Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: two distinct clinicopathologic entities

The term juvenile ossifying fibroma is used in the literature in naming 2 microscopically distinct fibro-osseous lesions of the craniofacial skeleton. One is characterized by small uniform spherical ossicles resembling psammoma bodies (psammomatoid juvenile ossifying fibroma). The other is distinguished by trabeculae of fibrillary osteoid and woven bone (trabecular juvenile ossifying fibroma). Three new cases of each type are reported, and the literature is extensively reviewed for published cases of these 2 entities.Psammomatoid juvenile ossifying fibroma is reported more commonly than trabecular juvenile ossifying fibroma. It affects patients from a wider age range (3 months to 72 years) and an older mean age range (16-33 years) as compared with 2 to 12 years and 8(1/2) to 12 years, respectively, for trabecular juvenile ossifying fibroma. In both types there is a slight male predominance and the lesions are unencapsulated and tend to infiltrate adjacent bone. A significant difference between the 2 tumors is their site of occurrence. Although psammomatoid juvenile ossifying fibroma occurs predominantly in the sinonasal and orbital bones, trabecular juvenile ossifying fibroma predominantly affects the jaws. Aggressive growth occurs in some-but not all-cases of both types. Such behavior may be related to younger patient age and the concurrent development of aneurysmal bone cysts, which is seen more frequently in psammomatoid juvenile ossifying fibroma.This study demonstrates that not only histologic but also demographic and clinical differences between psammomatoid juvenile ossifying fibroma and trabecular juvenile ossifying fibroma warrant their classification as 2 distinct clinicopathologic entities.     

Juvenile ossifying fibroma of maxilla

Juvenile ossifying fibroma is a benign, but potentially aggressive, fibroosseous tumour of the craniofacial bones. The authors describe a case of a juvenile ossifying fibroma of maxilla presenting in a 14-year-old girl and review the histology, clinical behavior, and management of this uncommon but disfiguring lesion.     

Management of juvenile ossifying fibroma in the maxilla and mandible

We present three cases with juvenile ossifying fibroma. Two occurring in the maxilla, and one in the mandible. All three cases presented with a major swelling in the face. After clinical and radiological evaluation the lesions were surgically excised and sent for histopathological evaluation. Two histological types of juvenile ossifying fibroma were found, the psammatous type in two cases and the trabecular pattern in one case. Although juvenile ossifying fibroma is an uncommon clinical entity, its aggressive local behaviour and high recurrence rate mean that it is important to make an early diagnosis. It is also important to apply the appropriate treatment and to follow-up the patient closely over the long term. This report describes the diagnosis and treatment of juvenile ossifying fibroma in the maxilla and the mandible. It also emphasizes the importance of considering the less aggressive options as a first line of treatment before choosing the aggressive approach when dealing with children.     

Psammomatoid juvenile ossifying fibroma: case study and a review

Background

The group of fibro-osseous lesions from the maxillofacial region is very heterogeneous, but what they all have in common is the substitution of normal bone by fibroblasts with the consequent formation of collagen fibers, as well as substitution by different types of mineralized tissues which may be similar to bone or cement. Within this group of lesions, the juvenile ossifying fibroma is found, considered a rare and recurrent benign fibro-osseous neoplasia. The term juvenile ossifying fibroma has been used in the literature to describe two histopathological variations of conventional ossifying fibroma. These variations are trabecular juvenile ossifying fibroma and psammomatoid juvenile ossifying fibroma. Psammomatoid juvenile ossifying fibroma is an uncommon bone-forming neoplasm with aggressive local growth. Diagnostic of this lesion occurs after the correlation of clinical, imaging, and histopathological findings. Proposed treatments range from enucleation and curettage to resection of the tumor.

Objectives

The present article has as its objectives to report an uncommon case of a 4-year-old male patient treated by conservative approach and revise the literature on juvenile ossifying fibroma.

