Year : 2009 | Volume
: 27 | Issue : 3 | Page : 170--174
Juvenile aggressive ossifying fibroma of the maxilla
M Guna Shekhar, K Bokhari
NTR University of Health sciences, AP, India
M Guna Shekhar
c/o M. Prakash, #160, 5th cross, 28th Main, Sector-1, HSR Layout, Bangalore-560 102
Juvenile aggressive ossifying fibroma is an uncommon and controversial, benign but aggressive osteogenic neoplasm of the jaws commonly occurring in children and young adults. Complete removal of the tumor at the earliest possible stage is recommended owing to its high recurrence rate of 30-58%. Reported here is a case of recurrent juvenile aggressive ossifying fibroma of the maxilla in a 15-year-old male treated with enucleation and curettage. A 10-month follow-up showed no signs of recurrence.
|How to cite this article:|
Shekhar M G, Bokhari K. Juvenile aggressive ossifying fibroma of the maxilla.J Indian Soc Pedod Prev Dent 2009;27:170-174
|How to cite this URL:|
Shekhar M G, Bokhari K. Juvenile aggressive ossifying fibroma of the maxilla. J Indian Soc Pedod Prev Dent [serial online] 2009 [cited 2020 Feb 28 ];27:170-174
Available from: http://www.jisppd.com/text.asp?2009/27/3/170/57098
Juvenile ossifying fibroma (JOF) is an uncommon benign fibro-osseous lesion that affects the craniofacial skeleton in young age patients.  JOF affects both males and females equally without any significant gender predilection.  Maxilla is more often affected than the mandible. Two microscopically distinct types of JOF have been described, trabecular juvenile ossifying fibroma and psammomatoid juvenile ossifying fibroma. Psammomatoid type is more commonly reported and it affects patients from wider range (3 months-72 years). It occurs predominantly in the sino-nasal and orbital bones. In contrast, trabecular variant affects patients with a mean age range of 2-12 years and predominantly affects the jaws. 
The juvenile ossifying fibroma has also been termed the juvenile active ossifying fibroma, the juvenile aggressive ossifying fibroma, and the aggressive psammomatoid ossifying fibroma. When JOF behaves in aggressive manner and the patient is under 15 years of age, the term juvenile aggressive ossifying fibroma (JAOF) is usually considered. ,
A 15-year-old male presented to a private dental hospital in July 2008 with a complaint of swelling on the right side of the face for 6 weeks. Past history revealed that he had undergone treatment for a similar complaint at the same site, by a private practitioner elsewhere 9 months back, but recurred again. Family and medical history was otherwise normal. Extra oral examination revealed a swelling with normal appearing skin on the right side of face localized to the maxilla [Figure 1]. No cervical lymphadenopathy was noted and the mouth opening was normal.
Intraoral examination presented an asymptomatic swelling extending anteroposteriorly from distal of right upper lateral incisor to the mesial of first permanent molar; superiorly to obliterate the sulcus and inferiorly to the level of attached gingiva of the retained deciduous teeth i.e., 53, 54, and 55. There was an expansion of the labial cortex with no apparent mucosal changes [Figure 2]. On palpation, the swelling was hard in consistency with no fluctuant elicited. Grade 1 mobility was seen in retained primary teeth which were noncarious.
Radiological investigations including an intraoral periapical radiograph of the lesion showed a radiolucent area with radiopaque specks [Figure 3]. Further PNS skull and panoramic radiograph showed a radiolucent and radiopaque appearing multilobulate mass involving the right maxillary antrum. Displacement of impacted 13 and 14 toward the midline and 15 to the distal was evident. No evidence of root resorption of permanent teeth was seen [Figure 4] and [Figure 5]. The clinical and radiographic picture was in favor of a fibro-osseous lesion.
Routine hematological and urine investigations ascertained values within normal limits. Aspiration yielded negative result, ruling out cystic lesion, and then an incision biopsy was performed under local anesthesia from the buccal side of the lesion and sent for histopathological examination.
