Juvenile Ossifying Fibroma Diagnosed on Fine Needle Aspiration Cytology: A Diagnostic Challenge Surbhi Goyal, M.D., D.N.B.,1 Sonal Sharma, M.D.,1* and Vipin Arora, M.D.2

Preoperative diagnosis of jaw lesions is not always possible on the basis of clinico-radiological findings alone and needs to be confirmed before attempting any surgical intervention. Fibroosseous lesions of the jaw comprise a spectrum of diseases which include cement-osseous dysplasia, fibrous dysplasia, and ossifying fibroma. The cytomorphological distinction between these individual entities is difficult. We present a case of maxillary fibro-osseous lesion in an adolescent girl diagnosed and categorized as juvenile ossifying fibroma preoperatively on cytology and confirmed on histopathology. Although aspirates are usually paucicellular in fibro-osseous lesions, certain cytological features if present in cellular cytosmears can offer further categorization and a definitive diagnosis may be possible in light of clinico-radiological correlation. Diagn. Cytopathol. 2015;43:75–79. VC 2014 Wiley Periodicals, Inc. Key Words: juvenile ossifying fibroma; jaw lesion; FNAC; fibro-osseous lesion

Introduction Fibro-osseous lesions of the jaw comprise a spectrum of diseases which include cement-osseous dysplasia (COD), fibrous dysplasia (FD), and ossifying fibroma.1 Morphological differentiation among these individual entities is difficult and a combined clinico-radiological correlation is essential for exact categorization. Fine needle aspiration cytology (FNAC) has proven to be a reliable and simple

1 Department of Pathology, University College of Medical Sciences, Delhi, India 2 Department of Otorhinolaryngology, University College of Medical Sciences, Delhi, India *Correspondence to: Dr. Sonal Sharma, M.D., Associate Professor, Department of Pathology, University College of Medical Sciences, University of Delhi, Dilshad Garden, Delhi 110095, India. E-mail: [email protected] Received 10 October 2013; revised 29 January 2014; Accepted 16 February 2014 DOI: 10.1002/dc.23136 Published online 3 March 2014 in Wiley Online Library (wileyonlinelibrary.com).

C 2014 WILEY PERIODICALS, INC. V

method for diagnosis of various lesions in long bones, vertebrae, and ribs.2 However, role of FNAC in diagnosis of jaw lesions is not established yet.3 To our knowledge, only a very few reports have described the cytological features of fibro-osseous lesionsin jaws.2,4,5 We report here a case of juvenile ossifying fibroma (JOF) in an adolescent girl, diagnosed on FNAC preoperatively and subsequently confirmed on histopathology, highlighting the diagnostic utility of FNA in jaw lesions.

Case Report A 16-year-old girl presented with a slow growing, painless swelling in the right cheek for the last 2 years. On examination, a diffuse ill defined swelling measuring 5 3 5 cm was seen in right cheek, which was firm, fixed, and nontender. The mass was causing a bulge in the right side of the hard palate, extending into right nasal cavity and pushing the nasal septum to the opposite side. Superior margin of the swelling was not palpable and inferiorly, it was extending below angle of the mouth. Orthopantomogram showed a well-defined unilocular expansile lytic lesion in the right maxillary sinus. Noncontrast followed by contrast-enhanced computed tomography of paranasal sinuses showed soft tissue attenuation with multiple irregular foci of calcifications and mild heterogeneous enhancement within the mass (Fig. 1). FNAC of the mass was performed intraorally using 23 gauge needle. Air dried smears and alcohol fixed smears were stained with May–Grunwald–Giemsa and Papanicolaou stains, respectively. Smears were moderately cellular and showed predominantly singly scattered round to plump cells with moderate amount of cytoplasm (Fig. 2). Tumor cells had minimal pleomorphism and atypia. At places, cells were oval to spindle shaped with elongated nucleus and distinct bipolar cytoplasmic processes, therefore, appeared fibroblastic in nature (Fig. 2). There was no necrosis, Diagnostic Cytopathology, Vol. 43, No 1

