Juvenile Psammomatoid Ossifying Fibroma Research Articles

Juvenile Psammomatoid Ossifying Fibroma with Visual Disturbance: A Case Report with Imaging Features

Juvenile psammomatoid ossifying fibroma (JPOF) of the sphenoid sinus is a rare subtype of ossifying fibroma of the sinonasal cavity and facial bone in young adults. Computed tomographic (CT) and magnetic resonance (MR) imaging features of JPOF have been reported, but to our knowledge, positron emission tomography (PET) findings have not been described. We present a 19-year-old woman with right visual disturbance whom we diagnosed with JPOF and describe imaging findings in her case. CT revealed a well-circumscribed fibro-osseous mass surrounding the right optic canal, with expansile, mixed soft tissue and thick bone density. MR imaging showed low signal intensity in the mass on both T(1)- and T(2)-weighted images. [(18)F]fluorodeoxyglucose ([(18)F]FDG) and [(11)C]methyl-L-methionine ([(11)C]Met) PET/CT showed abnormal uptake in the lesion, with standardized uptake values (SUV) of 6.2 ([(18)F]FDG) and 4.6 ([(11)C]Met). Familiarity with the imaging features of this rare disease aids its differentiation from other more familiar lesions to permit appropriately aggressive therapy and improve prognosis.

Psammomatoid juvenile ossifying fibroma of the mandible with secondary aneurysmal bone cyst: A case report

Juvenile psammomatoid ossifying fibroma (JPOF) is a rare, slowly progressive tumor of the extragnathic craniofacial bones, with a tendency towards locally aggressive behavior and recurrence. The pathognomonic histopathologic feature is the presence of spherical ossicles, which are similar to psammoma bodies. Very few cases in association with secondary aneurysmal bone cyst (ABC) formation have been reported in the literature. Treatment consists of complete surgical removal and incomplete excision has been associated with a high local recurrence rate. The prognosis is good because malignant change and metastasis have not been reported. Authors report a case of JPOF of the mandible with secondary ABC in a 17-year-old male patient.

Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review

Psammomatoid Juvenile Ossifying Fibroma (PJOF) is an uncommon neoplasm that is distinguished from the adult variant of ossifying fibromas on the basis of age, site, clinical behavior and microscopic appearance. It is seen in children younger than 15 years of age, and the maxilla is more commonly involved than the mandible. PJOF may exhibit erosion and invasion of the surrounding bone accompanied by rapid enlargement. PJOF can be distinguished from other maxillofacial fibro-osseous lesions by its tendency to recur and its clinical mimicry of malignant bone tumors, but some clinical and histological features of PJOF overlap with the other fibro-osseous lesions as well. We report a case of a 15-year-old female patient with a painless apparently slow growing tumor in the left malar region, which occupied almost the whole of the left maxillary sinus, eroding the orbital border of the sinus.

PSAMMOMATOID OSSIFYING FIBROMA-AN UNUSAL PRESENTATION

Psammomatoid juvenile ossifying fibroma (PsJOF) is a well defined clinical and histological entity with earlier onset at (childhood or adolescence) classified under the broad category of fibro-osseous lesion. Histological PsJOF is one of the entity of juvenile ossifying fibroma (JOF) characterized by small spherical ossicles resembling psammoma bodies and the other with trabecular or fibrillar osteoid and woven bone which is termed as trabecular juvenile ossifying fibroma (TrJOF). This case of PsJOF involved maxilla, premaxilla, antrum, lateral wall of nose on left side of face. The size, extent and aggressive behavior of the tumor which has lead to facial disfigurement and difficulty in breathing, inability to speak, eat and drink is rare in literature. The treatment done by resection with safe margins is adequate with no recurrence as patient is under follow up for more than one year.

Diagnostic Challenges of Benign Fibro-Osseous Lesions and Psammomatous Meningiomas of the Craniofacial Region: A Comparative Review of their Clinico-pathological Features

Conventional ossifying fibroma (COF), juvenile psammomatoid ossifying fibroma (JPOF), juvenile trabecular ossifying fibroma (JTOF), fibrous dysplasia (FD), cemento-osseous dysplasia (COD) and psammomatous meningioma (PM) share overlapping clinico-pathologic characteristics. This can be diagnostically challenging for pathologists. Although remarkable progress has been made over the years using ancillary studies like immunohistochemistry and molecular cytogenetics to distinguish histologically similar diseases; such diagnostic aids are yet to be successfully employed within this group of lesions. The implication is that pathologists have to rely heavily on traditional H&E stained sections in differentiating these lesions. It is important to make the distinction because of differences in their clinical behavior, modes of treatment and prognosis. In this article, the clinico-pathologic features of each entity are reviewed.

Transcranial resection of a large sinonasal juvenile psammomatoid ossifying fibroma

Juvenile psammomatoid ossifying fibroma (JPOF) is a benign but potentially locally aggressive fibroosseous lesion predominantly arising in the paranasal sinuses in children and young adults. Intracranial extension is rare but occurs sometimes. In such cases, tumor resection may often require the combination of neurosurgical and facial approaches. Histological diagnosis remains a challenge because the lesion can be easily mistaken for another fibroosseous lesion or for a meningioma. We report the case of a 12-year-old boy with a JPOF arising from the right paranasal sinuses and extension towards the anterior skull base and the orbit. Despite the tumor had eroded through nasal septum, medial orbit wall, and right maxilla, it could be entirely removed performing an extended frontobasal approach via a bifrontoorbital craniotomy, obviating the need for a transfacial approach. Radiologically and histologically, the lesion could be mistaken either for a meningioma or another type of ossifying fibroma. Histological aspects and alternative surgical approaches to these rare entities are discussed.

Psammomatoid Juvenile Ossifying Fibroma of the Mandible – A Histochemical insight!

Juvenile ossifying fibroma (JOF) are fibro osseous lesions that mostly occur in children. Although a benign entity, JOF is known to be locally aggressive and has a high tendency to reoccur. Two distinctive microscopic patterns have been described; a trabecular variant and a Psammomatoid variant. This latter variant is predominantly a craniofacial lesion and occurs rarely in the jaws. We report a rare case of Psammomatoid Juvenile Ossifying that occurred in the mandible of a young boy along with review of the relevant literature. In addition, the pathology of the lesion was also analyzed with picrosirus red stain and polarizing microscopy.

Juvenile psammomatoid ossifying fibroma of the neurocranium

Juvenile psammomatoid ossifying fibroma (JPOF) is a benign fibroosseous lesion predominantly arising within the paranasal sinuses in children and young adults. Neurocranial occurrence is exceedingly rare and a location within the neurocranial portion of the temporal bone has not been described. The authors report on one case of sinonasal JPOF secondarily extending into the cranial cavity and three cases primarily affecting the neurocranial bones to increase clinical awareness of this uncommon tumor, which may be easily mistaken for meningioma. Moreover, the absence of activating missense mutations of the GNAS1 gene in two cases strongly argues against a relationship between JPOF and fibrous dysplasia.