|
 
 |
| CASE REPORT |
|
| Year : 2014 | Volume
: 2
| Issue : 2 | Page : 57-59 |
|
Juvenile ossifying fibroma: An unusual case report and review of the literature
Parveen Rana Kundu1, Kalpana Beniwal1, Balwan Singh2, Swaran Kaur1
1 Department of Pathology, BPS Government Medical College for Women, Khanpur Kalan, Sonepat, Haryana, India
2 Department of Otolaryngology, BPS Government Medical College for Women, Khanpur Kalan, Sonepat, Haryana, India
| Date of Web Publication | 12-Sep-2014 |
Correspondence Address:
Parveen Rana Kundu
House No. 1393, Sector-3, Rohtak, Haryana - 124 001
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2320-3846.140694
Juvenile ossifying fibroma is a rare benign, but locally aggressive tumor with high recurrence potential. This distinct clinical entity poses a diagnostic challenge because of its rapidly progressive and osteolytic nature mimicking malignant lesion. A 16-year-old young female presented to otolaryngology department of our institute with a history of painless swelling in the left nasal cavity and maxillary region.
Keywords: Juvenile ossifying fibroma, nasal cavity, maxillary sinus
How to cite this article:
Kundu PR, Beniwal K, Singh B, Kaur S. Juvenile ossifying fibroma: An unusual case report and review of the literature
. Saudi Surg J 2014;2:57-9 |
How to cite this URL:
Kundu PR, Beniwal K, Singh B, Kaur S. Juvenile ossifying fibroma: An unusual case report and review of the literature
. Saudi Surg J [serial online] 2014 [cited 2022 Aug 16];2:57-9. Available from: https://www.saudisurgj.org/text.asp?2014/2/2/57/140694 |
| Introduction | |  |
The term "fibro-osseous lesions" refers to conditions in which normal bone architecture is replaced by fibroblasts and collagen fibers and contains various amounts of mineralized material. It includes entities such as ossifying fibroma, fibrous dysplasia etc. [1]
There are two types of ossifying fibromas, the central type and peripheral type. Central ossifying fibroma is again of two types, conventional type and juvenile type. Juvenile ossifying fibroma (JOF) is a locally aggressive variant of central ossifying fibromas of the jaws, and it has a high tendency of recurrence with more common in young ones. [2]
The present study attempts to highlight a case of JOF involving left nasal cavity presenting as a nasal mass, mimicking malignancy in a young female. It is a rare clinical entity, which is often misdiagnosed and mismanaged due to its aggressive and ostoelytic nature.
| Case Report | | |
A 16-year-old female presented to ear nose and throat department with painless swelling in the maxillary region since 6 months. The swelling had grown rapidly since last 1-month. There was no history of any trauma, pain, discharge or any tooth extraction. Lymph node of the corresponding side was palpable, mobile, and soft to firm in consistency and nontender. The color and the temperature of the overlying skin were normal. Vision was intact in the associated left eye. The nostril was obstructed by the growth. Intra-orally, the lesion involved whole of left maxillary region with little extension to contra lateral side, with little or no displacement of associated teeth. The general examination revealed mild pallor, weight loss and mild difficulty in swallowing.
Computerized tomographic (CT) findings showed expansile mass like lesion was in the left maxillary sinus with evident ground glass like thickening of its wall, including the pterygoid and maxillary alveolus. The CT findings were suggestive of fibrous dysplasia/ossifying fibroma encroaching upon adjacent naso-ethemoid sinus [Figure 1]. There was no overt intracranial extension of the disease process. For confirmation, the biopsy was performed, and we received multiple soft tissues pieces measuring 1 × 1 × 0.6 cm. The histopathological analysis confirmed it to be as ossifying fibroma [Figure 2]. | Figure 1: Computerized tomographic scan showing the mass lesion in the maxillary sinus and the left nasal cavity
Click here to view |
 | Figure 2: Histological picture (H and E, ×400) showing trabeculae of woven bone surrounded by moderately cellular fibroblastic tissue
Click here to view |
| Discussion | | |
According to the new classification of World Health Organization, ossifying fibromas that appear as fast-growing mass between 5 years and 15 years of age, are radiologically well-bordered and consistent with ossifying fibroma histologically are referred as JOF. [3]
Juvenile aggressive ossifying fibroma (JAOF) is a relatively rare fibro-osseous lesion of the jaws characterized by early age of onset usually <15, its location, the radiological appearance, and high recurrent potentials. JAOF may present as one of two histological variants: Juvenile psammomatoid ossifying fibroma and juvenile trabecular ossifying fibroma. The psammomatoid type of JOF is reported more commonly than the trabecular variety, is more aggressive, and it has a strong tendency to recur. [4],[5]
It presents as a well-defined, unilocular or multilocular expansile and well-circumscribed radiolucent lesions with cortical thinning. [6],[7],[8] The clinical presentation and radiographic features of our patient were consistent with the features of JOF considering age, its rapidly progressive nature, and characteristic radiographic picture.
