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CASE REPORT
Year : 2016  |  Volume : 4  |  Issue : 2  |  Page : 53-58

Massive maxillary juvenile ossifying fibroma: Report of three cases and review of literature


Department of Surgery/Dental and Maxillofacial Surgery, Usmanu Danfodiyo University, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication16-Aug-2018

Correspondence Address:
Dr. Adebayo Aremu Ibikunle
Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njecp.njecp_3_16

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  Abstract 


Juvenile ossifying fibroma (JOF) is a rare fibro-osseous lesion, similar to the conventional ossifying fibroma in many respects. However, unlike the conventional ossifying fibroma, it exhibits more aggressiveness and a high tendency to recur; therefore, it may be confused with malignant lesions. Consequently, it poses significant diagnostic, therapeutic, and rehabilitative challenges. Early recognition and subsequent treatment with complete surgical excision of the tumor is advocated because of its relatively rapid growth, associated morbidities, and tendency to recur. Three cases of massive JOF of the maxilla with attendant morbidities are here presented. The diagnostic challenges, airway management challenges, and varied surgical approaches to each were highlighted. There is a paucity of literature on JOF; hence, clinicians and pathologists alike often find it demanding to arrive at a diagnosis.

Keywords: Fibro-osseous lesions, juvenile ossifying fibroma, maxillectomy


How to cite this article:
Ibikunle AA, Taiwo AO, Braimah RO. Massive maxillary juvenile ossifying fibroma: Report of three cases and review of literature. Niger J Exp Clin Biosci 2016;4:53-8

How to cite this URL:
Ibikunle AA, Taiwo AO, Braimah RO. Massive maxillary juvenile ossifying fibroma: Report of three cases and review of literature. Niger J Exp Clin Biosci [serial online] 2016 [cited 2018 Nov 12];4:53-8. Available from: http://www.njecbonline.org/text.asp?2016/4/2/53/239101




  Introduction Top


Fibro-osseous lesions are a group of reactive, developmental, or neoplastic bone disorders characterized by replacement of bone by fibrous tissue and/or cementum-like tissue.[1] Ossifying fibroma is a fibro-osseous bone lesion which is often highly cellular and contains varying amounts of fibrous and calcified tissues.[2] While different types exist, ossifying fibroma and fibrous dysplasia are the most commonly occurring types.[3] Different types of ossifying fibroma have been discussed in literature; these include the central and peripheral types and the juvenile and conventional types.[4],[5] In contrast to the central and peripheral types, juvenile ossifying fibromas (JOFs) are found in the first and second decades of life, are more aggressive, have a higher tendency to recur, and may, therefore, be confused with malignant lesions.[5],[6] Two variants have been described, namely the trabecular and psammomatoid JOF; the mean age of diagnosis for the two is 11 years and 22 years, respectively.[7]

As much as 85% of JOF cases involve the facial bones with the maxilla/sino-antral skeleton being more commonly affected than the other facial bones.[8] Radiographically, the tumor exhibits varying patterns depending on the degree of mineralization. It appears well demarcated from surrounding tissues, although it may exhibit some degrees of bone disruption and occlusal disharmony.[5],[8] Histologically, it is characterized by the presence of bony strands, fibrous stroma, cementum-like particles, and varying amounts of cellular components.[5] The psammomatoid and trabeculae types differ histologically and even clinically.[9] The psammomatoid types contain spherical ossicles that appear similar to psammoma bodies, while the trabeculae type contains osteoid and woven bone.[9] JOF is rare and has a higher recurrence rate than the conventional ossifying fibroma, ranging from 30% to 58%.[7],[9] The mainstay of treatment is surgery.

We report three cases of JOF of the maxilla.


  Case Reports Top


Case A 10–year-old male with massive progressive maxillary tumor of 1-year duration with provisional diagnosis of rhabdomyosarcoma refractory to series of chemotherapy was referred to our unit for further evaluation.

