6360abefb0d6371309cc9857
Abstract
Ossifying fibroma is a rare, benign fibro-osseous tumor that primarily affects the jawbones, particularly the mandible. It is characterized by the replacement of normal bone with fibrous tissue and abnormal bone formation. Although slow-growing, ossifying fibromas can cause significant functional and aesthetic disturbances if left untreated.
We report a case of an 18-year-old male patient who presented with chronic nasal obstruction. physical examination revealed a hard mass filling the entire right nasal cavity. A CT scan of the facial sinuses helped orient the diagnosis of ossifying fibroma, which was only confirmed by pathological examination of the surgical specimen. The patient underwent surgical excision of the lesion and postoperative follow-up showed no signs of recurrence.
This case highlights the importance of early diagnosis and appropriate surgical management of ossifying fibroma to prevent complications and ensure optimal functional and aesthetic outcomes. A multidisciplinary approach involving oral and maxillofacial surgeons, radiologists and pathologists is crucial for accurate diagnosis and treatment planning.
Keywords: Ossifying fibroma; Fibro-osseous tumor; CT scan; Jawbones; Maxillofacial surgeons
esions cover a wide range of proliferative bone diseases, each type presenting a specific morphological model. The ossifying fibroma (OF) is a rare and benign fibro-osseous lesion that occurs mainly in children and young adolescents1.
Classified as an odontogenic tumor, this lesion has no
particular clinical specificities and the use of standard and modern imaging is
essential. It can be found in diverse anatomical locations as nasal cavity,
paranasal sinuses and orbit. Histologically, a number of subtypes have been
described: juvenile, aggressive, active, psammomata’s and cementifying2-3.
It’s aggressive in nature with a high risk of recurrence if
not totally resected, so it requires early radiological detection and surgical
intervention1.
We report a case of cemento-ossifying fibroma of the right
maxillary and ethmoidal sinuses treated surgically.
Case Presentation
We report the case of an 18-year-old patient with no
specific pathological history, admitted to our department for a chronic right
nasal obstruction that appeared 8 months ago, associated with purulent
rhinorrhea and homolateral chronic tearing. The patient was in good general
condition.
On rhinoscopy, a hard mass was noted in the right nasal
fossa, occupying the entire nasal cavity, modifying the normal architecture of
the nasal cavity, exerting a mass effect on the nasal septum and extending
forward to the level of the nasal vestibule. The nasopharynx remains free (Figure
1).
In the left nasal fossa, there was a huge deviation of the
nasal septum, preventing exploration. The rest of the ENT examination was
normal.
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Figure 1: Rhinoscopy images
showing the mass occupying the entire right nasal fossa (blue arrows) and
reaching as far as the nasal vestibule (orange arrow). The nasopharynx remains
free (green arrow)
A CT scan of the facial sinuses revealed a bony lesional
process of heterogeneous density, measuring 5cm in long axis and filling the
entire right nasal cavity (Figure 2).
It developed at the expense of the medial wall of the right
maxillary sinus, the right middle and inferior turbinate’s and the right
anterior ethmoid. It extends into the maxillary alveolar process and is
responsible for a leftward deviation of the nasal septum (Figures 3 and 4).
Figure 2: Coronal section CT
scan showing the expansive process filling the entire right nasal fossa (black
arrow)
Figure 3: Axial section CT
scan showing invasion of the medial wall of the right maxillary sinus, the
right middle and inferior turbinate’s (yellow arrow) and the right anterior
ethmoid (blue arrow)
Figure 4: Axial section CT
scan showing the mass effect on the nasal septum exerted by the mass
A biopsy of the lesion by endonasal approach was performed,
showing an aspect in favor of an ossifying fibroma (Figure 5).
Figure 5: Histological section
indicative of an ossifying fibroma
Following a multidisciplinary consultation meeting, an
external surgical approach (lateral rhinotomy) was decided after obtaining
patient agreement (Figure 5).
The surgery was performed under General anesthesia with
endotracheal intubation. Patient placed in a supine position with the head
elevated (15-30 degrees) to reduce venous congestion. A vertical incision is
made along the lateral aspect of the nose. Extends from the medial canthus to
the level of the nasal ala. The incision is deepened through the subcutaneous
tissue. The periosteum is elevated to expose the nasal bones and maxilla. The
angular artery and facial vessels are cauterized. The lateral nasal wall and
anterior maxillary bone are exposed. The nasal bones, maxillary and ethmoid
sinus walls are carefully removed for access. The ossifying fibroma showed as
well-demarcated with a bony consistency. The lesion is identified and separated
from normal sinus structures. Using curettes and osteotomes, the ossified mass
is excised. The lesion is removed carefully to avoid damaging adjacent
structures specially the orbit and skull base. Bleeding is controlled with
bipolar cautery (Figure 6).