Conclusions

Psammomatoid juvenile ossifying fibroma, for its being very uncommon, warrants further investigation in order to establish the best treatment, principally in children, with a view to minimizing mutilating treatments. In the case examined, a conservative treatment was chosen, where the surgeon established curette and cryotherapy, and the reintegration of the child in his social environment, without relapse during the first year of therapy.     

Trabecular juvenile ossifying fibroma with aneurysmal bone cyst: a rare presentation

Juvenile ossifying fibroma is an uncommon, benign, bone-forming neoplasm that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation, and potential behavior. It mainly occurs in juveniles and has a slight male predilection and more aggressive behavior than a common ossifying fibroma. There are 2 distinct histopathological variants of this lesion: (1) psammomatoid pattern and (2) trabecular pattern. An aneurysmal bone cyst may occur in association with other bone lesions, such as fibrous dysplasia, ossifying fibroma, and giant cell lesion. The clinical management and prognosis of juvenile ossifying fibroma is somewhat uncertain, and this tumor has high rates of recurrence. Such behavior may be related to younger patient age and the concurrent development of aneurysmal bone cyst. The purpose of this paper was to report a case of trabecular juvenile ossifying fibroma in a 9-year-old girl associated with an aneurysmal bone cyst, presenting an aggressive behavior, and causing significant facial asymmetry.     

Central ossifying fibroma: an analysis of 20 cases and review of the literature.

Central ossifying fibroma is the most common benign fibro-osseous neoplasm of the jaw. It is not easy to diagnose ossifying fibroma because its clinical, radiographic, and histologic criteria often overlaps with other fibro-osseous lesions. Based on the analysis of 20 cases, this article describes all the manifestations of central ossifying fibroma and some of the differences with the so-called juvenile active ossifying fibroma. Photographs and radiographs of 2 cases showing a number of all the clinical and radiographic features of central ossifying fibroma are presented. The surgical procedure of these cases is also illustrated.     

Juvenile ossifying fibroma of the jaw

Juvenile ossifying fibroma is a benign lesion with aggressive local growth. It is similar to an ossifying fibroma, although it is more aggressive and of earlier onset. Some cases of ossifying fibroma are part of a syndrome that includes hyperparathyroidism (caused by adenoma or carcinoma) and renal lesions, possibly caused by a recently discovered genetic mutation. These tumours must be treated by radical excision, to prevent recurrence. We report a case of recurrent juvenile ossifying fibroma, in an 18-year-old girl. The treatment was radical en-bloc resection, which was reconstructed at the same operation with an iliac crest bone graft. After one year, there has been satisfactory integration of the graft and the teeth have been implanted.     

Juvenile Ossifying Fibroma of Mandible: A Case Report

Fibro osseous lesions are benign mesenchymal skeletal tumours in which mineralized tissue, blood vessels and giant cells, in varied proportions replace normal bone. Included in this group are fibrous dysplasia (FD), cherubism, ossifying fibroma and osteoblastoma. Although fibro osseous lesions have similar histologic and radiographic features, they may exhibit a wide range of biological behaviours. Because the histologic appearance does not predict the rate of growth or prognosis, treatment is based on the clinical and biologic behaviour of the tumour. The term “Juvenile active ossifying fibroma”(JAOF) has been given to a less common, more aggressive variant of the central ossifying fibroma which occurs primarily in children and young adults. This lesion has been described in literature under variety of terms like “aggressive psammomatoid ossifying fibroma” or “juvenile ossifying fibroma”. We are presenting a case of Juvenile aggressive ossifying fibroma in a 17 years old girl who reported to our institute.     

Juvenile ossifying fibroma of the midface

Juvenile ossifying fibroma is an unusual maxillofacial fibro-osseous lesion characterized by cell-rich osteoid strands. A 7-year-old girl presenting with a massive juvenile ossifying fibroma of the maxilla resulting in facial deformity, orbital displacement, and extension into the anterior skull base is discussed. The importance of combining clinical, radiographic, and histopathological findings for the diagnosis and management of the tumor is discussed.     

