the demographics and histopathological features of the juvenile ossifying fibroma and its subtypes are described.

First presentation of a frameshift mutation in the SETD2 gene of a juvenile psammomatoid ossifying fibroma (JPOF) associated with an aneurysmal bone cyst

BackgroundThe rarity of juvenile psammomatoid ossifying fibroma (JPOF) and lack of cytogenetic studies prompted us to report a novel SETD2 gene mutation in a benign odontogenic tumour.Case presentationA 21-year-old man presented with a hard, expanded mandibular cortex. Computed tomography revealed multilocular radiopacity in the mandible; this was reconstructed via segmental mandibulectomy using a vascularised iliac crest flap. Based on the clinical and histological findings, we diagnosed JPOF associated with an aneurysmal bone cyst. Microscopically, the solid area was characterised by many rounded or angular ossicles in a cellular fibrous stroma. The stromal cells were spindle-like or stellate. Next-generation sequencing detected a frame shift mutation of the SETD2 gene, while the copy number was normal.ConclusionsOur findings suggest further genetic studies should be performed to assess whether this mutation is related to tumour genesis.

RECURRENT JUVENILE PSAMMOMATOID OSSIFYING FIBROMA: A CASE REPORT

<h3>Background</h3> Juvenile psammomatoid ossifying fibroma (JPOF) is a rare, benign fibro-osseous lesion with a mean patient age of 16-33 years. JPOFs present as bony expansion, invasion, and destruction of surrounding tissue. <h3>Objective</h3> To report a case of recurrent JPOF. <h3>Case Report</h3> We present a case of recurrent JPOF. An 8-year-old girl reported to our department with the chief complaint of gradual painless swelling in the chin. The oral examination revealed a hard and nontender swelling in the anterior region of the mandible. The orthopantomogram revealed a radiolucent mass with ill-defined borders. The computed tomographic scan showed a nearly round expansile lesion, which showed multilocular radiolucency, 4.3 cm × 3.0 cm in size, and with an indefinite border. Postoperative histopathological diagnosis was reported as cemento-ossifying fibroma. Before reporting to us, the patient had been admitted to another hospital 2 years earlier with painless swelling in a similar position of the mandible. The clinical manifestations were similar to those this time. Compared with this episode, the lesion scope was small, 2.0 cm × 3.0 cm in size. The postoperative histopathologic diagnosis was reported as odontogenic myxoma at that time. <h3>Discussion</h3> Two years ago, histopathology showed a fibrocellular stroma with myxoid areas, and no calcifications were observed. In this episode, microscopic examination revealed numerous spherical ossicles resembling psammoma bodies in the cellular fibrous tissue, which are a pathognomonic histopathologic feature of JPOF. JPOFs present with aggressive local growth and high risk of recurrence. Therefore, accurate pathological diagnosis and complete surgical resection are required.

Spheno-orbital juvenile psammomatoid ossifying fibroma: a case report and literature review

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.

Juvenile Trabecular Ossifying Fibroma (TrJOF) Orginating from Lateral Fronto-Orbital Bone with Secondary Aneurysmal Bone Cyst (ABC) Formation: Report of an Unusual Case and Literature Review

Juvenile trabeculated Ossifying Fibroma (JTrOF) is commonly arising from the maxilla and the mandibular bones, while Psammomatoid Juvenile Ossifying Fibroma (PsJOF) commonly arises in the sinonasal and calvarial bones. In our paper, we described a 12-year-old pediatric female who presented with a right fronto-orbital disfiguring swelling. She underwent total resection, peripheral osteotomy, and reconstruction. Pathology reported a TrJOF associated with Aneurysmal Bone Cyst (ABC) formation. TrJOF rarely originates from superolateral wall orbital bone, and it is exceedingly rare when it is associated with ABC formation. Pathologically, it is difficult to distinguish TrJOF from other FOLs due to similarities and overlap. The ideal treatment is total surgical excision and reconstruction. The Surgical resection is challenging as regards functional and cosmetic outcomes.

Juvenile ossifying fibroma of the maxillofacial region: analysis of clinico-pathological features and management.

Background The diagnosis and management of juvenile ossifying fibroma (JOF) remains a highly debated topic with paucity of studies with long-term follow-up, hence the aim of this study was to report on the clinico-pathological features and management of these neoplasms.Material and Methods A retrospective analysis was performed on all histopathologically confirmed JOF presenting 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 minimum post-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 patients ranged from 3–31 years (mean= 13 years) with male to female ratio of 1.8:1. The ages of patients diagnosed with Trabecular JOF were significantly younger than patients with Psammomatoid JOF (P = 0.01). The majority of patients 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 Trabecular 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 ostectomy was shown to be the least invasive method with an accepTable recurrence rate (10%). Six lesions underwent resection 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 site should not be a major determining factor in the diagnosis of JOF. Moreover, curettage with peripheral ostectomy should be used as the first line of management to minimize morbidity to the patient and that resection should be reserved for large and recurrent lesions. Key words:Juvenile ossifying fibroma, juvenile trabecular ossifying fibroma, juvenile psammomatoid ossifying fibroma, fibro-osseous lesions, maxillofacial region, multi-centre study.

