The Internet Journal of Otorhinolaryngology ISSN: 1528-8420

Sinonasal Ossifying Fibroma: A Study of

Six Cases and Review of Literature
Bhat K. Vikram MS, DNB, MNAMS (ORL - HNS) Associate Prof & PhD research scholar in
Otolaryngology, Head & Neck Surgery, Karnataka Institute of Medical Sciences Hubli India
S. G. Udayashankar MS, DLO Prof & Head of the department of Otolaryngology, Head &
Neck Surgery, Karnataka Institute of Medical Sciences Hubli India
Citation: B.K. Vikram, S.G. Udayashankar: Sinonasal Ossifying Fibroma: A Study of Six
Cases and Review of Literature. The Internet Journal of Otorhinolaryngology. 2006 Volume
4 Number 2
Keywords: ossifying fibroma, cementifying fibroma, jaw neoplasm, paranasal sinus
neoplasm

Abstract
Background: Ossifying fibroma is an uncommon tumor of the craniofacial skeleton
presenting in several variant histopathological subtypes. The overlapping clinical and
histopathological features of these subtypes have led to diagnostic dilemma and confusion.
Complete excision of this tumor has become a necessity since it is notorious for recurrence.
Aims: To study and compare the clinical profiles of various types of ossifying fibroma and
also the surgical approaches to the tumor. To discuss the diagnostic difficulties and
controversies associated with the tumor. Setting: Medical college referral hospital. Design:
Prospective descriptive study of 2 years duration. Methods: Planned surgical excision based
on criteria. Literature review with Medline search. Results: Six cases of different types of
ossifying fibroma were successfully treated by various surgical approaches. Thirty papers
related to diagnostic and treatment aspects of ossifying fibroma were studied. Eight papers
were found to be associated with some or the other controversy. Conclusion: The diagnostic
dilemma of ossifying fibroma can be overcome with a combination of clinical, radiological
and pathological criteria. Complete surgical excision of the tumor is possible when surgery is
based on preplanned criteria. Combination of 2 or more surgical approaches may be
necessary in many cases in order to ensure complete clearance and prevent recurrence of the
tumor. To avoid controversies, there should be a consensus on nomenclature and
classification of this uncommon tumor.

Key Messages
1. Diagnostic dilemma of ossifying fibroma can be overcome with a combination of
clinical, radiological and pathological criteria.
2. It is preferable to remove the tumor enmass, take multiple sections for
histopathological reporting to avoid missing the subtypes.
3. Complete surgical excision of the tumor is possible when surgery is based on
preplanned criteria.

Introduction
Ossifying fibroma is a destructive, deforming, slow growing, benign fibro-osseous tumor that
can occur almost anywhere in the facial skeleton. This uncommon tumor can present a
diagnostic dilemma for the clinician and the pathologist, owing to overlapping clinical and
histomorphologic features. Many synonymous nomenclatures exist for a single entity and the
controversy in classification and staging of the subtypes in the literature has added to the
confusion. Hence there is a need to highlight the points of controversy existing for this tumor
so that they could be avoided through a consensus in future. The tumor can produce sinus
obstruction, infection, facial deformity, proptosis and intracranial complications, even though
it can remain asymptomatic in the early stage. Therefore the tumor needs to be excised
completely in order to prevent recurrence. Complete excision is dependent on the correct
surgical approach. Hence we decided to base our surgeries on preplanned criteria to excise
the tumor completely and minimize recurrence. This paper has been presented with the
following objectives:
1. To present 6 cases of ossifying fibroma that were successfully treated by different
surgical approaches by us
2. To compare the clinical profiles of various types of ossifying fibroma and discuss the
controversies those exist for this tumor in literature.
3. To discuss the diagnostic dilemma associated with ossifying fibroma
4. To discuss the various surgical approaches to this uncommon tumor in the sinonasal
region.

Materials And Methods

This is a prospective descriptive study of all cases of sinonasal ossifying fibroma that were
diagnosed and treated in a period of 2 years in a medical college referral hospital catering to
both urban and rural population. Cases from all ages and both sexes were included. Cases
with tumor involving the orbit and the cranial cavity were also included. But those cases with
the tumor involving the lower jaw, recurrences of tumor and other conditions like fibrous
dysplasia were excluded. The diagnostic details of the patients are described in table 1.

Table 1: Diagnostic details of the individual cases

Investigations such as X-ray paranasal sinuses [PNS], CT scan PNS (Fig 1 to 3), diagnostic
nasal endoscopy (Fig 4) and biopsy were performed on all the patients. The classical
punched out appearance' and egg shell expansion' of the tumor can be appreciated in the
CT scan of case 2 (Fig 2).