Previous history was suggestive of weight loss, spontaneous bleeding, and pus discharge. On clinical examination, the patient was asthenic and appeared cachectic. There was facial asymmetry and dental anarchy secondary to a large well-circumscribed hard lesion measuring about 20 by 30 cm from the right maxilla to involve the midline, palate pharynx, and base of the skull with compression of the tongue and mandible. This resulted in narrowing of the tongue space [Figure 1]. A palatal ulceration which was consistent with dental traumatic ulcer and prominent superficial veins on the facial skin were observed.
Figure 1: Preoperative extraoral view of Case 1, note the prominent facial vessel (arrow)

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Subsequent investigations of full blood count, alkaline phosphatase, electrolyte, and urea were within normal range excluding the packed cell volume which was 25%. Accordingly, he was transfused. Radiographic findings revealed a circumscribed radiolucency with patchy radiopacities dispersed throughout the lesion. Histopathology revealed multiple fibroblastic spindle-shaped cells with bland cytological features and reactive, immature, bony trabeculae in a background of proliferating fibrous tissue [Figure 2]. A diagnosis of juvenile aggressive ossifying fibroma was made.
Figure 2: photomicrograph showing bony trabeculae surrounded by proliferating fibrous tissue (H and E, ×40)

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Thereafter, the patient had maxillectomy under general anesthesia using the intraoral approach and adjunctive lower lip split, after temporary ligation of the ipsilateral external carotid artery [Figure 3] and [Figure 4]. The lesion shelled out completely leaving a fibrous bed, which was further curetted and copiously irrigated with normal saline. A nasogastric tube was passed pending dental rehabilitation and to allow healing of the operative site while also improving the nutritional status as he found it difficult to swallow food. Subsequent reviews of the patient were satisfactory with no evidence of recurrence.
Figure 3: Dissection of the left external carotid artery

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Figure 4: Intraoperative picture showing the lower lip split and the clean tumor bed

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Case 2

A 20-year-old female presented at our clinic with a 3-year history of a recurrent giant maxillary swelling, which was progressively increasing in size. She had paring down of the same lesion 2 years before she presented to us. Clinical examination revealed the presence of a protruding globular maxillary lesion which was slightly skewed to the right, hard in consistency, and had two lobes with a trough centrally [Figure 5]. Consequently, the patient was unable to close her mouth and both nostrils were not patent. The upper lip was stretched, with loss of muscle tone, and the mucosal covering over the lesion was dry with prominent veins traversing it. Gross dental anarchy and pan-palatal involvement was seen. The vital signs were satisfactory (BP 120/80 mmHg; PR 88Bpm; RR 16 Cpm; and Temperature 37.2°C).
Figure 5: Clinical picture showing a massive maxillary tumor with flaccid upper lip

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Full blood count, serum alkaline phosphatase levels, electrolyte, and urea were within normal range. The posteroanterior and lateral radiographs of the jaws revealed an area of mixed radiolucency and radio-opacity with definite borders involving the entire maxilla with opacification of the maxillary antra [Figure 6]. Histology showed irregular, poorly oriented, and discontinuous trabeculae of immature bone surrounded by osteoblasts exhibiting zonal maturation in a cellular fibrous stroma. Areas of red blood cells extravasation with multinucleated giant cells were also seen [Figure 7] and [Figure 8]. Hence, a diagnosis of JOF was made.
Figure 6: Radiograph showing massive maxillary tumor with areas of mixed radiodensities

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Figure 7: Photomicrograph shows irregular, poorly oriented, and discontinuous trabeculae of immature bone (H and E, ×40)

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Figure 8: Photomicrograph shows areas of red blood cells extravasation with multinucleated giant cells were also seen (H and E, ×100)

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The patient was then scheduled for maxillectomy under general anesthesia. Intubation was achieved through the orotracheal route with the aid of fiberoptics. Subsequently, it was converted to submental route to allow unhindered access to the operative site. The protruding extraoral part of the tumor was sectioned; thereafter, total maxillectomy with preservation of the orbital floors was done [Figure 9]. The lesion shelled out with little resistance. The surgical bed was curetted and flushed copiously with normal saline. A gauze pack soaked in povidone-iodine and liquid paraffin paste was secured in place intraorally. An orogastric tube was passed to facilitate feeding. Subsequent reviews were satisfactory.
Figure 9: The tumor bed with bone wax in situ

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Case 3

An 11-year-old male presented at our clinic with a rapidly progressive right maxillary tumor of 6-month duration. There was a circumscribed bulbous right maxillary lesion with obliteration of the right nasolabial fold and right nostril [Figure 10]. In addition, there was dental anarchy and palatal involvement beyond the midline with near obliteration of the palatal vault [Figure 11]. The vital signs were satisfactory (BP 110/70 mmHg; PR 91Bpm; RR 25 Cpm; and temperature 37.3°C).
Figure 10: A bulbous maxillary lesion