Intraoral Approach for Surgical Treatment of Psammomatoid Juvenile Ossifying Fibroma

Psammomatoid juvenile ossifying fibroma (PJOF) is a rare benign tumor that usually affects the paranasal sinuses, orbit, and skull. In most cases, extensive incisions are necessary for full access to the tumor site. The aim of this paper is to report a case of extensive PJOF in which an intraoral surgical approach was performed with complete excision of the tumor. A female patient, 18-year old had a deforming volume increase in the region of the left facial middle third with an approximate evolution time of 2 years. She complained of headache, epiphora in the left eye, and total obstruction of the left nostril. Extraoral examination showed facial asymmetry with dystopia, ocular proptosis, and considerable sclera exposition of the left eye. Tomography examination showed a mixed aspect lesion on the left side of the face, well delimited. The intraoral surgical approach was chosen for the excision of the lesion. Under general anesthesia and nasotracheal intubation, total resection was performed, followed by exodontia of the directly involved teeth. In the definitive histopathological examination, the diagnosis was confirmed. The patient is currently with 2 years and 6 months of postoperative follow-up and has good healing of intraoral surgical wounds and stable occlusion. Extraoral examination showed harmonic projection of the facial middle third, but still with excessive exposure of the sclera of the left eye due to the defect in the orbital floor. The surgical treatment of PJOF was possible by intraoral approach, even taking into account the limitations of the access and the complexity of the anatomy of the facial bones involved.

Intracranial extended Psammomatoid Juvenile Ossifying Fibroma: case report and systematic review

Psammomatoid Juvenile Ossifying Fibroma is an uncommon fibro-osseous neoplasm of aggressive but benign nature found in the younger age. Its aggressive path can lead to facial deformation, eye proptosis, and development of intracranial extensions leading to various neurological symptoms. A systematic review based by the MOOSE guideline in Medline, EMBASE and Lilacs, resulted in 23 reported cases of intracranial extended PJOF. Hence, we found it relevant to present a case report of a 15-year-old male with facial deformation and left eye proptosis absent of visual disturbances with PJOF. The lesion was present in the left anterior base of the skull and extended to the intra-orbital space and over the zygomatic arch. The diagnosis was only confirmed as PJOF by histopathological analysis of the completely resected lesion. Follow up visits documented unremarkable regression of the facial deformity and post-op images showed a completely resected lesion. Our case raises the need to be aware of this rare tumor that can be confused with a meningioma or other intracranial tumors.

A Case of Juvenile Psammomatoid Ossifying Fibroma in the Ethmoid Sinus

A Case of Juvenile Psammomatoid Ossifying Fibroma in the Ethmoid Sinus

Skull Base Juvenile Psammomatoid Ossifying Fibroma: Clinical Characteristics, Treatment, and Prognosis

The diagnosis and management for juvenile psammomatoid ossifying fibroma (JPOF) of the skull base are challenging, and clinical data are limited. A retrospective review of JPOF was performed, and the clinical characteristics, treatment strategy, and prognosis were analyzed. There were 23 patients pathologically confirmed with JPOF, most with JPOF located in the skull base area (19/23, 82.6%). Of those tumors, 43.5% presented with dura matter breakthrough. Most of the chief complaints were headache (n= 8, 34.8%) and visual impairment (n= 5, 21.7%). Most of the tumors were solid tumors with spherical appearance, frequently accompanied by cysts of various size (n= 14, 60.9%). Craniotomy, mostly via the frontal approach, was the most common approach in the present series, comprising 73.6% (17/23) of all cases. The endoscopic endonasal approach was performed in 6 cases (26.1%). In total, 62.5% of patients (15/23) underwent gross total resection, 8.7% of patients (2/23) underwent subtotal resection, and 26.1% of patients (6/23) underwent partial resection. After a mean follow up of 66.1 ± 36.1 months (range, 3-124), 3 patients (13.6%) suffered from tumor recurrence with a mean recurrence time of 13 months. The present series of skull base JPOFs showed that radical surgery combined with skull base reconstruction contributed to overall good prognosis. Further studies are needed to evaluate the long-term outcomes and to characterize its pathologic characteristics.

