Abstract
The ossifying fibroma is a rare fibro-osseous benign lesion of bone in the head and neck region. The mandible is the most common site reported followed by maxilla and other bones of the skull. A paediatric male presented with protrusion of the right eyeball for one-month duration. Further evaluation by diagnostic nasal endoscopy revealed a smooth mass confined to the superior and middle meatus on the right side. Computed tomography of paranasal sinus showed a large heterogenous bony lesion involving the ethmoid and sphenoid sinus and extending laterally into the orbit and superiorly into anterior skull base. Endoscopic biopsy was suggestive of ossifying fibroma. Transnasal endoscopic excision of the lesion was done and the patient is currently on follow-up. This case is reported for the rarity of presentation and the difficulties in management.
Keywords: Paediatric Ossifying Fibroma, Sinonasal Ossifying Fibroma, Transnasal Endoscopic Excision, Optic Nerve, Ethmoidal roof involvement, Lamina Papyracea
Introduction
The term fibro-osseous lesion describes a group of bony disorders(ranging from inflammation to neoplastic) that microscopically exhibit connective tissue matrix and trabeculae of bone. Ossifying Fibroma is a rare benign locally aggressive fibro-osseous neoplasm that commonly involves the mandible in the head and neck region. The ethmoidal localization of this lesion can be explained by incomplete migration and maturation of the periodontal membrane. This neoplasm is most common in females during the 3rd and 4th decades of life. [1] The clinical symptoms of this neoplasm depend on the site of origin and the extent of tumor. Ossifying fibroma is a slow-growing neoplasm that gradually increases in size within the normal bone that is generally replaced by a fibrocellular stoma which contains foci of ossification or mineralization. [2] Ossifying fibroma is histopathologically divided into 3 types which include juvenile psammomatous, juvenile trabecular, and cement ossifying fibroma [3, 4]. The juvenile psammomatous ossifying fibroma is usually described as an aggressive form. The surgical approach to excision of this neoplasm depends on the site and extent of the lesion. This case report discusses the rarity of presentation and management challenges faced by transnasal endoscopic excision.
Case Report
A 11-year-old male presented with complaints of bulging right eyeball for one month. He also had a right-sided nasal obstruction. There was no history of nasal discharge, headache, facial pain, watering of eyes, or loss of vision. There were no other associated comorbid conditions. On examination, there was proptosis in the right eye. Extraocular movements were full and free and vision was normal. On anterior rhinoscopy examination, a smooth bulge was seen in the right middle meatus which was firm in consistency and non-tender. Diagnostic nasal endoscopy showed a smooth globular pink mass which was involving the middle meatus and extended posteriorly up to the choana.
A contrast-enhanced computed tomography of the paranasal sinus was done which showed a heterogenous lesion of 5*4 cm extending laterally up to the optic nerve (abutting the optic nerve), eroding lamina papyracea, posteriorly up to the sphenoid sinus, and superiorly up to the roof of ethmoids and adherent to the anterior skull base (Figs. 1,2). The periorbita was found to be intact. Informed consent was obtained from the patient and his attendees regarding the procedure, complications of the surgery, and a second surgery if needed in their own language. Endoscopic transnasal multiple biopsies were taken from the mass which was suggestive of ossifying fibroma (Fig. 3). Later, the patient was planned for a definitive surgical procedure via the transnasal corridor under general anaesthesia. Piecemeal excision of the tumor was done. Tumour was found to be involving the lamina papyracea but the periorbita was intact. Tumour was found to be highly vascular and a diffuse ooze was seen during the excision of the tumor. The tumor was completely removed except the area near the carotico optic recess and the posterior ethmoids due to brisk bleeding which was controlled with pressure packing and cautery (Fig. 4). Since the tumor was adherent to the covering of the optic nerve near the posterior ethmoids, a conservative resection was done in this region [5]. The patient was discharged on the next day. The patient was regularly followed up and was asymptomatic. Follow-up computed tomography was done a year later which showed a minimal residual lesion in the posterior ethmoids (Fig. 5).