Juvenile ossifying fibroma of the maxilla

Juvenile ossifying fibroma is a rare fibro-osseous neoplasm in young children. This lesion is locally aggressive and spreads quickly, and because it has a very high recurrence rate complete excision is essential. Reported here is a case of a massive juvenile ossifying fibroma of the maxilla in an 11-year-old male child. A titanium mesh was used to reconstruct the facial contour after a left total maxillectomy, achieving a satisfactory facial appearance.     

Juvenile ossifying fibroma. An analysis of 33 cases with emphasis on histopathological aspects

Juvenile ossifying fibroma (JOF) is a maxillofacial fibro-osseous lesion that may show cell-rich osteoid strands or psammoma-like ossicles. Whether both types are variants of a single entity or different lesions under the same diagnostic label is a subject of debate. This problem was investigated by analyzing a series of 33 patients with lesions having one or both of the above histological appearances. It was concluded that osteoid strands define a unique fibro-osseous lesion but that lesions with psammoma-like ossicles fall within the morphological spectrum of cemento-ossifying fibroma. Therefore the term juvenile ossifying fibroma should be reserved for the lesion with the osteoid strands.     

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 sublabial approach for anterior skull base juvenile ossifying fibroma

Juvenile ossifying fibroma is a rare fibro-osseous neoplasm seen in the pediatric age group of 5 to 15 years. It has been recognized as a separate histopathologic entity among the fibro-osseous group of lesions. Occasionally, it may grow aggressively and extend to involve the orbits and skull base, resulting in serious cosmetic and functional problems. We present a case of a juvenile ossifying fibroma arising below the skull base of a 9-year-old boy. This case demonstrates that cemento-ossifying fibroma in case of maxilla grow to considerable size before causing symptoms because of the remodeling of the surrounding bony compartment and intraoral sublabial approach can be used to excise lesion completely even at the skull base.     

Juvenile Ossifying Fibroma of the Maxilla: A Case Report

Ossifying fibroma is a benign neoplasm of the bone, usually involving the posterior tooth bearing area of the mandible, predominantly seen in females in 2nd–4th decade of life with 5:1 prediliction. Fibro-osseous lesions other than FD seem to arise from the periodontal membrane. These lesions are usually asymptomatic, well defined clinically and radiologically amenable for enucleation. Fibro-osseous lesions of the jaws, including Juvenile Ossifying Fibroma (JOF), pose diagnostic and therapeutic difficulties due to their clinical, radiological and histological variability. Ossifying fibromas which appear as fast growing mass between 5 and 15 years of age, radiologically well bordered, and consistent with ossifying fibroma histologically, are referred as juvenile (aggressive) ossifying fibroma. We report a case of JOF of left side of the maxilla in an 11 year old girl which is an uncommon site of occurrence.     

Spontaneous regeneration after juvenile ossifying fibroma resection: a case report

A case of unusual bone regeneration after resection of a juvenile ossifying fibroma (JOF) is presented. Secondary mandibular reconstruction with autogenous grafts was delayed due to the rapid bone formation. To the best of our knowledge there are no reports of this unusual response following JOF resection.     

Juvenile trabecular ossifying fibroma of the mandible

Juvenile Ossifying Fibroma (JOF) is a rare fibro-osseus neoplasm in young children. This lesion is locally aggressive and spreads quickly. It is usually asymptomatic, achieving a large size. Owing to its aggressive behavior and high recurrence rate, early detection and complete surgical excision are essential. This report details the diagnosis and treatment of a 3-year-old girl presenting with a mandibular swelling that was subsequently determined to be juvenile ossifying fibroma.     

