Mandible / maxilla – Benign tumors / tumor-like conditions: juvenile ossifying fibroma. Juvenile ossifying fibroma is a rare, benign fibro-osseous tumour. In the light of a clinical case, the authors review the diagnosis, treatment and histological. Introduction. Juvenile ossifying fibroma is a rare benign fibro-osseous lesion. It is characterized by the early age of onset, the localization of the tumor, the.
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Juvenile Aggressive Ossifying Fibroma of the Maxilla: A Case Report and Review of the Literature
Juvenile aggressive ossifying fibroma is a rare benign but locally aggressive tumor with high recurrent potentials. Juvenile aggressive ossifying fibroma poses diagnostic challenges because of ossifyung rapidly growing nature. A 7-years-old female child presented to the pediatric unit of our institution with a 9-month history of right maxillary tumor.
An initial diagnosis of Burkitt’s lymphoma was made and the child has several courses of chemotherapy without adequate histologic confirmation. The importance of early recognition and adequate management is highlighted. Juvenile aggressive ossifying fibroma JAOF is a rare benign but locally aggressive tumor with juvdnile recurrent potentials. JOAF is a distinct clinical entity, often confused with malignant conditions because of its rapidly progressive and osteolytic nature.
A 7-year old female child presented to the pediatric fiibroma of our institution with a 9-month history of right maxillary tumor. A clinical assessment of Burkitt’s lymphoma was made, and juveinle child was subjected to several courses of chemotherapy without adequate histologic confirmation.
The child was later referred to the maxillofacial unit after about 2 months of unsuccessful management by the pediatricians. On presentation, there was an expansile lesion, measuring about 12 cm by 8 cm in its greatest diameter, involving the right maxilla with proptosis of the right juevnile and moderate hypertelorism.
Vision was, however, intact in the associated right eye. The right nostril was completely obstructed by an exophytic growth, and thus patient had to breathe via the left side [ Figure 1 ]. Intra-orally, the lesion had involved the whole of the right hemipalatal region with a little extension to the contralateral side. The associated teeth had little or no displacement but with grade 1 mobility.
On general examination, there was mild palor and weight loss, but there was no difficulty in swallowing. Juvenile ossifying fibroma involving the right maxilla. Note proptosis of the right eye. Computerized tomographic findings showed a well-delineated osteolytic lesion, which had destroyed the right maxilla and with extension into the maxillary antrum [ Figure 2 ].
Incisional biopsy was done, and the histopathologic analysis confirmed a diagnosis of juvenile aggressive ossifying fibroma of the trabecular variety [ Figure 3 ]. Computerised tomographic scan of lesion. Coronal section showing well-circumscribed unilocular lesion. A hemi-maxillectomy was planned.
A Weber-Feguson incision was used to expose the lesion, which was excised. The lesion was well-defined, and some areas of cystic degeneration were encountered. The lesion was excised along the line of cleavage. Gross examination of the specimen showed multiple gray-white, soft to firm fragments measuring 5. The whole specimen was sent for histopathologic diagnosis. Slides from the initial biopsy were ossifyin and compared.
The initial histologic diagnosis of trabeculae juvenile ossifying fibroma was confirmed. The nasal lesion was found to be in isolation from the maxillary lesion and was excised, labeled separately, and sent for histopathologic diagnosis. The result came out as nasal polyp.
Juvenile Aggressive Ossifying Fibroma of the Maxilla: A Case Report and Review of the Literature
The patient was rehabilitated by fabrication of an obturator to cover the surgical defect. Post-operative recovery was uneventful, and patient was discharged after 1 week following surgery.
Juvenile aggressive ossifying fibroma JAOF is a relatively rare fibro-osseous lesion of the jaws characterized by the early age of onset usually less than 15location of the tumor, radiological appearance, and high recurrent potentials.
JOAF may present as one of two histologic variants: Juvenile psammomatoid ossifying fibroma and juvenile trabecular ossifying fibroma. There has been so much confusion about the terminology of fibro-osseous tumors. Some authors in the past have considered cemento-ossifying fibroma and ossifying fibroma adult or juvenile form to be two distinct histological entities on the basis that the former was an odontogenic tumor and the latter was not.
Thus, the dental origin of these tumors was excluded. Three forms of ossifying fibromas have now been distinguished: Classical ossifying fibroma, psammomatoid juvenile ossifying fibroma, and trabecular juvenile ossifying fibroma. Juvenile trabecular ossifying fibroma occurs in young patients with peaks occurring in those who are below 15 years of age and is usually characterized by rapid growth and a high tendency for recurrence.
