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CASE REPORT
Year : 2022  |  Volume : 25  |  Issue : 1  |  Page : 28-31

Juvenile aggressive psammomatous ossifying fibroma of the maxilla: A report of three cases and review of the literature


1 Department of Maxillofacial Surgery, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
2 Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
3 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Jos, Jos, Nigeria
4 Department of Dental, General Hospital Minna, Minna, Nigeria

Date of Submission07-Jul-2020
Date of Decision01-Dec-2020
Date of Acceptance05-Jan-2021
Date of Web Publication30-May-2022

Correspondence Address:
Dr. Benjamin Fomete
Department of Maxillofacial Surgery, Ahmadu Bello University Teaching Hospital, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/smj.smj_80_20

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  Abstract 


Juvenile aggressive ossifying fibroma occurs in early life, and approximately 79% are diagnosed before the age of 15 years. Approximately 85% of cases were found in the face, while the calvarium and extracranial sites accounted for 12% and 3%, respectively. We present three females with psammomatous juvenile aggressive juvenile ossifying fibroma (JOF) of the maxilla diagnosed in our institution. While two of them were children of 11 and 12 years, the third was a 30-year-old. The histological type of JOF in all the three cases was the psammomatous type. They all had surgery with one of them having a radical maxillectomy.

Keywords: Aggressive ossifying fibroma, maxilla, psammomatous


How to cite this article:
Fomete B, Okeke AU, Samaila MO, Agbara R, Ajike SO, Mgbemena CC, Amuda O, Abugu HO. Juvenile aggressive psammomatous ossifying fibroma of the maxilla: A report of three cases and review of the literature. Sahel Med J 2022;25:28-31

How to cite this URL:
Fomete B, Okeke AU, Samaila MO, Agbara R, Ajike SO, Mgbemena CC, Amuda O, Abugu HO. Juvenile aggressive psammomatous ossifying fibroma of the maxilla: A report of three cases and review of the literature. Sahel Med J [serial online] 2022 [cited 2022 Aug 10];25:28-31. Available from: https://www.smjonline.org/text.asp?2022/25/1/28/346291




  Introduction Top


Juvenile ossifying fibroma (J OF) is an uncommon benign fibro-osseous lesion with a rapid growth that affects the facial skeleton and has high potential of recurrence.[1],[2] It occurs in early life, and approximately 79% are diagnosed before the age of 15 years.[3] It affects both sexes equally, and the maxilla is more involved compared to the mandible,[1] though cases have also been found in the orbit and skull. Approximately 85% of cases were found in the face, while the calvarium and extracranial sites accounted for 12% and 3%, respectively.[3] Clinical presentation of patients is variable and depends on the site of affectation and often includes swelling, pain, displaced teeth, nasal obstruction, and proptosis.[2] JOF is locally aggressive and associated with cortical osteolytic changes and involvement of closely related anatomical structures, thus simulating a malignancy.[4] Radiological examination, particularly computed tomography (CT) scan of the lesion, shows a low-density mass due to cystic changes.[3] Two microscopically separate entities of psammomatous JOF and trabecular JOF have been described. The psammomatous variant which affects the sinonasal and orbital bones and has a wider age distribution from 3 months to 72 years has been widely reported while the trabecular JOF preferentially affects the jaw bones and is seen in younger patients aged 2–12 years. We present three females with psammomatous juvenile aggressive JOF of the maxilla diagnosed in our institution.


  Case Reports Top


Case 1

A 30-year-old female presented at the dental clinic of a general hospital with a 17-month history of left maxillary swelling, which rapidly increased in size over the last 12 months. There was no history of prior trauma, associated pain, or relevant contributory medical history. Clinical examination of lesion showed left facial asymmetry and a maxillary swelling extending from the occlusal line to the infraorbital rim and from the nasolabial fold to the lateral canthal region with loss of the nasolabial fold. Intraorally, mouth opening was adequate and lesion was present on the left maxillary region; the teeth were mobile from 5 to 8. There was loss of palatal bone and labial fold.