Figure 1: CT Scan of the head (Axial section case 1) showing tumor mass in the medial part
of the right orbit

Figure 2: CT Scan of the head (Coronal section case 2) demonstrating punched out
appearance' and egg shell expansion' of the tumor

Figure 3: CT scan of the head (Coronal section case 4) showing intracranial extension of the
tumor

Figure 4: Nasal endoscopic view showing tumor mass in nasal cavity

Figure 5: Tumor mass excised in case 2.

We decided to plan our surgeries based on the following criteria:
1. To perform the least radical tumor resection possible

2. To excise the tumor in toto and also try to remove it enmass wherever possible.
3. To cause least damage to the surrounding structures that might affect the growth of
the craniofacial skeleton
4. To try to avoid an external scar, especially in pediatric patients
5. To try to prevent recurrence of tumor

Surgical approaches
The type of approach was decided by the following factors:
1. Age of the patient
2. Presence of complications
3. Histopathological type of tumor
4. Surgical criteria already mentioned
A Medline literature search with key words ossifying fibroma' was made to look for the
references on the nomenclature, classification, diagnostic details and treatment of ossifying
fibroma.

Results
Six cases of craniofacial ossifying fibroma were surgically treated by various surgical
approaches. All the 6 patients were operated under general anesthesia. Details of the surgeries
are given in table 2. The photograph of the tumor mass that was excised in case 2 is shown in
figure 5. In case 4, a dual approach had to be employed due to the significant intracranial
extension of the tumor. The help of a neurosurgeon was taken to excise the intracranial
portion of the tumor by a bifrontal craniotomy. The CSF leak was sealed and the dura was
repaired with fascia lata. The nasal part of the tumor was removed piecemeal by rigid nasal
endoscopy in the same sitting. Intraoperative bleeding was moderate in all cases except in
case 5 where blood transfusion had to be given. The excised mass in all cases was sent for
histopathological examination. An endoscopic assessment of the surgical cavity after tumor
excision was done in every case. No recurrence was observed clinically, endoscopically and
radiologically in any of the cases in a follow up period of one year.

Table 2: Surgical details of the individual cases

Thirty papers related to the diagnostic and treatment aspects of ossifying fibroma were
studied after the Medline search. Eight papers were found to be associated with some or the
other controversy. A detailed discussion of the diagnostic difficulties and controversies
associated with ossifying fibroma has been made in the discussion part of this paper.

Discussion
Ossifying fibroma is a relatively uncommon benign fibro-osseous tumor commonly affecting
the craniofacial region. The neoplasm closely resembles the non-neoplastic condition fibrous
dysplasia in many respects especially in histology. Hence many pathologists still consider it
to be a variant of fibrous dysplasia. The classification of ossifying fibroma with many
synonymous nomenclatures has been a matter of controversy. The various names associated
with ossifying fibroma are cementifying or cemento-ossifying fibroma, peripheral ossifying
fibroma, psammomatoid or juvenile ossifying fibroma and ossifying fibromyxoid tumor. While
some authors believe that all these names refer to the same tumor or its variants, others
believe that they are separate entities. The absence of typical histopathological features
unique to ossifying fibroma and its subtypes or variants has been one of the main reasons for
the controversies in reporting. Hybrid fibro-osseous lesions have also been described that
contained entities of aneurysmal bone cyst, ossifying fibroma and cementifying fibroma
within the same tumor mass.1 This suggests that simultaneous occurrence of multiple related
lesions is possible in the same tumor mass, which can further complicate the overall picture.
Hence multiple sections have to be taken for proper histopathological reporting.
The conventional ossifying fibroma usually presents as a solitary, slow growing, monostotic
tumor in the third and the fourth decades of life. It shows female predilection and the male to
female ratio is around 1:5.2 Although found predominantly in the mandible (75%), it can also
arise in the skull base and PNS.2 It has also been reported to occur in the temporal bone.3
The tumor is known to be more aggressive in young patients.3 In the jaws, the tumor shows
affinity for the molar area.4 In the mandible it can give rise to pathological fractures and
osteomyelitis in the long run if left untreated.4

