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International Journal of Medical and Dental Case Reports (2014), Article ID 060914, 3 Pages

C A S E R E P O R T

Psammomatoid juvenile ossifying fi broma: Case report with managementDominic Augustine1, B. Sekar2, S. Saravannan3, Shankar Gouda Patil4, Vijay Kumar Reddy2

1Department of Oral and Maxillofacial Pathology, Bangalore Institute of Dental Sciences & Hospital, Lakkasandra, Bengaluru, Karnataka, India, 2Department of Oral and Maxillofacial Pathology, Vinayaka Missions Sankarachariyar Dental College & Hospital, Ariyanoor, Salem, Tamil Nadu, India, 3Department of Oral and Maxillofacial Surgery, Vinayaka Mission Hi Tech Hospital, Seerangapadi, Salem, Tamil Nadu, India, 4Department of Oral and Maxillofacial Pathology, MS Ramaiah Dental College & Hospital, MS Ramaiah Educational Campus, Bengaluru, Karnataka, India

Introduction

Ossifying fi broma is a rare tumor entity and a well-demarcated benign fi bro-osseous lesion that is composed of fi brous tissue, metaplastic bone and varying amounts of mineralized material. The ossifying fi bromas have been grouped into conventional and juvenile clinicopathologic types.[1] Conventional ossifying fi bromas are slow growing and tend to occur in the third-fourth decades of life. The juvenile form is clinically more aggressive, invasive and has a high recurrence rate.[1] Hence, it is a clinically signifi cant lesion which requires aggressive therapy and post-operative follow-up.

Case Report

An 18-year-old female visited the dental clinic with a chief complaint of swelling and pain on the right side of the jaw for the past 2 months. On extra oral examination, a diff use ill-defi ned swelling was seen involving the right body of the mandible causing facial asymmetry [Figure 1].

No lymph nodes were palpable. Her medical history was non-contributory.

On intraoral examination, a solitary large swelling was seen extending from the right mandibular fi rst premolar region to the right mandibular 3rd molar region. Orthopantomogram showed

CorrespondenceDr. Dominic Augustine, Department of Oral and Maxillofacial Pathology, Bangalore Institute of Dental Sciences & Hospital, Hosur Main Road, Lakkasandra, Bengaluru - 560 029, Karnataka, India. Phone: +91-9886226214, Email: dominic2germain@gmail.com

Received 02.09.2014;Accepted 07.10.2014

doi: 10.15713/ins.ijmdcr.6

How to cite the article:Augustine D, Sekar B, Saravannan S, Patil SG, Reddy VK. Psammomatoid juvenile ossifying fi broma: Case report with management. Int J Med Dent Case Rep 2014:1-3.

AbstractThe juvenile aggressive ossifying fi broma or juvenile ossifying fi broma (JOF) is a controversial lesion that has been distinguished from the conventional ossifying fi bromas on the basis of the age of the patients, most common sites of involvement and clinical behavior. Due to their diverse clinical and histological variations JOFs, pose diagnostic and therapeutic diffi culties. A case of psammomatoid juvenile ossifying fi broma (PJOF) is described here with aggressive clinical characteristics.

Keywords: Aggressive, juvenile, ossifying fi broma

Figure 1: A diff use ill-defi ned swelling involving the right body of the mandible

Augustine, et al. Psammomatoid juvenile ossifying fi broma

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a multilocular lesion in the body of the right mandible causing pathologic fracture of the inferior border. The margins appeared scalloped, and resorption of roots was seen in relation to teeth 46, 47 and 48 [Figure 2].

A provisional diagnosis of ameloblastoma was arrived at. A diff erential diagnosis of keratocystic odontogenic tumor was established.

Microscopic examination of the incisional biopsy revealed a highly cellular stroma consisting of plump, uniform, stellate, and spindle-shaped cells arranged in the form of whorls [Figure 3]. Spherical calcifi cations were seen distributed throughout the stroma that demonstrated a basophilic center and peripheral brush border [Figure 4]. A fi nal diagnosis of psammomatoid juvenile ossifying fi broma (PJOF) was concluded.

On surgical exploration, the lesion was fi brous in consistency and poorly demarcated from the adjacent normal bone. Due to the high recurrence rate of JOF, the patient was treated aggressively by a wide segmental resection; a mandibular reconstruction plate was placed [Figure 5]. The patient was discharged uneventfully. There was no evidence of recurrence of the tumor 4 years after surgery, and the patient is continuing to receive routine follow-up.

Discussion

The PJOF is an aggressive lesion. The maldevelopment of the basal generative mechanism essential for root formation appears to play a role in the pathogenesis of these jaw lesions. The developing tooth can either be missing, displaced or remain unerupted.[2]

The age at diagnosis varies markedly with cases being reported in infants less than 6 months to adults over 70 years of age. Although both trabecular and psammomatoid variants reveal similar radiographic features and growth patterns, the trabecular form is, usually, diagnosed in younger patients. The mean age of trabecular JOFs is approximately 11 years, whereas the age of patients diagnosed with the psammomatoid variant approaches 22 years. Both patterns occur in either jaw but reveal a maxillary predominance.