Radiographs done were of poor qualities. Histopathological diagnosis of JOF was made on the incisional biopsy of lesion sent to the histopathology laboratory. She had a surgical excision under general anesthesia, via an upper buccal sulcus incision, access to the lesion was gained, and the lesion was exposed. Intraoperatively, lesion was un-encapsulated and extended to the floor of the orbit and the ethmoids with some areas of cystic degenerations. Excision of the lesion was carried out including the palatoalveolar segment involving 5–8. Hemostasis was achieved and the cavity was packed with povidone-iodine for 3 days. The patient was placed on antibiotics and analgesics, and recovery was uneventful. She was discharged home on the 5th day and the specimen was sent for histology. Report was consistent with initial diagnosis of JOF.

Case 2

A 12-year-old girl presented with a 2-year history of a painless gradually increasing left maxillary swelling. She gave a history of trauma before development of the swelling, with no other contributory medical history. Clinical examination revealed a left facial asymmetry with a maxillary swelling extending from the infraorbital rim to the occlusal plane and from the nasolabial fold to the preauricular region. Lesion measures 8 cm by 7 cm in diameter, not fluctuant. Intraorally, the lesion was present on the buccolabial sulcus and extended from upper left 3 to upper left 7. The mucosa over the lesion was intact, and there were no mobile teeth. Occipitomental view radiographs were taken. Incisional biopsy was reported as psammomatoid JOF. Definitive surgery was done under general anesthesia. The patient had a course of antibiotics and analgesics. Postoperatively, status was uneventful. She has been seen twice on follow-up with no recurrence.

Excised lesion weighed 24 g and measured 5 cm × 5 cm × 2.5 cm; microscopy showed a lesion composed of a moderate markedly cellular fibroblastic tissue admixed with concentric psammomatoid cementum-like material. Scattered woven and lamellar type of bony trabeculae were seen at the periphery of the lesion. Histopathology report was consistent with initial report.

Case 3

An 11-year-old girl presented with left maxillary swelling of 2-year duration. Growth of swelling increased in the last couple of months before presentation with associated discharging sinus. History of fall from a chair preceded the swelling. Traditional medication had also been applied before hospital presentation. Clinical examination revealed a massive left maxillary swelling extending to the right side [Figure 1], from the infraorbital rim to the lower border of the mandible. Swelling measured 12 cm by 13 cm in diameter and protruded out of the mouth, not fluctuant. There was associated proptosis on the left eye. Intraorally, there was lip incompetence with swelling ulcerated per areas and bleeding on touch. Swelling was present on the palate and crossed the midpalatal line to the right. There was alveolar expansion, and mobile teeth were upper left 2 and 3.
Figure 1: The 11-year-old female patient with the massive psammomatous ossifying fibroma

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Histopathology of biopsy specimen was reported as juvenile aggressive ossifying fibroma. The patient had total maxillectomy and tracheostomy was used for intubation as both Mallam and Patti American Society of Anesthesiologists were 4 each due to size of lesion. Lesion weighed 719 g and measured 17 cm × 14 cm × 10 cm. Microscopy revealed a fibro-osseous lesion which consisted of an intensely cellular fibroblastic stroma admixed with loose fibromyxoid areas. Numerous psammomatoid ossicles and ossifying nests of spindle cells were scattered within the lesion. No cellular atypia nor abnormal mitosis was noticed. Other areas showed an ulcerated oral mucosa and bony trabeculae. She has come for review about 6 times and has been give an obturator. She remains tumor free after 6 follow-up visits over a 2-year period [Figure 2].
Figure 2: The same patient 6 months postoperative

