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.

Analysis of Diagnosis and Treatment Strategies for Primary Juvenile Psammomatoid Ossifying Fibroma Complicated With Primary Aneurysmal Bone Cyst.

BACKGROUND: Juvenile Psammomatoid Ossifying Fibroma (JPOF) is a type of noncancerous bone tumor that usually affects adolescents in the craniomaxillofacial area. Clinical manifestations are usually symptoms caused by the tumor's invasive compression of surrounding tissues. Aneurysmal Bone Cyst (ABC) is also a benign bone tumor, and it typically occurs in long bones and the spine. Only 2% to 3% of cases occur in the head and neck. Due to the rarity of this combination of clinical cases, clinicians face difficulties in comprehensively understanding this complex lesion. Therefore, a comprehensive review of the clinical manifestations and characteristic imaging findings is necessary for surgeons. CASE PRESENTATIONS: On April 6, 2019, a 13-year-old boy presented with left maxillofacial bulge and pain for 1 month. Magnetic resonance imaging of the paranasal sinuses showed an irregular hive-like mass signal in the left maxillary sinus, and cystic changes with fluid levels were seen in the lesion. After the initial diagnosis of JPOF with primary ABC, we decided to perform a facial mid-facial resection of maxillary sinus tumor to remove the tumor tissue. Finally, after 3 recurrences and 4 operations, there was no tumor recurrence for 20 months after the last operation, and the patient was still under continuous follow-up. CONCLUSIONS: This case provided a reference for the diagnosis and treatment of JPOF combined with ABC. In particular, a new understanding of the association between the two diseases and the management of recurrence were proposed, which had the potential to improve clinical understanding of this complicated condition.

Spontaneous Regression of Ossifying Fibroma in the Oral and Maxillofacial Region: A Rare Case With 4-Year Follow-Up.

The case presented in this study is a rare instance of an ossifying fibroma (OF), a type of benign fibro-osseous lesions, spontaneously regressed without surgical intervention. A 9-year-old boy with an intraosseous lesion in the left maxilla was diagnosed as OF. The surgeon suggested surgical excision, but due to personal reasons, the patient and his parents deferred the surgery and opted for regular follow-up. During a 4-year follow-up, the surgeon found that the lesion had significantly decreased in size and the facial deformity had remitted. At the latest follow-up, the deformity of his left face became almost unnoticeable, and the lesion seemed to be subtle on cone-beam computed tomography images. This case highlights the possibility of spontaneous regression of OF. This phenomenon may occur due to the teeth eruption, which can lead to the formation of periodontal ligaments and engage the regression of OF.

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.

Synchronous Reconstruction of the Jaw Using a "One and a Half"-Barrel Fibular Bone Graft for Ossifying Fibroma Patients.

BACKGROUND: Ossifying fibroma is one of the common benign tumors that affect the appearance and functions of the jaw. Ossifying fibroma may exhibit a wide range of biological behaviors, leading to deformities involving the jaw and other secondary facial deformities. Hence, to improve the function of the jaw and the patient's general facial appearance (bearing in mind each patient's facial shape and, or appearance), the authors thus, however, used a ''one and a half"-barrel fibular bone graft to achieve the ideal height and radian of the bone graft. CASE PRESENTATION: Between July 2017 and January 2021, the authors retrospectively collected and analyzed clinical and surgical data from 39 patients who had undergone operations in our hospital. Twenty patients were operated on using our new surgical method, whereas 19 patients received conventional or debulking operation. Clinical factors associated with the operation were assessed, including classification of the jaw defects, perioperative complications, and postoperative outcomes. RESULTS: All the flaps ultimately survived. According to the postoperative satisfaction survey, patients who underwent reconstruction were satisfied with their postoperative facial appearance, with an average of 8.5 out of 10. Based on the preoperative clinical data, 26 patients had suitable bone grafts for dentures to improve their oral function. CONCLUSIONS: A ''one and a half''-barrel fibular bone graft effectively improves the facial appearance of patients and as well as provides an appropriate height and radian for the bone graft.

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.