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Figure 11: Intraoral view showing near obliteration of the palatal vault

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Full blood count, alkaline phosphatase, electrolyte, urea, and creatinine results were within normal range. Occipitomental radiographs of the skull revealed an area of mixed radiolucency and radio-opacity, with opacification of the right antrum and peripheral circumferential relative radiolucency. Histopathology showed interlacing bony trabeculae lacking osteoblastic rimming in a cell-rich connective tissue stroma with multiple plump spindle-shaped fibroblasts and few multinucleated giant cells [Figure 12] and [Figure 13].
Figure 12: Photomicrograph shows interlacing bony trabeculae lacking (H and E, ×40)

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Figure 13: Photomicrograph shows cellular connective tissue stroma with multiple plump spindle-shaped fibroblasts and few multinucleated giant cells (H and E, ×100)

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He had surgical excision of the tumor with adjunctive elective preoperative tracheostomy owing to anticipated difficult intubation. The tumor was excised, leaving a fibrous bed which was curetted and copiously irrigated with normal saline. A nasogastric tube was inserted and a gauze pack soaked in povidone-iodine was secured in place. Succeeding reviews have been satisfactory with no evidence of recurrence [Figure 14].
Figure 14: Shows postoperative extraoral view with acceptable scar

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  Discussion Top


Ossifying fibromas may be subclassified as the juvenile and conventional subtypes.[5],[10],[11] JOF is a rare lesion which is often confused with other lesions; it exhibits a rapid growth rate and may present with osteolytic lesions; hence, mismanagement is a real danger in patients presenting with it.[3],[6],[12] The conventional type is seen more in the mandible than maxilla, slow-growing and is seen more in the third and fourth decades of life.[12] They are amenable to surgery and recurrence is a rarity.[5],[6] However, the juvenile subtype has been reported to have a higher tendency to recur and shows more aggressiveness.[5],[6],[12] Ossifying fibromas seen in patients between the ages of 5 years and 15 years which exhibit fast growth have been designated as JOF.[13] JOF has histological subtypes which are the psammomatoid and the trabecular types.[9],[11] The psammomatoid JOF occurs primarily in the sinonasal and orbital bones while trabecular JOF is seen primarily in the jaws.[5],[8],[10] In this article, we presented two males and female aged 10, 11, and 20 years, respectively, all of whom presented with the trabecular subtype of JOF. The second patient had a previous surgery for the same lesion 2 years before presentation, and the lesion had been present since she was 13 years old.

Some authors have reported trauma as an etiologic factor, though in both cases reported here, there was no history of such.[5] Furthermore, some have reported a lack of a clear demarcation from uninvolved tissues radiologically and the presence of multilocular radiolucency; in these cases, the radiolucency was patchy, and the demarcations were easily seen, perhaps because they were relatively long-standing lesions.[14]

Clinically ossifying fibroma is symptomless in the initial stages; however, large tumors in the maxillofacial region may compress vital structures such as nerves, reduce the tongue space, and may even obliterate the nasal apertures, thus leading to a reduction in the patients' quality of life.[2],[7] In the cases reported, there was difficulty in feeding owing to the protrusion of the tumor into the buccal cavity. In the second case and third cases, there was complete and partial obliteration of the nostrils, respectively. Johnson et al.[15] reported the lesion to be more commonly seen in the mandible, especially in the young, this is in contrast to what was observed in these series. Observations made are similar to that of some authors who found it more commonly in the maxilla.[8]

Histologically, the psammomatoid subtype exhibits small spherical ossicles that are similar to psammoma bodies, while the trabecular subtype shows the presence of irregular, interlacing trabeculae of fibrillar osteoid, and woven bone.[5],[12] Osteoblastic rimming may or may not be present.[5],[6] Furthermore, the presence of numerous spindle-shaped or plump fibroblasts and multinucleated giant cells has also been reported. Diagnosis is often made by juxtaposing clinical and histopathological evidence.[5],[9]

Treatment of JOF may be by radical excision, excision with or without curettage.[2],[5],[6] It has been reported that incomplete excision predisposes to recurrence; hence, some researchers have advocated more radical approaches such as resection as a mode of treatment.[5],[6] Achieving and maintaining the airway intraoperatively was quite challenging, especially that these were shared airway surgeries with its attendant challenges. Therefore, there was a need to employ variations in airway management such as submental and fiber-optics intubation.