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  Discussion Top


Juvenile aggressive ossifying fibroma is a rare localized benign lesion of the bone with tendency to recur and with aggressive nature.[2] Its aggressiveness in nature and high tendency to recurrence differentiates it from cemento-ossifying fibroma with which it shares similar radiographic appearance.[3] Histologically, there are two types: the juvenile psammomatoid ossifying fibroma and the juvenile trabecular ossifying fibroma. The psammomatoid type is frequently reported than the trabecular, more aggressive, and a stronger tendency for recurrence.[4] The psammomatoid 'type' is found mostly in the paranasal sinuses, the orbit and sinus while the trabecular type is found in the maxillary region.[5]

Controversies have surrounded fibro-osseous lesions in the past. Cemento-ossifying fibroma and ossifying fibroma have been considered by some authors to be two distinct entities: reason being that the former was an odontogenic tumor and the later was not.[4],[6] These have been abandoned and the two lesions are now referred to as one due to their histology.[4],[7],[8] Moreover, because their cementum-like material was actually extragnatic and not dental in origin, their odontogenic origin was also abandoned.[4],[7],[8] Hence, ossifying fibroma is divided into classical ossifying fibroma, psammomatoid JOF, and trabecular JOF.[9]

Its age of onset is early usually before 15 years. These series recorded a median of 12 years and a range of 11–30 years which is in line with the reviewed literature.

Both sexes are equally affected though Gupta et al.[3] noted that some authors reported a male prevalence, while Johnson et al. reported the mandibular lesion to be more in girls. All our three patients were females, and the lesions involved the left maxilla.

Trauma is a known etiological factor for development of JOF. In our series, two out of three patients gave a history of trauma before swelling while the third gave a history of trauma as the tumor increased rapidly in size.

JOF should be differentiated from lesions such as aneurysmal bone cyst, cemento-osseous dysplasia, fibrous dysplasia, odontogenic tumors, osteoblastoma, and osteosarcoma.[1],[3],[4] JOF is known for its expansile nature, destruction of the cortex, and local aggressiveness all on CT scan.[3] Aggressive cortical changes are characteristics of juvenile form while sclerotic changes are peculiar to the adult form.[3]

JOF has a radiographic variation ranging from unilocular to multilocular radiolucency with well defined borders 'and' some degree of opacity that is related to when the patient presents. The aggressive nature is characterized by the presence of cortical thinning, perforation, tooth displacement, and root resorption.[10] The high rate of recurrence of JOF has been attributed to the propensity of JOF to perforate cortical bone. Radiologically, the key distinctive feature of JOF from fibrous dysplasia is the presence of circumscribed well-defined border.[11] It means Burkitt's lymphoma is on of the differential based on age, site, and the aggressive growth and radiographic appearance. However, the “dental anarchy” and abdominal symptoms should differentiate between the two.[4]

JOF is histologically characterized by cellular fibrous stroma, immature bony strand, and cement particles.[4] They are classified as two distinct clinicopathologic entities, namely the trabecular and the psammomatoid type. The juvenile psammomatoid variant comprises a propagation of benign spindle-shaped fibroblastic cells with embedded mineralized structures, which can present with round-to-ovoid collections of bone with an osteoid rim.[12] Multinucleated osteoclast-like giant cells can be seen with occasional normal mitotic figures, but atypia is not a usual feature. The trabecular variant is made up of a fibroblastic spindle cell stroma, containing osteoid matrix surrounded by osteoblasts and anastomosing trabeculae of immature woven bone, frequently intermixed with scattered clusters of multinucleated giant cells; mitoses may be present, but cystic degeneration is rare.[13]

The case reported by Osunde et al.[4] was trabecular. Two out of the three cases reported by Abuzinada and Alyamani[14] were psammomatoid and the other trabecular. All the cases in this study were psammomatoid types [Figure 3].
Figure 3: Cellular lesion with sheets of plumb fibroblasts interspersed by calcific/psammoma bodies. Part of thin fibrous capsule present at peripheral (H and E, ×40)

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The clinical management of JAOF remains uncertain. Treatment varies from curettage, enucleation to radical surgery depending on the size of the lesion.[1],[4] An open surgical approach, such as transfacial, or Weber–Ferguson approach has been used.[4],[15] In the present series, we used an upper buccal sulcus incision and did a modified maxillectomy in one case, total maxillectomy for the other, and curettage for the last.