Endoscopic Resection of a Massive Ossifying Fibroma in the Sinonasal Cavity.

ABSTRACT: An ossifying fibroma (OF) is a type of benign fibro-osteoma that rarely involves the sinonasal cavity. Recent developments in endoscopic sinus surgery allow the removal of large benign tumors from the nasal cavity and sinuses. Here, the athors report the case of a 48-year-old female who underwent endoscopic sinus surgery under general anesthesia to completely remove a large OF involving the sphenoid sinus and nasal cavity. No recurrence was noted during the recent 3-year follow-up. Endoscopic resection of OFs is an excellent choice for very experienced surgeons, affording the advantages of direct observation as well as visual enhancement and magnification, thus reducing intra- and post-operative morbidity.

Juvenile psammomatoid ossifying fibroma.

Juvenile psammomatoid ossifying fibroma (JPOF) is an uncommon benign and locally aggressive tumor. We report an unusual head tumour with extremely rare extensiveness and aggressivness. The patient was 18-year-old female with three-day-lasting headache and repetitive oral bleeding. Computed tomography revealed a large, well-circumscribed, expansile mass occupying ethmoid cells, nasal cavities and ventral part of the sphenoid sinus, with extention into the anterior cranial fossa. Pterional craniotomy was carried out. On one-year follow-up recurrence of the lesion was identified and the second surgery was performed. The lesion is under supervision now, due to incomplete removal.

Fibro-Osseous Lesions Of The Craniofacial Complex In Horses: 30 Cases (2001-2019).

OBJECTIVE: To describe the presentation, diagnosis, treatment, and outcome for horses with fibro-osseous lesions of the craniofacial complex. STUDY DESIGN: Retrospective multicenter case series. ANIMALS: Thirty horses evaluated for fibro-osseous lesions of the skull from January 1, 2001 through December 31, 2019 in four centers. METHODS: Medical records were reviewed for signalment, clinical presentation, histological and diagnostic imaging findings, treatment instituted, and outcome. Long-term outcome information was obtained by owner questionnaire or the medical record. RESULTS: Diagnoses included ossifying fibroma in 20 of 30 horses, osteoma in eight of 30 horses, and fibrous dysplasia in two of 30 horses. Twelve of 30 lesions were diagnosed in horses <1 year old, and 20 of 30 lesions originated from the rostral mandible. The most common treatment was rostral mandibulectomy. Recurrence was not reported after complete excision. Incomplete excision was confirmed in eight horses (four ossifying fibromas, three osteomas, and one fibrous dysplasia), and follow-up information was available for seven horses. Recurrence occurred in one horse, while six horses had long-term resolution of clinical signs. Prognosis for survival and return to use was excellent in 23 horses with long-term follow-up. CONCLUSION: Fibro-osseous lesions were uncommon in this multicenter study; they were most commonly diagnosed in young animals and most frequently affected the rostral mandible. Long-term survival was excellent. CLINICAL SIGNIFICANCE: The definitive diagnosis of fibro-osseous lesions of the craniofacial complex in horses is made from results of histopathology and cannot be determined on the basis of clinical presentation alone. Surgical excision is indicated, and prognosis can be favorable even when complete surgical margins are not obtained.

Concomitant bilateral mandibular cemento-ossifying fibroma and cementoblastoma: case report of an extremely rare occurrence.

BACKGROUND: Cemento-ossifying fibroma (COF) and cementoblastoma (CB) are rare benign odontogenic tumors with a predilection for the mandible. Cemento-ossifying fibroma is a fibro-osseous lesion that originates in the tooth bearing areas of jaw and shows cementum-like tissue in a fibrotic stroma. Cementoblastoma is classically related to roots of teeth with the presence of calcified cementum-like material. To date, only a single case of concomitant unilateral COF and CB has been reported in the literature. CASE PRESENTATION: We present an unusual case of a 37-year-old female who presented with two discrete bilateral swellings in the right and left mandible for 10 years. The larger tumor involved the left posterior mandible with extension anteriorly to the left and right anterior mandibles, and the smaller tumor was present in right posterior mandible. Radiology revealed two distinct lesions involving both sides of mandible. Histopathological examination showed characteristic features of cemento-ossifying fibroma in sections of the larger tumor and cementoblastoma in sections of smaller tumor. CONCLUSION: This case shows the very unique bilateral co-existence of COF and CB, the second case reported in literature to date.