In postoperative care, the patient stayed 72 hours for
observation. And was under Antibiotics (Amoxicillin/clavulanic acid) to prevent
infection. Analgesics for pain control. Nasal saline irrigation for sinus care.
The follow-up was based on regular nasal endoscopy, facial
inspection to check for recurrence and imaging (CT-scan) at 6 and 12 months.
This confirmed total resection of the tumor with no signs of recurrence or post
operative complications specially no signs of orbital injury or breach of the
skull base.
Figure 6: Per-operative image
showing lateral rhinotomy procedure
Discussion
In 1872, Manes was the first to identify OF as a rare benign bone lesion4. “Ossifying fibroma” has been used in 1927. A more precise definition was later established by the World Health Organization (WHO) in 1971, which categorized cement-containing lesions into ossifying fibroma, fibrous dysplasia, cementing fibroma and cemento-ossifying fibroma. Benign fibro-osseous lesions are also classified as osteogenic neoplasms and non-neoplastic bone lesions5,6.
Makek's examination of 86 cases revealed an age range from 3 to 49 years, with a mean age of 17.7 years and a slight male predominance7
Most ossifying fibromas are found in the mandible or maxilla. Also, it affects the maxillary and ethmoid sinus; the frontal sinus and sphenoid sinus are rarely affected. Initial symptoms often involve bone expansion, frequently observed. Symptoms may include proptosis, nasal obstruction, headache, facial swelling, pain and recurrent sinus infections. Depending on the location of the disease, the circumstances in which it is discovered may vary. Paranasal sinus involvement may cause exophthalmos, reduced visual acuity or nasal obstruction. Large lesions may extend to the skull base8,9
Cement-ossifying fibroma is believed to originate in peri dental tissue, which has the potential to form cement and bone tissue. In the case of the mandibular region, this would be the result of a tooth extraction; in the case of the other regions, it would be of ectopic origin10.
CT and MRI scans are the tools used to diagnose these lesions. The density of fibrous tissue and bone is the main determinant of the radiological appearance of these lesions. The center of advanced OF is filled with mature bone tissue, while in earlier stages, the center is usually soft and fibrous. Usually on CT, it's a thick, well-bounded mass of bone density, the fibrous center is of low density. On MRI, the calcified periphery of lamellar bone is hypo signal in T1 and T2, with homogeneous or heterogeneous enhancement. Also, calcifications can happen and usually have hypointense signals on T211,12.
The main differential diagnoses on imaging are osteoblastoma, osteoma, meningioma and fibrous dysplasia. The many subtypes described in histology are indistinguishable on imaging13.
Histologically, the boundaries between damaged and healthy tissue. Conjunctive tissue is rich in fibroblasts and mineralized substance. Mineralized tissue is composed of a variable combination of mature and immature bone with osteoblast trabeculae excavated by osteocytic lodges14.
e treatment is surgical
with complete resection to avoid recurrence. In the absence of complete removal
of the lesion, recurrence may occur between 6 months and 7 years after
resection15.
The strategy for managing OF
differs according to the location of the lesion and its current stage of
expansion. A radical surgical resection is the preferred treatment modality for
lesions involving the sinonasal tract orbit and skull base. External approaches
offer better tumor control and exposure than endoscopic approaches. External
approaches include lateral rhinotomy, sublabial approach and craniofacial
resection. There are some inconveniences associated with external approaches,
notably the resulting facial scarring, affecting facial growth and symmetry16,17
References
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Osborn AG. Handbook of
neuroradiology: brain and skull. St Louis: Mosby edit 1996.
10.
Abou-Elhamd KE. Frontal
sinus cementifying ossifying fibroma. Saudi Med J 2005;26:470-472.
14.
Tchane IB, Adjibabi W, Biaou O, et al. Le fibrome
ce´mento-ossifiant : a` propos de deux cas. Rev Stomatol Chir
Maxillofac 2005;106:30-32.
15.
MacDonald-Jankowski DS. Fibro-osseous lesions of
the face and jaws. Clin Radiol 2004;59:11-25.
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