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CASE REPORT CYST & TUMORS
Year : 2020  |  Volume : 10  |  Issue : 1  |  Page : 262-266

Aggressive ossifying fibroma of the maxilla


Department of Oral and Maxillofacial Surgery, JSS Dental College and Hospital, Mysore, Karnataka, India

Date of Submission13-Mar-2018
Date of Acceptance11-Feb-2020
Date of Web Publication8-Jun-2020

Correspondence Address:
Dr. Saubhik Dasukil
Department of Oral and Maxillofacial Surgery, JSS Dental College and Hospital, Mysore, Karnataka
India
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DOI: 10.4103/ams.ams_121_18

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  Abstract 


The aggressive ossifying fibroma is an uncommon benign fibro-osseous lesion which has been described in the literature under a variety of terms. This tumor is distinguished from standard ossifying fibroma based on its more clinically aggressive biological behavior, occurrence in children and young adults, and tendency to occur in different anatomic sites. We report a case of a 45-year-old female who presented with a unilateral swelling of the right middle face for 5 months. Clinical examination showed a mass extended over the right maxilla. Orthopantomogram and computed tomography scan were performed. Biopsy suggests a fibro-osseous lesion. The complete surgical excision of tumor was performed under local anesthesia. The histopathological examination revealed the diagnosis of an aggressive ossifying fibroma-trabeculae type. No recurrence was noted. Because of its aggressive and compressive nature, aggressive ossifying fibroma requires an early complete surgical excision. A long-term clinical and radiological surveillance is necessary to prevent recurrence.

Keywords: Aggressive ossifying fibroma, fibro-osseous tumor, maxilla, trabeculae variant


How to cite this article:
Shetty SK, Dasukil S, Arora G. Aggressive ossifying fibroma of the maxilla. Ann Maxillofac Surg 2020;10:262-6

How to cite this URL:
Shetty SK, Dasukil S, Arora G. Aggressive ossifying fibroma of the maxilla. Ann Maxillofac Surg [serial online] 2020 [cited 2020 Aug 10];10:262-6. Available from: http://www.amsjournal.com/text.asp?2020/10/1/262/286131




  Introduction Top


The 4th Edition of the World Health Organization (WHO) Classification of Odontogenic and Maxillofacial Bone Tumours 2017 included ossifying fibroma in the classification of odontogenic tumors. It was however discussed under “bone-related lesions” in 2005. The ossifying fibroma occurs in the jaws, perhaps exclusively in the jaws, which is neoplastic and histologically distinct from juvenile trabecular or juvenile psammomatoid ossifying fibromas (JPOFs). The tumor is of periodontal ligament origin and therefore odontogenic. The ossifying fibromas, including the cemento-ossifying fibroma, juvenile trabecular ossifying fibroma (JTOF), and JPOF, have similar and overlapping fibroosseous appearance histologically.[1]

Ossifying fibroma is characterized by fibrous connective tissue matrix with varying amount of osteoid, immature, and mature bone. The ossifying fibroma is subdivided into conventional and juvenile or aggressive clinicopathologic subtypes.

Aggressive or juvenile ossifying fibroma (JOF), a rare tumor entity occurs predominantly but not exclusively in children, with no gender predilection. It is usually benign and locally aggressive. The lesion is located mainly (85%) in facial bones, in some cases (12%) in calvarium and very seldom (3%) extracranially.[2] Ninety percent of the lesions located in the face region, involve the sinuses, mainly the maxillary antrum.[2] Mandibular lesions are seen in 10% of the cases.[2] It usually expands aggressively with cortical disruption and involvement of adjacent anatomical structures. Depending on the site of occurrence, symptoms such as pain, paresthesia, malocclusion, sinusitis, and proptosis can occur. Radiographically, lesion appears as radiopaque, radiolucent, or mixed radiopaque-radiolucent with a well-defined sclerotic border. Two forms of JOFs have now been distinguished: Psammomatoid JOF and trabecular JOF.

We report a case of aggressive ossifying fibroma because of its relative rarity, emphasizing its clinical, radiographic, histopathological features, and successful surgical management.


  Case Report Top


A 45-year-old female patient reported to our outpatient department with the chief complaint of swelling on the right side of the face for 5 months. She stated that the swelling was neither preceded nor followed by trauma, toothache, pain, discomfort, or fever. The swelling was fresh and started suddenly and spontaneously adjacent to the right nostril and had gradually grown to its present size and was asymptomatic. There was no significant medical or family history was found.