Surgical approaches were varied to reflect the peculiarities of each case. The lower lip split and Weber Fergusson approaches were used in Cases 1 and 3 respectively, while an entirely intraoral approach was used in Case 2. For Case 2, maxillectomy was done segmentally, with initial resection of the globular extraoral part, followed by resection of the residual maxillae. This was also possible because, the lips were stretched considerably, making access to the residual maxillary segment less challenging than it would have been otherwise. Thus, an entirely intraoral approach may be used for maxillary lesions, thereby avoiding the esthetic challenges of making a Weber Fergusson incision. Temporary suture ligation of the left external carotid artery was done for Case 1 because of the relative anemia and hemorrhagic nature of the lesion which bled profusely on slight provocation. They all had nasogastric or orogastric tubes to aid in feeding and to protect the operative site during the healing phase.


  Conclusion Top


JOF is an aggressive lesion which often has an insidious onset. It rapidly progresses in size and may have devastating effects on the quality of life of patients. There is a need for early diagnosis and management of the lesion. Surgical treatment is advocated; the need for rehabilitation and follow-up of patients is also of utmost importance.

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.
Toyosawa S, Yuki M, Kishino M, Ogawa Y, Ueda T, Murakami S, et al. Ossifying fibroma vs. fibrous dysplasia of the jaw: Molecular and immunological characterization. Mod Pathol 2007;20:389-96.  Back to cited text no. 1
    
2.
Liu Y, Wang H, You M, Yang Z, Miao J, Shimizutani K, et al. Ossifying fibromas of the jaw bone: 20 cases. Dentomaxillofac Radiol 2010;39:57-63.  Back to cited text no. 2
    
3.
Khan SA, Sharma NK, Raj V, Sethi T. Ossifying fibroma of maxilla in a male child: Report of a case and review of the literature. Natl J Maxillofac Surg 2011;2:73-9.  Back to cited text no. 3
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4.
Mehta D, Clifton N, McClelland L, Jones NS. Paediatric fibro-osseous lesions of the nose and paranasal sinuses. Int J Pediatr Otorhinolaryngol 2006;70:193-9.  Back to cited text no. 4
    
5.
Keles B, Duran M, Uyar Y, Azimov A, Demirkan A, Esen HH, et al. Juvenile ossifying fibroma of the mandible: A case report. J Oral Maxillofac Res 2010;1:e5.  Back to cited text no. 5
    
6.
Osunde O, Iyogun C, Adebola R. Juvenile aggressive ossifying fibroma of the maxilla: A case report and review of the literature. Ann Med Health Sci Res 2013;3:288-90.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Neville BW, Damn DD, Allen CM, Bouquot JE, editors. Oral and Maxillofacial Pathology. 1st.ed. Philadelphia: Saunders; 1995.  Back to cited text no. 7
    
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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. 8
    
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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. 9
    
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Nwizu NN, Aguirre A, Chen F. Diagnostic challenges of benign fibro-osseous lesions and psammomatous meningiomas of the craniofacial region: A comparative review of their clinico-pathological features. North Am J Med Sci2010;3:17-23.  Back to cited text no. 10
    
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Yadav N, Gupta P, Naik SR, Aggarwal A. Juvenile psammomatoid ossifying fibroma: An unusual case report. Contemp Clin Dent 2013;4:566-8.  Back to cited text no. 11
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12.
de Andrade M, Silva-Sousa YT, Marques MF, Pontual ML, Ramos-Perez FM, Perez DE, et al. Ossifying fibroma of the jaws: A clinicopathological case series study. Braz Dent J 2013;24:662-6.  Back to cited text no. 12
    
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Reichart PA, Philipsen HP, Sciubba JJ. The new classification of head and neck tumours (WHO) – Any changes? Oral Oncol 2006;42:757-8.  Back to cited text no. 13
    
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Noffke CE. Juvenile ossifying fibroma of the mandible. An 8 year radiological follow-up. Dentomaxillofac Radiol 1998;27:363-6.  Back to cited text no. 14
    
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Johnson LC, Yousefi M, Vinh TN, Heffner DK, Hyams VJ, Hartman KS, et al. Juvenile active ossifying fibroma. Its nature, dynamics and origin. Acta Otolaryngol Suppl 1991;488:1-40.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14]



 

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