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.

Use of the Cover-Lifting Technique in Mandibular Cemento-Ossifying Fibroma Excision to Preserve the Inferior Alveolar Nerve.

Cemento-ossifying fibroma (also known as ossifying fibroma or cementifying fibroma) is a benign osteogenic neoplasm. Pain and paresthesia are rarely associated with cemento-ossifying fibroma; thus, nerves must be preserved during excision. With the advent of computer-aided techniques, the use of virtual surgical planning and a customized template can improve the precision of resection and reconstruction, reduce operating time, and improve postoperative outcomes. In this report, we describe a case of cemento-ossifying fibroma in a female patient who underwent segmental mandibulectomy and reconstruction with an iliac bone graft. Additionally, we describe a simple and effective way to preserve the inferior alveolar nerve.

A Clinical Audit of Histopathologically Diagnosed Ossifying Fibroma of the Jaws in a Nigerian Population.

BACKGROUND: Ossifying fibroma (OF) is a benign tumor of the jaws, which belongs to the group of fibro-osseous lesions, and exhibits slowly progressive growth leading to jaw expansion with well-defined borders. OBJECTIVE: To evaluate the clinical and radiological characteristics of OFs and their treatment. MATERIALS AND METHODS: A 5-year retrospective study was done. The case notes of subjects were retrieved, analyzed, and evaluated. Relevant information about patients was categorized into bio-data, clinical, and radiological. The diagnosis of lesion was made histopathologically by the oral pathologist. RESULTS: Forty- five patients' case notes were retrieved and analyzed and the patients' age ranged from 2 to 70 years (mean 29.4 ± 16.9). Majority of the patients (55.5%; P = 0.23) were 10-29 years. There were 16 (35.6%) males and 29 (64.4%) females giving a male: female ratio of 1:1.8 (P = 0.01). The cross-sectional dimension of the tumors measured vertically and horizontally ranges from 1 cm2 to 420 cm2 (mean = 54.7 cm2 ± 82.9). There were 60% in the maxilla and 40% in the mandible (P = 0.014). Most of the lesions (68.9%; P = 0.01) showed patchy opacity and cotton wool appearances. Majority of the lesions were extirpated by enucleation alone (P = 0.01). CONCLUSION: The prevalence of OF among the fibro-osseous lesions was 51.0% while against other benign lesions, it was 16.0%. In conclusion, contrary to most other reports across the globe, this lesion in southeast Nigeria occurred more frequently in the maxilla than mandible.

[A clinical observation of laryngeal ossifying fibromyxoid tumor]. / Nablyudenie ossifitsiruyushchei fibromiksoidnoi opukholi gortani.

A rare observation of ossifying fibromyxoid tumor of the larynx in a 23-year-old young man is presented. Surgical intervention, performed in two stages, provided a positive result. With dynamic observation of this patient for three years, continued growth and tumor recurrence were not observed. Due to the fact that ossifying fibromyxoid tumor is rare, differs in the complexity of differential diagnosis and an uncertain prognosis of the course of the disease, registration and dispensary observation of patients with this pathology is necessary.

Juvenile Psammomatoid Ossifying Fibroma of the Orbital Roof: A Rare Cause of Proptosis among Children.

INTRODUCTION: Juvenile psammomatoid ossifying fibroma (JPOF) is a rare bone tumor characterized by a predilection for the sinonasal region and a tendency to affect younger patients, with a potential for aggressive growth and high recurrence (30-56%). JPOF warrants complete surgical resection to avoid recurrence. CASE PRESENTATION: In this article, we report a young boy who presented with unilateral prop-tosis with an expansile bony tumor with ground glass appearance involving the left frontal bone and orbital roof on his images. Complete surgical resection was done, and histopathological examination revealed JPOF with abundant psammomatoid bodies. DISCUSSION: This patient is a rare case of neurocranial JOPF and adds new features to the typical features already described for JPOF.