Juvenile Psammomatoid Ossifying Fibroma: a Case Report with 12 Months of Follow-Up

Juvenile psammomatoid ossifying fibroma (JPOF) is a type of ossifying fibroma with a unique histopathological pattern. We report a case of JPOF in a 12-year-old girl, causing an asymptomatic swelling in the posterior right mandibular region, which is a rare site for this tumor. The lesion was misdiagnosed as fibrous dysplasia, 5 years prior, at another hospital. On a physical examination, we also observed the absence of second premolars. There was no history of trauma, pain, or extraction of any teeth. An orthopantomograph revealed an extensive multilocular radiolucency occupying the entire mandibular body on the right side, with well-defined borders. An incisional biopsy was performed and the histopathological examination revealed psammomatoid ossifying fibroma as a final diagnosis. The complete excision of the tumor, osteotomy, and extraction of teeth 33, 34, 36, 75, 74, and 73 were performed, and the inferior alveolar nerve was preserved. There was no recurrence after a 12-month follow-up.

Juvenile psammomatoid ossifying fibroma of the ethmoid sinus: A rare case report

Juvenile psammomatoid ossifying fibroma (JPOF) is a rare, aggressive benign bony tumour that has been distinguished from a larger group of ossifying fibromas (OF) based on age of occurrence, most common site of involvement, and its clinical behaviour.We reported a case of asymptomatic 14 year old boy who had incidental finding of left ethmoidal bony mass on his CT brain imaging done for post traumatic cerebral concussion. The diagnosis of juvenile psammomatoid ossifying fibroma (JPOF) was made based on radiological and histological findings.International Journal of Human and Health Sciences Vol. 02 No. 04 October’18. Page : 228-231

Recurrent juvenile psammomatoid ossifying fibroma with secondary aneurysmal bone cyst of the maxilla: a case report and review of literature.

Juvenile ossifying fibroma is a benign fibro-osseous lesion commonly affecting the extra-gnathic craniofacial skeleton of the young individuals. The psammomatoid and trabecular variants are its two histopathological subtypes having distinctive clinico-pathological characteristics. Secondary aneurysmal bone cysts are frequently reported to arise in the pre-existing fibro-osseous lesions but rarely reported in the psammmomatoid variant of the juvenile ossifying fibroma. Such hybrid lesions, especially massive in size, tend to exhibit a greater aggressive growth potential and higher recurrence rate and mandate complete surgical removal of the lesion along with a long-term follow-up. The objective of this case report was to present a rare incident of recurrent psammomatoid ossifying fibroma associated with a secondary aneurysmal bone cyst in the maxillary jaw bone of a young patient and review the similar published reports in the English literature.

Juvenile Psammomatoid Ossifying Fibroma of Fronto-Ethmoid Complex Mimicking Fibrous Dysplasia: Case Report

Juvenile Psammomatoid Ossifying Fibromais a rare fibro-osseous tumor seen in children and adolescentand mostly arising from the cranio-facial bone.We report a case of 18-year-old boy who presented with diplopiaand progressive right fronto-orbital swelling. On plain radiograph and CT, it was diagnosed as fibrous dysplasiaand mucocele as differential diagnosis. The tumor was resected and histopathological examination showed psammomatoid features. Therefore, the diagnosis of Juvenile Psammomatoid Ossifying Fibroma must be based on both radiological and histopathological findings.

CT and MR imaging characteristics of histological subtypes of head and neck ossifying fibroma.

The current study aimed to assess CT and MRI characteristics of histological subtypes of head and neck ossifying fibroma (OF). 12 patients with histopathologically-proven head and neck OF were included in this study. Lesions were pathologically classified into three histological subtypes: eight cement-OFs (COFs), three juvenile psammomatoid OFs (JPOFs), and one juvenile trabecular OF (JTOF). All patients underwent CT examination, while seven also underwent MRI. Imaging characteristics were retrospectively assessed. On CT images, the lesion margins were well-defined in nine patients (75%) (seven COFs and two JPOFs), partially ill-defined in two (17%) (one COF and one JTOF), and ill-defined in one (8%) (one JPOF). The continuity of the eroded overlying bone cortex was maintained in nine patients (75%) (seven COFs and two JPOFs) but disrupted in three (25%) (one COF, one JPOF, one JTOF). With respect to lesion density, homogeneous ground-glass opacity was observed in five patients (42%) (five COFs), target-like appearance in three (25%) (two COFs, one JPOF), and mixture of hyper- and hypodense areas were observed in four (33%) (one COF, two JPOFs, one JTOF). MR signal intensity was homogeneous in two patients (29%) (two COFs) and heterogeneous in five (71%) (two COFs, two JPOFs, one JTOF). COFs tended to exhibit well-defined margins and preserved continuity of the overlying bone cortex. COFs were usually homogeneous, whereas JPOFs and JTOF were always heterogeneous. Target-like appearance was one of the characteristics of OFs, but it was observed in both COF and JPOF.