Juvenile ossifying fibroma of the jaws and paranasal sinuses: a systematic review of the cases reported in the literature

The aim was to compare clinical and radiological features of the two juvenile ossifying fibroma (JOF) variants, trabecular (JTOF) and juvenile psammomatoid ossifying fibroma (JPOF). An electronic search was undertaken in March 2019. Eligibility criteria included publications having sufficient clinical, radiological, and histological information to confirm the diagnosis. A total of 185 publications and 491 cases were included. Most JOFs, including both variants, showed bone expansion, were painless, presented no cortical perforation and no secondary aneurysmal bone cyst, did not cause tooth root resorption, and had a mixed unilocular radiodensity appearance and well-defined limits on radiological examination. Patients with JPOF were on average older than those with JTOF. Enucleation and curettage was associated with a considerably high recurrence rate, regardless of the anatomical location or variant type of the lesion. Enucleation followed by either curettage or peripheral osteotomy showed lower recurrence rates than enucleation only. When resection was performed, only one case of JTOF presented recurrence. In conclusion, JOF lesions presented high rates of recurrence after treatment by curettage and enucleation only. Although surgical resection of JOFs resulted in the virtual absence of recurrence, enucleation followed by peripheral osteotomy/curettage should be the treatment of choice for both JOF variants to avoid the disfigurement usually associated with surgical resection.     

Aneurysmal Bone Cyst of the Jaws: Clinicopathological Study

Aim

The aim of the present study was to analyze clinical, radiological and histopathological features of aneurysmal bone cysts (ABCs) of jaws.

Materials and Methods

Archival data from 2009 to 2012 present in the Oral Pathology Department was retrieved and clinicopathological features of all the cases which had been previously diagnosed as ABC were analyzed in detail.

Results

Seven cases ranging in age from 10 to 50 years were included, in which maximum (5/7) cases were below 20 years. Ratio of presentation of lesion in male to female and in maxilla to mandible was 3:6 and 1:6 respectively. Swelling and pain were the most common presenting features. Variable presentation of the lesion was observed radiographically; although multilocular, well defined, bone expansion and perforation were the most common observations. Histopathological analysis revealed association of one case with ossifying fibroma and two cases with trabecular variety of juvenile ossifying fibroma. Predominance of solid variety was noted and other features like stroma, giant cells, nature of blood vessels, bone destruction and perforation and presence of any osteoid or calcified material was also accounted for. The current study showed association of two cases with trabecular variety of juvenile ossifying fibroma, which is a rare finding.

Conclusion

ABCs of jaws, thus have varying patterns of presentation which are diagnostically challenging. A thorough examination of the incisional/excisional tissue is thus required to confirm the association with any other lesion which will affect the treatment plan for the patient.     

Fibro-osseous lesions of the jaw and facial bones: a clinico-histologic-radiologic study of 138 cases

A clinical-histologic-radiologic study of 138 cases of fibro-osseous lesions of the jaw and facial bones is presented. In this series, all were monostotic lesions except 8 polyostotic fibrous dysplasia. In the 130 monostotic lesions, 59 occurred in maxilla, 66 in mandible, 4 in zygoma and one in ethmoid bone. Clinically, a painless enlargement of the involved bone was the main sign. Histologically, these lesions were composed of proliferating fibrous connective tissues and various amounts of calcified tissues, but different lesion has different feature. Radiologically, fibrous dysplasia was characterized by lesions with diffuse blending. The other three tumors including ossifying fibroma, cementifying fibroma and fibro-osteoma were all represented by lesions with well delineated borders. In this article, Diagnosis of the fibro-osseous lesions and some questions about fibro-osteoma of the jaw and facial bones and osteofibrous dysplasia of long bones are discussed.     

Juvenile ossifying fibroma: a case study

We present the case of an 8-year-old patient with a juvenile ossifying fibroma in the right mandibular angle and a radicular cyst in the left mandibular angle, which produced a major swelling at the level of the mandibular angles and prevented the correct eruption of the lower first molars. After the clinical and radiological (panoramic X-ray and dental scan of the mandible), the corresponding surgical treatment (cystectomy and remodelling) and histopathological study were carried out. Although juvenile ossifying fibroma is an uncommon clinical entity, its aggressive local behaviour and high recurrence rate mean that it is important to make an early diagnosis, apply the appropriate treatment and, especially, follow the patient up over the long term.