Fig. 1.
CECT Neck axial: showing heterogenous lesion in right nasal cavity extending to the roof sphenoid, Optic canal and pushing the lamina laterally
Fig. 2.
CECT neck coronal: Heterogenous lesion involving right nasal cavity pushing the lamina laterally and extending to the roof of ethmoids
Fig. 3.
HPE shows fibrocellular stroma with no evidence of atypia
Fig. 4.
Optic nerve and the near complete excision of the tumour near the region of Optico Carotid recess where brisk bleeding was noted
Fig. 5.
Post operative CT neck showing residual lesion in the roof of ethmoid
Discussion
Ossifying fibroma was first described by Montgomery in 1927. This tumor is generally asymptomatic until the tumor is large enough to produce a mass effect on the adjacent anatomical structure. Depending on the site of the lesion, it can present as persistent headache, facial pain, diplopia, proptosis, and loss of vision. This tumor is classified into conventional ossifying fibroma, aggressive psammomatoid ossifying fibroma (APOF), and cement ossifying fibroma (COF) [6]. The important differential diagnosis for this lesion is fibrous dysplasia which radiologically shows a diffuse margin compared to ossifying fibroma which has a well-defined unilocular lesion with cortical margins [7]. Ossifying fibroma has sharply defined calcifying spherulation which is an important differential finding not seen in fibrous dysplasia. Magnetic resonance imaging helps in differentiating the extent of the lesion in the orbit or intra cranial [8, 9].
The choice of treatment is complete excision which can be done by open approach or endonasal endoscopic technique. Open approaches have been reported including Caldwell-Luc operation when the tumor is located on the floor of the maxillary sinus, lateral rhinotomy with medial maxillectomy when the tumor is located in the medial wall of the maxillary sinus; transcranial/subcranial approach, and subfrontal approach. The advantages of the endonasal endoscopic approach include reduced morbidity with direct visualization and magnification resulting in decreased intraoperative trauma. In our case, this giant ossifying fibroma could be completely excised endoscopically except near the carotico optic recess where brisk bleeding was seen and the proximity of a normally functioning optic nerve and it was thought to be prudent not to proceed further as this had a risk of injury to the optic nerve. The tumor also was adherent to the skull base and posterior ethmoids that were exenterated as much as possible. Wang et al. had emphasized the importance of excising the outer lamella to avoid recurrence [10]. In our case, it was close to the optic nerve and the dura. Excision of the same in this region would have a possibility for optic nerve injury or CSF rhinorrhoea. Considering the age of the child and also normal vision, a careful exenteration of the lesion was done.
The advantage of non radical treatment in such a case can be a topic for discussion. Radical treatment will provide the clearance of the disease, but it should be carried out at the risk of potential damage to vital structures. The clinical progress of the tumor is unknown due to rarity of tumor, thus being a rare benign lesion, near complete resection was attempted without compromising optic nerve. Taking into account the younger age group of the patient, the authors made a judicious call of leaving a residual tissue near optic nerve where excessive bleeding was observed, eliminating the risk of vision loss for such benign lesion. The disadvantage of this treatment is that the patient should be followed up for a long time and assessment of the recurrence should be evaluated. There is possibility of the tumor recurrence causing direct compression of the optic nerve in the long run, which can be diagnosed at the earliest by close follow up and the resection can be done in second surgery preserving the vital structures. The decision should be tailor made and taken after thorough discussion with the patient and the relatives. This patient is under regular follow up. The child has been followed up for 2 years without any relapse of symptoms and with normal vision. This is authors modest experience on this debatable topic and the good prognosis in this study prompted us to share it to the medical fraternity.
Radiation therapy is not suggested because of the conversion to malignancy [11]. In this case, the lesion is very close to vital structures- the optic nerve, a conscious decision was taken not to proceed with aggressive surgery or radiation therapy.