Recurrence rates ranging from 30% to 58% have been reported.[1] The last two cases in this study have been followed up for a year and no recurrence and the first for 4 months.


  Conclusion Top


JOF is a benign locally aggressive bone lesion with high tendency for recurrence. There is a need for long-term follow-up with good clinical and radiological assessment.

Ethical approval was gotten from the Health Research Ethical Committee of Ahmadu Bello University Teaching Hospital, Zaria, Nigeria (ABUTH/ZHREC/Z01/2020).

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.
Shekhar MG, Bokhari K. Juvenile aggressive ossifying fibroma of the maxilla. J Indian Soc Pedod Prev Dent 2009;27:170-4.  Back to cited text no. 1
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2.
Ollfa BG, Nadia R, Safa N, Madiha M, Wafa A, Ines H, et al. Juvenile ossifying fibroma of the maxilla. EJENTA 2017;18:145-9.  Back to cited text no. 2
    
3.
Gupta K, Jain A, Rohatgi A. An observational study of Vitamin B12 levels and peripheral neuropathy profile in patients of diabetes mellitus on metformin therapy. Diabetes Metab Syndr 2018;12:51-8.  Back to cited text no. 3
    
4.
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. 4
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Lemoine S, Cassagnau E, Bertin H, Poisson M, Corre P, Guiol J. Juvenile ossifying fibroma: Case report and literature review. Management and differential diagnosis. J Oral Med Oral Surg 2018;24:67-71.  Back to cited text no. 5
    
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Maksoud C, Laujac MH, Prince G. Cemento-ossifying fibroma or fibrous dysplasia? A case report. Actual Odontostomatol 2000;54:287-92.  Back to cited text no. 6
    
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Eversole LR, Leider AS, Nelson K. Ossifying fibroma: A clinic-pathologic study of sixty-four cases. Oral Surg Oral Med Oral Pathol 1985;60:505-11.  Back to cited text no. 7
    
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Kramer IR, Pindborg JJ, Shear M. The WHO histological typing of odontogenic tumours. A commentary on the second edition. Cancer 1992;70:2988-94.  Back to cited text no. 8
    
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Breheret R, Jeufroy C, Cassagnau E, Malard O. Juvenile ossifying fibroma of the maxilla. Eur Ann Otorhinolaryngol Head Neck Dis 2011;128:317-20.  Back to cited text no. 9
    
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Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions. Oral Maxillofac Surg Clin North Am 2005;17:415-34.  Back to cited text no. 10
    
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Singh AK, Kumar N, Singh S, Pandey A, Verma V. Juvenile ossifying fibroma of the mandible: A case report and review. J Dent Allied Sci 2018;7:34-7.  Back to cited text no. 11
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12.
Bohn OL, Kalmar JR, Allen CM, Kirsch C, Williams D, Leon ME. Trabecular and psammomatoid juvenile ossifying fibroma of the skull base mimicking psammomatoid meningioma. Head Neck Pathol 2011;5:71-5.  Back to cited text no. 12
    
13.
Slootweg PJ, Panders AK, Koopmans R, Nikkels PG. Juvenile ossifying fibroma. An analysis of 33 cases with emphasis on histopathological aspects. J Oral Pathol Med 1994;23:385-8.  Back to cited text no. 13
    
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Abuzinada S, Alyamani A. Management of juvenile ossifying fibroma in the maxilla and mandible. J Maxillofac Oral Surg 2010;9:91-5.  Back to cited text no. 14
    
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Lawton MT, Heiserman JE, Coons SW, Ragsdale BD, Spetzler RF. Juvenile active ossifying fibroma. Report of four cases. J Neurosurg 1997;86:279-85.  Back to cited text no. 15
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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