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.

The use of 3D printing in shared decision making for a juvenile aggressive ossifying fibroma in a pediatric patient.

Juvenile aggressive ossifying fibromas (JAOF) are rare, typically benign pediatric tumors that are locally aggressive and have high recurrence rates. A 7-year old male presented with a palatal mass and a 3D printed model was created and used as a visual aide to highlight the importance of management in terms of functional, cosmetic, and disease-free outcomes with the family. The patient ultimately underwent successful enucleation with final pathology consistent with JAOF. To our knowledge, this is the first description of the use of 3D printing to help in the shared decision-making process for the treatment of this aggressive tumor.

Craniofacial Approaches to Pediatric Orbital Tumors.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. A total of 9 patients were identified between the years 2008 to 2017. Pathologies included rhabdomyosarcoma, juvenile ossifying fibroma, optic nerve glioma, and retinoblastoma. Surgical approaches to the orbit included supraorbital bar osteotomy (transcranial) or lateral orbitotomy (extracranial). Surgical team members included neurosurgery, craniofacial surgery, and ophthalmology. This study aims to review the role of surgery in management as well as the specific indications for performing transcranial or extracranial osteotomies. It also highlights the excellent access achieved with the use of these osteotomies in certain cases, especially when compared with transconjunctival or transcutaneous approaches.

Management of an extensive equine juvenile ossifying fibroma by rostral mandibulectomy and reconstruction of the mandibular symphysis using String of Pearls plates with cortical and cancellous bone autografts.

OBJECTIVE: To describe a surgical technique for rostral mandibulectomy and reconstruction of the mandibular symphysis. STUDY DESIGN: Case report. ANIMAL: One 5-month-old warmblood cross filly. METHODS: A preoperative diagnosis of a juvenile ossifying fibroma was made on the basis of clinical appearance and was later confirmed via histology. The tumor was large, rapidly growing and extending to the caudal aspect of the mandibular symphysis. Computed tomography was performed for surgical planning. The tumor was excised by performing a mandibulectomy caudal to the symphysis. The mandibular symphysis was reconstructed with String of Pearls (SOP) plates. Cortex screws placed in lag fashion, a cortical bone graft from one of the mandibles and a cancellous graft harvested from the tuber coxae. RESULTS: At 9 months of age there was no sign of tumor reoccurrence, the implants were removed because of infection and the mandible had formed a new stable symphysis. The horse was able to prehend food and masticate normally. The owner was pleased with the cosmetic and functional outcome. CONCLUSION: The described surgical technique is a viable option for mandibulectomy and reconstruction of the mandibular symphysis. The technique provided adequate stability for healing to occur in the face of infection. CLINICAL SIGNIFICANCE: Neoplasia and other conditions affecting the entire mandibular symphysis can be treated by complete surgical excision and reconstruction.

Recurrent benign ossifying fibroma of the orbit - clinical, radiological profile and management options in a child.

Ossifying fibroma (OF) is a benign fibro-osseous neoplasm which may be mistaken for other similar lesions due to overlapping clinical and radiological features. We report a 5-year-old male child with recurrent benign OF of the orbit. The child had two episodes of recurrence in a span of 18 months. Computed tomography (CT) of orbit showed a large, lobulated expansile fibro-osseous lesion involving the greater wing of sphenoid and orbital roof without intracranial extension. An excisional biopsy was done though an orbital approach. Histopathology showed fibroblast rich stroma with bony trabeculae. Osteoblastic rimming without any mitotic activity was suggestive of juvenile OF. The child developed a recurrence 6 months following the initial excision, and surgical excision was done by a neurosurgeon using a bicoronal approach. The patient had another recurrence after 1 year requiring further surgery. At 2-year follow up there was no recurrence. Juvenile OF is the most aggressive variant that commonly occurs in children, the other benign fibro-osseous lesions being fibrous dysplasias (FDs), osseous dysplasias, and familial gigantiform cementomas.