Conclusion
Ossifying fibroma of the nose and paranasal sinus is a rare condition. Radiological and evaluation along with histopathology gives a definitive diagnosis. Complete surgical excision is the treatment of choice. But ossifying fibroma is a benign condition, using prudence while surgical resection, especially near the vital structures will avoid morbidity. Preoperative counselling to patients and attenders regarding the second stage procedure would be vital to manage such large tumors near vital structures.
Funding
None.
Declarations
Conflict of interest
The authors declare that they no conflict of interest.
Informed Consent
Obtained.
Footnotes
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Contributor Information
Sudhagar Eswaran, Email: zwaggerz@gmail.com.
Usha Djeamoorthy, Email: usha.djeamoorthy@gmail.com.
Prasanna Kumar Saravanam, Email: sprasannakumar10@gmail.com.
References
- 1.Salina AC, de Souza PM, da Costa Gadelha CM, Aguiar LB, de Castro JD, Barreto AR. Ossifying fibroma: an uncommon differential diagnosis for T2-hypointense sinonasal masses. Radiology case reports. 2017;12(2):313–317. doi: 10.1016/j.radcr.2017.03.019. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Speight PM, Carlos R. Maxillofacial fibro-osseous lesions. Curr Diagn Pathol. 2006;12(1):1. doi: 10.1016/j.cdip.2005.10.002. [DOI] [Google Scholar]
- 3.Commins DJ, Tolley NS, Milford CA. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses. J Laryngol Otol. 1998;112(10):964–968. doi: 10.1017/S0022215100142203. [DOI] [PubMed] [Google Scholar]
- 4.Mehta D, Clifton N, McClelland L, Jones NS. Paediatric fibro-osseous lesions of the nose and paranasal sinuses. Int J Pediatr Otorhinolaryngol. 2006;70(2):193–199. doi: 10.1016/j.ijporl.2005.09.031. [DOI] [PubMed] [Google Scholar]
- 5.Jurlina M, Skitarelić N, Passali D, Passali FM, Mladina R. Endonasal endoscopic resection of ossifying fibroma involving the ethmoid sinus, orbit and anterior skull base: case report and literature review. Acta Otorhinolaryngol Ital. 2016;36(2):144. doi: 10.14639/0392-100X-674. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Manes RP, Ryan MW, Batra PS, Mendelsohn D, Fang YV, Marple BF. Ossifying fibroma of the nose and paranasal sinuses. Int Forum Allergy Rhinol. 2013;3(2):161–168. doi: 10.1002/alr.21067. [DOI] [PubMed] [Google Scholar]
- 7.Gondivkar SM, Gadbail AR, Chole R, Parikh RV, Balsaraf S. Ossifying fibroma of the jaws: report of two cases and literature review. Oral Oncol. 2011;47(9):804–809. doi: 10.1016/j.oraloncology.2011.06.014. [DOI] [PubMed] [Google Scholar]
- 8.Mohsenifar Z, Nouhi S, Abbas FM, Farhadi S, Abedin B. Ossifying fibroma of the ethmoid sinus: report of a rare case and review of literature. J Res Med Sci. 2011;16(6):841. [PMC free article] [PubMed] [Google Scholar]
- 9.Lam SY, Ramli NM, Harikrishnan D, Sia SF, Pailoor J. Ossifying fibroma of the occipital bone—A case report and literature review. Eur J Radiol Extra. 2008;67(1):19–23. doi: 10.1016/j.ejrex.2008.04.021. [DOI] [Google Scholar]
- 10.Wang H, Sun X, Liu Q, Wang J, Wang D. Endoscopic resection of sinonasal ossifying fibroma: 31 cases report at an institution. Eur Arch Otorhinolaryngol. 2014;271(11):2975–2982. doi: 10.1007/s00405-014-2972-z. [DOI] [PubMed] [Google Scholar]
- 11.Alghonaim Y, ALHumaid SA, Arafat A. Aggressive ossifying fibroma of right ethmoidal sinus: A case report. Int J Surg Case Rep. 2018;53:513–516. doi: 10.1016/j.ijscr.2017.12.026. [DOI] [PMC free article] [PubMed] [Google Scholar]