Psammomatoid or juvenile ossifying fibroma is an aggressive variant of ossifying fibroma

usually seen in the pediatric age group that is histologically characterized by the formation of
Psammoma body islands. Juvenile ossifying fibroma has been further classified into 2
separate subtypes as psammomatoid and trabecular ossifying fibroma. 5 Psammomatoid
ossifying fibroma of the paranasal sinuses has been considered as an extragnathic variant of
cemento-ossifying fibroma by some authors.6 Some authors consider juvenile active ossifying
fibroma and Psammomatoid ossifying fibroma as separate entities. Juvenile or psammomatoid
ossifying fibroma has its peak incidence in the I and II decades of life with a female
predilection. Twenty one percent of the patients are older than 15 years and hence some
clinicians refuse to accept the word juvenile'. 7 The preferred sites are the maxilla, ethmoid
and the frontal bones.24The term active' refers to clinical aggressive behavior such as bone
erosion, soft tissue invasion and in rare instances death.8 The most common clinical
manifestation is proptosis.8 Other symptoms include nasal obstruction, headache, swelling
and rarely epistaxis. The most distinctive component in these lesions (Even though not
pathognomonic) is the presence of calcified ossicles containing osteocytes known as
psammomatoid bodies'. Because of a superficial resemblance between these ossicles and the
cementum spheres of the odontogenic ossifying fibroma, the lesion has sometimes been
mislabeled as cemento-ossifying fibroma, implying an odontogenic origin, which is rather
unlikely in extragnathic bone.9 Hence Psammomatoid bodies have been synonymously
referred to as cementicles' by some authors.1,9
Ossifying fibromyxoid tumor is a recently described mesenchymal neoplasm that can also
occur in the head and neck region. It was originally defined as a borderline or low-grade
malignant lesion.10 Some authors have considered it as a tumor of intermediate malignancy
that can also give rise to metastases.30 The tumor has been further classified into typical',
atypical' and malignant' subtypes.30 The tumor usually occurs in the deep subcutis of the
extremities, trunk and the head and neck region. Histologically the tumor is characterized by
the presence of a peripheral rim of lamellar bone within the collagenous capsule, a lacelike
and glomoid arrangement of tumor cells in a fibromyxoid background and also round tumor
cells that have a bland cytologic appearance.11 The histogenesis of this tumor is uncertain
although the preponderance of evidence suggests a Schwann cell origin.10
Cementifying fibroma is a distinctive jaw lesion and has its origin from the periodontal
membrane that has the characteristic feature of cementum formation.12,13 Hence it is also
known as periodontoma. The term cementifying fibroma has been applied to lesions
containing curvilinear trabeculae and spheroidal calcifications (Cementum).14 Cementifying
fibroma most frequently occurs in the III and IV decades primarily affects females and
typically involves the mandibular premolar-molar area.8 Some authors consider cementoossifying fibroma separately and refer them to lesions containing bone and cemental
tissues.14 These lesions are usually found in the tooth bearing areas of the jaws.5 Pediatric
dentists have referred to a reactive gingival nodule arising from the periodontal ligament as
peripheral ossifying fibroma that is probably similar to cementifying fibroma. 15
Interestingly, some authors have suggested that all the fibro-osseous lesions relating to
ossifying fibroma and its subtypes should be referred to as ossifying fibroma only since they
claim that there is no difference in behavior between the subtypes and the histological
designations are only academic.14
Even though some authors opine that fibrous dysplasia and ossifying fibroma cannot be
distinguished by microscopy due to histological overlap3,6 others opine that there are many