Histopathologic examination revealed features which include trabeculae of osteoid lying in a cellular fibrous connective tissue stroma with plumps or spindle-shaped cells without much cytoplasm and peripheral area of the lesion showing capsule, cellular fibrous tissue, and areas of mineralization [Figure 6] and [Figure 7]. The overall clinical, radiological, and histopathological picture was consistent with JAOS.
Under general anesthesia, enucleation of the tumor was performed (right partial maxillectomy) and a clear demarcation was evident between the tumor and healthy bone. Multilobulated nature of lesion was revealed clinically by uneven scoops of bony septae formed on the under surface of palate which was the inferior extent of the tumor. Retained deciduous 53, 54, and 55 were extracted [Figure 8],[Figure 9],[Figure 10].
Histologic picture of the excised postoperative specimen was identical with that of a biopsy specimen. Follow-up examination of the patient after 10 months showed no signs of recurrence [Figure 11] and [Figure 12]. The patient is under regular clinical and radiological follow-up due to recurrent nature of the lesion and awaits prosthetic rehabilitation.
Fibro-osseous lesions of craniofacial skeleton are rare and are thought to be the result of replacement of normal bony architecture by fibrous tissues, which may mineralize in various forms like woven, lamellae bone, or cementum and include a broad spectrum of distinct entities with different clinical presentations and microscopic appearances.  Most benign fibro-osseous lesions of jaws are asymptomatic and slowly progressing. Moreover, an unusual clinical presentation with apparent aggressive and destructive growth may be expected when the lesion is encountered in a younger patient, especially below the age of 15 years. ,,
JAOF is a benign but aggressive neoplasm commonly seen in children and young adults. JAOF has been thought to arise as a result of differentiation of the mesenchymal cells of the periodontal ligament, multipotential precursor cells, into cementum, osteoid, or fibrous tissue.  Lawton et al speculated that they perhaps originate from the maldevelopment of the tissue generating the bony septa between the roots of molar teeth. Pimenta et al,  recently reported association of a new tumor suppressor gene (HRPT2) mutation with ossifying fibroma and suggested that these lesions could arise as a result of haploinsufficiency of the particular gene.
Johnson et al reviewed 3000 fibro-osseous lesions and found that a majority of tumors were located in facial bones, among which approximately 90% originated from paranasal sinuses. Single or multiple sinuses may be involved. When jaws are involved, the maxilla is involved more frequently than the mandible, as seen in the present case.
JAOF is a relatively rare fibro-osseous lesion of the jaws characterized by the early age of onset i.e., under 15 years of age, the location of the tumor, and the radiological appearance and the tendency to recur.  JAOF appears as a unilobulated/multilobulated radiolucent lesion in their early stages of development followed by radiopaque appearance and is surrounded by a uniform radiolucent trimming at later stages.  Persistent growth of the lesions that involve paranasal sinuses may obstruct the normal sinus outflow and cause mucoceles. 
JAOF appears as a discrete mass, well demarcated from the surrounding bone, though not encapsulated. , On sectioning, the cut surface is typically a tan-white, rubbery, homogeneous mass with a firm-to-gritty consistency. The characteristic histologic features include the presence of a mineralized component composed of numerous spherical cementum-like bodies surrounded by osteoblastic rimming and a cellular stroma. 
Fibro-osseous lesions present a diagnostic dilemma owing to overlapping clinical and histopathologic findings. A differential diagnosis of fibrous dysplasia, cystic lesions, and osteosarcoma should be considered. Fibrous dysplasia was ruled out, as it typically blends with the normal bone at the margins of the lesion and is characterized by a less cellular stroma and its osteoid do not exhibit osteoblastic rimming. , The case discussed here presented a well-demarcated lesion from the surrounding bone and with highly cellular fibrous stroma and prominent osteoblastic rimming histologically which were suggestive of JAOF. Benign radiographic appearance, absence of cytological atypia, and infiltration to adjacent structures in the present case ruled out osteosarcoma.