PJOF is a variant of JOF that has a predilection for sites such as the sinonasal tract, orbital, frontal and ethmoid bones. The lesion has the potential to be aggressive, proliferative and invasive in nature. A recurrence rate as high as 30-58% has been reported.[3]

A feature that helps diff erentiate trabecular JOF from psammomatoid JOF is its location, with the psammomatoid variant frequently appearing outside the jaws with over 70% arising in the orbital, frontal bones and paranasal sinuses whereas

Figure 2: Orthopantomogram showing a large lesion in the body of the mandible causing pathologic fracture

Figure 3: Photomicrograph showing a highly cellular stroma consisting of plump, uniform, stellate, and spindle shaped cells arranged in the form of whorls (H and E, ×100)

Figure 4: Photomicrograph section showing Psammoma-Like bodies, with a central basophilic area and a peripheral eosinophilic fringe or peripheral brush border (H and E, ×400 magnifi cation)

Figure 5: Post-operative orthopantomogram with mandibular reconstruction plate

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Psammomatoid juvenile ossifying fi broma Augustine, et al.

the trabecular JOF occurs mainly in the maxilla. Mandibular and extra cranial involvement is rare. Only 10% of cases involve the mandible.[4] The present case occurred in the body of the mandible.

Based on the site, symptoms such as paresthesia, pain, sinusitis, malocclusion and proptosis can also occur. Malignant transformation has been reported to develop in lesions that, usually, recur.[5]

Although many of these tumors are initially discovered upon routine radiographic examination cortical expansion may result in clinically detectable facial enlargement.

Radiographically a well-defi ned, osteolytic or mixed lesion with a cystic appearance is observed, as seen in the present case. Sclerotic changes in the lesion may produce a ground-glass appearance. The lesions may range in size from 2 to 8 cm in diameter.[6] In tumors arising near the cribriform plate, intracranial extension is a common fi nding because of the circumscribed growth pattern of the tumor. The frontal lobe is typically elevated without any associated neurologic signs.[7] Root displacement is common, and resorption is rare.[8] Both expansion as well as perforation is observed as seen in the present case.

Histologically both trabecular and psammomatoid patterns are nonencapsulated with demarcation from the adjacent bone. A tumor consists of a highly cellular and dense fi brous connective tissue stroma. The mineralized component in the two patterns is very diff erent. The trabecular variant shows irregular trabeculae of cellular osteoid with plump and irregular osteocytes. In contrast the psammomatoid pattern forms concentric lamellated and spherical ossicles that vary in size and demonstrate a central basophilic area with peripheral eosinophilic rims forming brush borders that blend into the adjacent stroma. Golg (1949)[9] was the fi rst to term these spherical calcifi ed structures. The term is derived from the Greek word psammos meaning “sand.” The psammoma bodies appear as numerous small, round ossicles or called as “psammomatoid” bodies embedded in a cellular fi brous stroma. The ossicles are mineralized collagenous foci that vary from small, smoothly contoured round-to-oval patterns to larger, irregular shapes, with concentric layering.[10]

The treatment for smaller lesions is complete local excision or thorough curettage. For rapidly growing lesions, a wider resection may be required. The present case was treated by wide

resection, and a mandibular reconstruction was planned. No recurrence has been observed in the present case 4 years after surgery.

Conclusion

Even though, the ossifying fi broma is a benign lesion, the PJOF has aggressive clinical behavior along with a very strong tendency to recur. JOF as a progressively growing lesion can attain enormous sizes with resultant deformity if left untreated. Hence, radical surgery is necessary with close follow-up. Early diagnosis and treatment can aid in better prognosis and lesser morbidity.

References

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2. El-Moft y S. Psammomatoid and trabecular juvenile ossifying fi broma of the craniofacial skeleton: Two distinct clinicopathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304.

3. Park S, Lee BJ, Le JH, Cho KJ. Juvenile ossifying fi broma: A  clinicopathologic study of 8  cases and comparison with craniofacial fi bro-osseous lesions. Korean J Pathol 2007;41:373-9.

4. Offi ah C, Hall E. Case of the month. The rapidly enlarging chin mass. Br J Radiol 2005;78:175-6.

5. Brannon RB, Fowler CB. Benign fi bro-osseous lesions: A review of current concepts. Adv Anat Pathol 2001;8:126-43.

6. Eversole R, Su L, ElMoft y S. Benign fi bro-osseous lesions of the craniofacial complex. A  review. Head Neck Pathol 2008;2:177-202.

7. Rinaggio J, Land M, Cleveland DB. Juvenile ossifying fi broma of the mandible. J Pediatr Surg 2003;38:648-50.

8. Zama M, Gallo S, Santecchia L, Bertozzi E, De Stefano C. Juvenile active ossifying fi broma with massive involvement of the mandible. Plast Reconstr Surg 2004;113:970-4.

9. Patigaroo SA. Juvenile Psammomatoid Ossifying Fibroma (JPOF) of Maxilla-a Rare Entity. J  Maxillofac Oral Surg 2011;10:155-8.

10. Waknis P, Sarode SC, Dolas RS. Psammatoid juvenile ossifying fi broma of the mandible with secondary aneurysmal bone cyst: A case report. Asian J Oral Maxillofac Surg 2011;23:83-6.