features like the absence of cementicles, lamellar trabeculae and osteoblasts to distinguish
them.1 The differentiation between the 2 entities is of great importance because of their
divergent clinical behavior. Fibrous dysplasia is often polyostotic whereas ossifying fibroma
is usually monostotic.17 Fibrous dysplasia is usually a self-limiting disease and therefore a
complete resection is unnecessary and in most instances impossible.18 Radiographically
ossifying fibromas are classically described as circumscribed unilocular lesions with a
surrounding rim of eggshell thin bone giving a punched out 'appearance.19,20 Irregular
thickening and thinning of the edges of this eggshell has been described as moth-eaten'
appearance.21 In contrast, fibrous dysplasia has a ground glass' appearance with indistinct
borders that blend imperceptibly into the adjacent bone without expansion or bone
destruction.22 But again some authors have also described ground glass' appearance in
juvenile active ossifying fibroma. 7,17 Hence CT scan with the bone window technique is the
most essential investigation since it defines the extent of the tumor and the destruction of the
surrounding tissues. The differential diagnosis of ossifying fibroma includes fibrous
dysplasia, giant cell tumor, giant cell reparative granuloma, aneurysmal bone cyst,
osteoblastoma and osteosarcoma.
The overall prognosis with most types of ossifying fibroma appears to be good. Despite their
tendency for local invasion and recurrence, there are no reported instances of metastatic
disease with the exception of certain subtypes of ossifying fibromyxoid tumor. The
development of aneurysmal bone cyst in psammomatoid ossifying fibroma has been
reported.6 Malignant transformation is very rare.2 Meningitis secondary to invasion into the
cranial cavity has been reported and rarely even death may occur.23 Surgery is the mainstay
of treatment for all types of ossifying fibroma. Growth of the facial skeleton is an important
consideration in the pediatric age group.24 Extensive tumors in this age group are more
difficult to manage since the tumors here tend to be more invasive, aggressive and have more
chances of recurrence. Mandibular tumors can be treated with conservative surgery but
aggressive surgery is warranted for midface and paranasal sinuses because of their more
aggressive behavior.2 Even though small tumors can be removed by simple curettage and
enucleation, they are better avoided for the fear of recurrence especially in juvenile ossifying
fibroma. One study showed a recurrence rate of 28% after curettage.14 Endoscopic removal
has also been tried successfully.28 But these conservative techniques and piecemeal
approaches could make histological interpretation more difficult, especially in cases of hybrid
lesions. Hence wherever possible, an open surgical technique is advised for adequate
visualization and complete excision.8 Various radical and semi radical resection procedures
have been tried. Fronto-parietal craniotomy with cranioplasty has been done for lesions of the
parietal bone.5 Similarly partial mandibulectomy followed by plate fixation has been done
for mandibular lesions.5 Extensive bilateral tumors require combined multiple approaches
whereby the tumor is excised portion by portion. Bilateral nasal tumors have been removed
by modified lateral rhinotomy with bilateral ethmoidectomy and sphenoidotomy.9 Larger
lesions with intracranial extension may require enbloc resection or craniofacial
resection.25,26
Surgical complications may include significant blood loss, requiring transfusion and loss of
vision.23,27Recurrences after surgery have been common and reported to be in the range of
30 to 56%.23Treatment of recurrences by revision surgery is difficult again in the pediatric
group. Radiotherapy has been proven to be ineffective and is contraindicated due to an
increased incidence of malignant transformation ranging from 0.4 to 44%9 with the exception
of certain subtypes of ossifying fibromyxoid tumor.

In our experience, the Lynch Howarth approach has been particularly useful to relieve
proptosis when the tumor was confined only to the medial portion of the orbit and the
ethmoid. The extended Caldwell Luc approach and sublabial approach are more suitable for
lesions involving the maxilla and the premaxilla and had the advantage of having no external
scar. Lateral rhinotomy approach gave the widest access and exposure to all the regions, but
with an external scar. Nasal endoscopic excision had the advantage of tumor excision under
direct vision and had no external scar. Hence it could be used as adjuvant for other
approaches especially when the tumor was extensive and had to be removed piecemeal. It
was also useful to assess the surgical cavity for bleeding and tumor remnants after the
excision of the tumor by any approach. But it had the disadvantage of piecemeal removal,
risk of recurrence and excessive time consumption for the procedure especially if it was the
sole approach employed for removal of the tumor. Hence it appeared to be more suitable for
early tumors especially in the pediatric patients where radical surgeries were not advisable.
Craniotomy approach had the maximum hospital stay due to higher morbidity.
This study has highlighted the versatile presentation and the heterogeneous nature of this
benign tumor in the sinonasal region. Larger comparative studies of longer duration and
follow up might be required to suggest an algorithm that could be useful in the decision
making process for this tumor. But since the tumor is uncommon, it is difficult to undertake
such prospective studies in a reasonable period of time.

Conclusion
Ossifying fibroma is a benign fibro-osseous tumor of the craniofacial region that is diagnosed
with a combination of clinical, radiological and pathological criteria. Due to the possibility of
the presence of hybrid lesions in this tumor, it is preferable to remove it enmass and take
multiple sections for histopathological reporting. This would avoid missing a particular
subtype of the tumor that might need a different surgical management. The classification of
the subtypes and the nomenclature of this tumor have remained controversial. There is a need
for consensus regarding the nomenclature and classification of this tumor. The surgical
approaches and techniques have also not been well defined especially in the young patients.
Complete surgical excision of the tumor is possible when surgery is based on preplanned
criteria. Six cases of different types of sinonasal ossifying fibroma managed successfully by
various surgical approaches have been presented.

Acknowledgements
The authors would kindly like to acknowledge the support given by the Department of
pathology, KIMS, Hubli in reviewing the histopathology slides, discussing them and
providing the photographs to us.

Correspondence to
Dr.Vikram Bhat K MS, DNB, MNAMS (ORL HNS) No: 4-64, Sagri Nole, Kunjibettu Post,
Udupi 576102, Karnataka, India Tel (Res): +918362370579 Cell: +919448658213 Email:
entvikram@rediffmail.com

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