Although computed tomography has been suggested to add value to the diagnosis and confirm the extent of destruction,  but because of financial constraints, the patient could not afford it. However, a good combination of clinical, radiological, and pathological findings can distinguish JAOF from its counterparts. In the case discussed here, clinical, radiological, and histological features along with a positive past history of swelling and treatment confirmed the diagnosis of JAOF.
The clinical management of JAOF remains uncertain. Small lesions can be treated conservatively by curettage or enucleation. An open surgical approach, such as transfacial, is ideal for resecting large and irregular-shaped tumors that infiltrate sinuses and fronto nasal bones.  Recurrence rates ranging from 30% to 58% have been reported. , Recurrence of the tumor in the present case can be related to its incomplete excision provided elsewhere 9 months back.
Complete removal of the lesion at the earliest possible stage has been suggested by majority of the authors., The appropriate treatment for a benign fibro-osseous lesion, irrespective of its aggressive nature includes either curettage or enucleation of the lesion, until the healthy bony margins are reached, , which corroborates with the treatment provided in the present case. Early detection and complete surgical excision of these lesions followed by long-term follow-up bears importance in clinical management due to their aggressive nature and high recurrence rate.
Our special and sincere thanks to Dr. Gannamaneni Srinivas, Sri Sri Dental Specialities, Tadepalligudem, AP, and Dr. Saraswathi, Senior Professor in Oral and Maxillofacial Pathology, Chennai. We also would like to thank Dr. Manikya Kumar, Dr. Prasanthi Bh and Dr. Rajeshwari P for their technical assistance in the preparation of the manuscript.
|1||Brannon RB, Fowler CB. Benign fibro-osseous lesions: A review of current concepts. Adv Anat Pathol 2001;8:126-43.|
|2||Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1993;51:828-35.|
|3||El-Mofty S. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicpathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304.|
|4||Williams HK, Mangham C, Speight PM. Juvenile ossifying fibroma: An analysis of eight cases and comparison with other fibro-osseous lesions. J Oral Pathol Med 2000;29:13-8.|
|5||Bertrand B, Eloy PH, Cornelis JP, Gosseye S, Clotuche J, Gilliard CL. Juvenile aggressive cemento-ossifying fibroma: Case report and review of the literature. Laryngoscope 1993;103:1385-90.|
|6||Lawton MT, Heiserman JE, Coons SW, Ragsdale BD, Spetzler RF. Juvenile active ossifying fibroma: Report of four cases. J Neurosurg 1997;86:279-85.|
|7||Pimenta FJ, Gontijo Silveria LF, Tavares GC, Silva AC, Perdigγo PF, Castro WH, et al. HRPT2 gene alterations in ossifying fibroma of the jaws. Oral Oncol 2006;42:735-9.|
|8||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.|
|9||Sun G, Chen X, Tang E, Li Z, Li J. Juvenile ossifying fibroma of the maxilla. Int J Oral Maxillofac Surg 2007;36:82-5.|
|10||Koury ME, Regezi JA, Perrott DH, Kaban LB. Atypical fibro-osseous lesions: Diagnostic challenges and treatment concepts. Int J Oral Maxillofac Surg 1995;24:162-9.|
|11||Artico M, Cervoni L, Salvata M, Raco A, Ciappetta P. Ossifying fibroma of the skull: Clinical and therapeutic study. Tumori 1994;80:64-7.|
|12||Jung SL, Choi KH, Park YH, Song HC, Kwon MS. Cemento-ossifying fibroma presenting as a mass of the parapharyngeal and masticator space. AJNR Am J Neuroradiol 1999;20:1744-6.|
|13||Kristensen S, Tveteras K. Aggressive cementifying fibroma of the maxilla. Arch Otorhinolaryngol 1986;243:102-5.|