On general physical examination, the patient was conscious, cooperative, healthy, moderately built, and well nourished. All vitals were within the normal range. On extraoral examination, facial asymmetry noted with a single diffuse swelling on the right middle face [Figure 1]. The swelling measured approximately 2.5 cm × 3.5 cm in size and extended mediolaterally from right ala of the nose obliterating the nasolabial fold to 4.5 cm in front of the lobule of the right ear and superioinferiorly, extended from the right infraorbital region to corner of the mouth. The skin over the swelling and the surrounding area appeared normal. On palpation, the swelling was well circumscribed, nontender, hard in consistency, and fixed to the underlying bone. There was no evidence of paresthesia.
Figure 1: Preoperative extraoral profile

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Intraoral examination revealed a solitary, well-defined swelling obliterating the vestibule and extending from the right maxillary central incisor region (11) to the right maxillary second premolar region (15) posteriorly [Figure 2], associated with the swelling on palatal aspect. The surface was covered by normal mucosa. There was Grade III mobility and nontender on percussion with the associated teeth. Class III glass-ionomer cement restoration noted on 11 and 21. On palpation, the swelling was hard in consistency, nontender, causing the expansion of the buccal cortical plates.
Figure 2: Preoperative intraoral image

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Based on the history and findings of the clinical examination, a provisional diagnosis of a benign odontogenic neoplasm was made. The possibility of a radicular cyst, fibro-osseous lesion and a benign tumor of the bone was considered in the differential diagnosis.

Hematological findings were unremarkable, but there was elevation in serum alkaline phosphatase level.

Radiological investigations included intraoral periapical radiographs, orthopantomogram (OPG), and computed tomography (CT) scan (coronal and axial view). OPG revealed irregular, well-bordered mixed radiolucent-radiopaque lesion in the right maxilla extending from midline to the right molar region. Teeth displacement with root resorption noted (12,13,14). The CT scan showed well-defined soft-tissue solid hypodense lesions with osteolytic destruction involving the alveolar process of maxilla extending to the lower lateral aspect of the nasal cavity [Figure 3] and [Figure 4]. Intraorally, the lesion was extending into the buccal mucosa and measure 1.8 cm × 1.3 cm. Irregular erosion of the adjacent bone noted with intact dentin portion of the teeth. No extension to the adjacent right maxillary antrum. No calcification with in the lesion. Skin and subcutaneous tissue overlying the mass was intact [Figure 3].
Figure 3: Computed tomography scan (axial cut)

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Figure 4: Computed tomography scan (coronal cut)

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Under local anesthesia, a cravicular approach was made and surgical excision of the tumour was performed [Figure 5]. The tumor was completely removed followed by extraction of involved teeth (11,12,13,14). Right orbital floor, nasal floor, and palatal mucoperiosteum were preserved. Closure was done with resorbable suture. Postoperative recovery was uneventful [Figure 6]. Regular follow-up was done with every 2 weeks' interval.
Figure 5: Intraoperative image

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Figure 6: Postoperative extraoral profile

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Microscopic appearance

Hematoxylin- and eosin-stained section reveals epithelium and connective tissue stroma. Epithelium is stratified squamous parakeratinized in nature. Stroma is highly collagenous with dense collagen of bundles, plump proliferating fibroblasts and lots of areas of calcification resembling trabeculae of bone mostly and with few psammamatoid type of calcifications. Stroma also consists of numerous multinucleated giant cells, blood vessels, and inflammatory cells [Figure 7], [Figure 8], [Figure 9].
Figure 7: Microscopic view showing dense proliferation of fibro-collagenous tissue among which are seen areas of bony trabeculae lined by plump osteoblasts

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Figure 8: Proliferating fibroblastic tissue with blood vessels and osteoid showing plump osteoblastic rimming

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Figure 9: High-power view showing focus of proliferating fibroblastic cells with few specs of calcification (psammamatoid type)

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  Discussion Top


Fibro-osseous lesions of jaws are usually benign, asymptomatic, and tend to grow slowly. However, if the lesion occurs in a younger patient, especially below the age of 15 years, an unusual clinical presentation with apparent aggressive and destructive growth may be expected.[3]

The JOF is a rare fibro-osseous lesion that occurs mainly in the facial bones.[4] It is also called aggressive ossifying fibroma due to its aggressiveness and the high tendency to recur, unlike other fibro-osseous lesions. According to the WHO classification 2017, JOF has been recognized as a separate histopathological entity among the fibro-osseous group of lesions due to its distinct histological features.

JOF affects both males and females equally without any significant gender predilection. However, some researches showed that it is more common among men. In contrast, Johnson et al. stated that mandibular tumors are more frequently common in girls between the age of 5–11 or during the second to fourth decades of life.[5] In the present case report, a 45-year-old female was presented.

JOF commonly seen in the maxilla than in mandible and usually expand aggressively with cortical disruption and involvement of adjacent anatomical structures. Depending on the site of occurrence, symptoms such as pain, paresthesia, malocclusion, sinusitis, and proptosis can occur. In the present case, the patient presented with a solitary, well-defined swelling obliterating the vestibule and extending from the right maxillary central incisor region (11) to the right maxillary second premolar region (15) posteriorly [Figure 2], associated with the swelling on palatal aspect. There was Grade III mobility and nontender on percussion in associated teeth. On palpation, the swelling was hard in consistency and nontender.