Histologically, JOF is characterized by the presence of cellular fibrous stroma, immature bony strands, and cement particles. The juvenile psammomatoid variant is characterized by a proliferation of benign spindle-shaped fibroblastic cells with embedded mineralized structures, which may present with round to ovoid collections of bone with an osteoid rim. The most prominent condition in the differential diagnosis is fibrous dysplasia. The rapidity of growth, monostotic nature, and the well-delineated radiographic margins differentiates it from fibrous dysplasia.
Burkitt lymphoma should also be considered in the differential diagnosis of JAOF because of the similarity in the age and site of presentation, rapidity of growth, and radiolucent radiographic appearance. In addition, patients with Burkitt’s lymphoma may present with varying abdominal symptoms, which may range from splenomegally, hepatomegaly, or enlargement of both organs. Burkitt’s lymphoma usually presents with weight loss, and our patient also had some degree of weight loss.
The few clinical similarities, outlined above, between Burkitt’s lymphoma and the present case presented was, however, not enough evidence to have excluded other clinical entities in favor of Burkitt’s lymphoma. It is the standard of care to have a histopathologic confirmation for any suspected malignant lesion before administering any forms of chemotherapy because of the potential morbidity associated with these types of medications.
Juvenile aggressive ossifying fibroma is a rare clinical entity often misdiagnosed and mismanaged because of its rapidly progressive and osteolytic nature. The present case was the victim of such misdiagnosis.
The distinction between psammomatoid variant of juvenile ossifying fibroma, especially if it affects the paranasal sinuses and psammomatoid meningiomas, can be quite challenging.
Pathology Outlines – Benign tumors / tumor-like conditions: juvenile ossifying fibroma
JOAF is treated by surgical excision and may recur if local resection is not complete. Long-term follow-up is necessary owing to its locally aggressive nature and high recurrent potentials. A careful fibfoma of its clinical, radiographic, and histopathologic features is necessary to overcome the diagnostic and therapeutic challenges associated with this lesion.
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This article has been cited by other articles in PMC. Abstract Juvenile aggressive ossifying fibroma is a rare benign but locally aggressive tumor with high recurrent potentials. Aggressive, Juvenile, Ossifying fibroma.
Introduction Juvenile aggressive ossifying ossifyinh JAOF is a rare fibrmoa but locally aggressive tumor with high recurrent potentials. Case Report A 7-year old female child presented to the pediatric unit of our institution with a 9-month history of right maxillary tumor. Clinical examinations On presentation, there was an expansile lesion, measuring about 12 cm juvenioe 8 cm in its greatest diameter, involving the right maxilla with proptosis of the right eye and moderate hypertelorism.
Open in a separate window. Investigations Computerized tomographic findings showed a well-delineated osteolytic lesion, which had destroyed the right maxilla ossjfying with extension into the maxillary antrum [ Figure 2 ].
Juvenile ossifying fibroma with anastomosing trabeculae of immature woven bone. Treatment A hemi-maxillectomy was planned. Discussion Juvenile aggressive ossifying fibroma JAOF is a relatively rare fibro-osseous lesion of the jaws characterized by the early age of onset usually less than 15location of the tumor, radiological appearance, and high recurrent potentials.
Footnotes Source of Support: Juvenile ossifying fibroma of the mandible: J Oral Maxillofac Res. Paediatric fibro-osseous lesions of the nose and paranasal sinuses. Int J Pediatr Otorhinolaryngol. Psammomatoid and Trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathologic entities.
Fibrome cemento-ossificant ou dysplasia fibreuse? A clinic-pathologic study of sixty-four cases.
A commentary on the Second edition. Juvenile ossifying fibroma of the maxilla. Sinonasal psammomatoid ossifying fibromas: CT and MRI manifestations.
A clinicopathologic study of 28 cases. J Formos Med Assoc. Juvenile aggressive cement-ossifying fibroma: A case report and review of the literature. Juvenile ossifying fibroma of the jaw. Br J Oral Maxillofac Surg. Trabecular and psammomatoid juvenile ossifying fibroma of the skull base mimicking psammomatoid meningioma. A sino-orbital mass in a year old adolescent girl.
Arch Pathol Lab Med. An analysis of 33 cases with emphasis on histopathological aspects. J Oral Pathol Med. Aggressive psammomatoid ossifying fibromas of fibroa sinonasal region: A clinicopathologic study of a distinct group of fibro-osseous lesions.