Microscopically, the trabecular variant is composed of a fibroblastic spindle cell stroma, containing osteoid matrix surrounded by osteoblasts and anastomosing trabeculae of immature woven bone, often intermixed with scattered clusters of multinucleated giant cells; mitoses may be present, but cystic degeneration is rare. [9],[10]
The most prominent condition in the differential diagnosis is fibrous dysplasia. The rapidity of growth, monostotic nature, and the well-delineated radiographic margins differentiates it from fibrous dysplasia. [11] Other major conditions in the differential diagnosis include aneurismal bone cyst, osteoblastoma, osteosarcoma, and cemento-osseous dysplasia. Burkitt lymphoma should also be considered in the differential diagnosis of JAOF because of the similarity in the age and site of presentation, rapidity of growth, and radiolucent radiographic appearance. The characteristic gross mobility and displacement of the associated teeth in what is usually termed "dental anarchy" may serve as a dividing line between the two lesions clinically. In addition, patients with Burkitt's lymphoma may present with varying abdominal symptoms, which may range from splenomegally, hepatomegaly, or enlargement of both organs. Burkitt's lymphoma usually presents with weight loss, and our patient also had some degree of weight loss. [12]
JOAF is treated by surgical excision and may recur if local resection is not complete. Long-term follow-up is necessarily owing to its locally aggressive nature and high recurrent potentials.
| Conclusion | | |
A careful assessment of its clinical, radiographic, and histopathologic features is necessary to overcome the diagnostic and therapeutic challenges associated with this lesion. The importance of its early recognition and adequate management is here emphasized as it is a rare clinically entity, which is often misdiagnosed and mismanaged due to it's rapidly and ostoelytic nature.
| References | | |
| 1. | Park S, Lee BJ, Lee JH. Juvenile ossifying fibroma: A clinico-pathologic study of 8 cases and comparison with craniofacial fibro-osseous lesions. Korean J Pathol 2007;41:373-9. 
|
| 2. | Saiz-Pardo-Pinos AJ, Olmedo-Gaya MV, Prados-Sánchez E, Vallecillo-Capilla M.Juvenile ossifying fibroma: A case study. Med Oral Patol Oral Cir Bucal 2004;9:456-8.
|
| 3. | Keles B, Duran M, Uyar Y, Azimov A, Demirkan A, Esen HH. Juvenile ossifying fibroma of the mandible: A case report. J Oral Maxillofac Res 2010;1:e5.
|
| 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.
|
| 5. | 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.
[PUBMED] |
| 6. | Kramer IR, Pindborg JJ, Shear M. The WHO histological typing of odontogenic tumours. A commentary on the second edition. Cancer 1992;70:2988-94.
|
| 7. | Breheret R, Jeufroy C, Cassagnau E, Malard O. Juvenile ossifying fibroma of the maxilla. Eur Ann Otorhinolaryngol Head Neck Dis 2011;128:317-20.
|
| 8. | Han MH, Chang KH, Lee CH, Seo JW, Han MC, Kim CW. Sinonasal psammomatoid ossifying fibromas: CT and MR manifestations. AJNR Am J Neuroradiol 1991;12:25-30.
|
| 9. | Eversole LR, Leider AS, Nelson K. Ossifying fibroma: A clinicopathologic study of sixty-four cases. Oral Surg Oral Med Oral Pathol 1985;60:505-11
|
| 10. | 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.
|
| 11. | Wenig BM, Vinh TN, Smirniotopoulos JG, Fowler CB, Houston GD, Heffner DK. Aggressive psammomatoid ossifying fibromas of the sinonasal region: A clinicopathologic study of a distinct group of fibro-osseous lesions. Cancer 1995;76:1155-65.
|
| 12. | 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.
[PUBMED]  |
[Figure 1], [Figure 2]
|
Search Pubmed for