The term Juvenile Aggressive Ossifying Fibroma (JAOF) describes two distinct histopathological entities of ossifying fibromas known as JPOF and JTOF as identified by El-Mofty in his study.[6]

JPOF has been reported under many names, including osteoid fibroma with atypical ossification” of the frontal sinus by Benjamins in 1938, psammomatoid fibroma of the nose and paranasal sinuses by Gogl in 1949, juvenile active ossifying fibroma by Johnson et al. in 1952, psammomatous desmo-osteoblastoma by Makek and juvenile psammomatoid cement-ossifying fibroma which was used in the 2005 WHO classification of odontogenic tumors and reclassified as a separate entity as JPOF under fibro-osseous lesion in the 2017 WHO classification of odontogenic tumors.[1] JPOF is commonly seen in children and young adults with a mean age 16–33 years and shows male predilection. JPOF commonly involves paranasal sinuses, orbit, and fronto-ethmoidal complex.

In 1965, Reed and Hagy suggested JTOF under the designation of JAOF,[4] Makek, in 1983,[7] published the largest series and he named these tumors as trabecular desmo-osteoblastoma. Subsequently, the tumor was named as JTOF in the 2017 WHO classification of odontogenic tumours. JTOF commonly affects maxilla than mandible. It is commonly seen in young individuals with a mean age 8–12 years and shows slight male predilection. JTOF is usually confused with osteosarcoma due to rapid growth and with fibrous dysplasia due to similar demographical and histopathological features.

According to the literature plane radiography, CT, magnetic resonant imaging can be used, but they are neither specific to JAOF nor diagnostic. The radiological features are variable depending on the site and the extent of the ossification. It can be radiolucent, mixed, or radiopaque, depending on the degree of calcification and extent of the cystic changes. Root displacement is common and resorption, though rare, can occur. In the present case, OPG revealed irregular, well-bordered mixed radiolucent-radiopaque lesion in the right maxilla extending from midline to the right molar region. Teeth displacement with root resorption noted (12,13,14). On CT JAOF appears as an expansive but circumscribed lesion surrounded by a thick shell of bone and internal content of varying density, while in our case, the CT scan showed a well-defined soft-tissue solid hypodense lesions with osteolytic destruction involving alveolar process of the maxilla extending to lower lateral aspect of nasal cavity [Figure 3] and [Figure 4].

Histopathologically, JOF appears in two distinct forms, psammomatoid and trabecular types. In our case, connective tissue stroma was highly collagenous with dense collagen bundles, plump proliferating fibroblasts, and areas of calcification resembling trabeculae of bone with few psammomatoid types of calcifications. Correlating with clinical findings with histological features was suggestive of trabeculated type, aggressive ossifying fibroma.

There is no definitive treatment of JOF. Radical resection, local excision conservatively or enucleation with curettage are among the treatment alternatives. Slootweg and Müller suggested that there were no differences between the cases that have limited surgical treatment and those with major surgery in terms of results, and they recommended conservative surgery.[7] On the other hand, Waldron et al. suggested that local excision and curettage should be a more preferable method and added that local surgical excision can be applied for recurrent tumor treatment. However, rate of recurrence after conservative treatment was reported in 30%–58% of cases. Incomplete resection causes recurrence in aggressive tumors. Therefore, some authors were recommended en block resection as an adequate treatment. Curettage together with peripheral osteotomy is suggested in prevalent or recurrent cases. Sarcomatous degeneration is reported to develop in lesions that have recurrence in long-term. However, in the present case, conservative treatment done includes complete surgical excision with curettage. No paresthesia and esthetically significant changes noted postoperatively. There was no recurrence or complication during 6 months of follow-up period [Figure 6].


  Conclusion Top


JOF is a rare fibroosseous entity characterized by its aggressive nature and high recurrence rate. Hence, it is essential to make an early diagnosis, provide the appropriate treatment and especially the long-term follow-up to prevent recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Wright JM, Vered M. Update from the 4th Edition of the World Health Organization classification of head and neck tumours: Odontogenic and maxillofacial bone tumors. Head Neck Pathol 2017;11:68-77.  Back to cited text no. 1
    
2.
Khoury NJ, Naffaa LN, Shabb NS, Haddad MC. Juvenile ossifying fibroma: CT and MR findings. Eur Radiol 2002;12 Suppl 3:S109-13.  Back to cited text no. 2
    
3.
Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1993;51:828-35.  Back to cited text no. 3
    
4.
MacDonald-Jankowski DS. Fibro-osseous lesions of the face and jaws. Clin Radiol 2004;59:11-25. Erratum in: Clin Radiol 2009;64:107.  Back to cited text no. 4
    
5.
Johnson LC, Yousefi M, Vinh TN, Heffner DK, Hyams VJ, Hartman KS. Juvenile active ossifying fibroma. Its nature, dynamics and origin. Acta Otolaryngol Suppl 1991;488:1-40.  Back to cited text no. 5
    
6.
El-Mofty S. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304.  Back to cited text no. 6
    
7.
Slootweg PJ, Müller H. Juvenile ossifying fibroma. Report of four cases. J Craniomaxillofac Surg 1990;18:125-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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