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Fig. 1. NCCT axial section at the level of bilateral maxillary sinuses showed a well-defined expansile lytic lesion in the right maxillary sinus with presence of multiple foci of calcification. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary. com.]

not seen in the smears examined. Pap smear showed largely crush artefact, however, few preserved foci showed similar tumor cell morphology with fine nuclear chromatin with inconspicuous nucleoli (Fig. 3b). Based on the age of the patient, radiological and cytological findings, a diagnosis of “fibro-osseous lesion, possibly JOF” was suggested and wide local excision was advised. Patient underwent right total maxillectomy and we received a well circumscribed tumor measuring 5 cm in diameter within the right maxilla. Underlying mucosa was normal and teeth were displaced (Fig. 4a). Cut surface was firm, gray brown, homogenous with whorled appearance without necrosis or hemorrhage. Microscopy showed a well circumscribed tumor comprising of fibroblastic stroma with variable cellularity from loose to densely cellular areas with fascicular arrangement. Irregular deposits of pink hyaline cementum like material with central calcification were seen (Fig. 4b). Higher magnification showed curved ossicles which were poorly cellular, had central basophilic calcification and thick irregular pink collagenous rim (Fig. 5c). Some of these were spherical with concentric lamellations and threadlike calcifications (psammomatoid bodies, Fig. 5b). At places, ossicles fused to form irregular trabeculae. On careful search, occasional trabeculae of woven bone with osteoblastic rimming (Fig. 5d) and scattered calcified psammoma bodies like structures (Fig. 5a) were seen, confirming the diagnosis of JOF—psammomatoid variant.

Discussion

Fig. 2. FNAC smears show singly scattered round to plump to spindle fibroblastic cells with moderate amount of pale blue cytoplasm. Occasional osteoblast with abundant deep blue cytoplasm and eccentrically placed nucleus was seen adjacent to darkly stained calcified acellular material (May–Grunwald–Giemsa, 3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

hemorrhage, or mitosis. Some darkly stained, acellular, calcified material was seen. On careful searching, occasional osteoblast with abundant deep blue cytoplasm and eccentrically placed nucleus, was seen adjacent to this calcified material (Fig. 2). At places this material showed slight metachromasia which we thought could be cementum (Fig. 3a). Giant cells and cystic macrophages were

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Preoperative diagnosis of jaw lesions is not always possible on the basis of clinico-radiological findings alone and needs to be confirmed before attempting any surgical intervention. Radiologically, the differential diagnoses of a radiolucent jaw lesion with scattered radiopacities can be categorized into three broad groups: Odontogenic epithelial tumors, odontogenic mesenchymal tumors, and bone-related tumors like (ossifying fibroma and central giant cell granuloma). Odontogenic epithelial tumors included Adenomatoid odontogenic tumor, Calcifying epithelial odontogenic tumor (Pindborg tumor), Odontoameloblastoma, and Ameloblastic fibro-odontoma, while Cementoblastoma and Odontogenic myxoma/myxofibroma were the main differentials in the mesenchymal tumors. Absence of epithelial component excluded the epithelial odontogentic tumors with or without associated ectomesenchyme. Cellular smears and lack of significant amount of metachromatic or myxoid material ruled out the diagnosis of cementoblastoma and myxoma/myxofibroma. Cytological features did not support the diagnosis of giant cell lesion. Absence of nuclear atypia and necrosis helped to exclude malignant lesions from

Diagnostic Cytopathology DOI 10.1002/dc

JUVENILE OSSIFYING FIBROMA ON FNAC

Fig. 3. (a) At places acellular material showed slight metachromasia, which could be probably cementum (May–Grunwald–Giemsa, 3400). (b) Pap smear showed crush artefact, cells had fine bland nuclear chromatin and inconspicuous nucleoli (Papanicolaou, 3200). (c) Tumor cells varied from round to oval to spindly, with some fibroblasts and occasional osteoblasts (May–Grunwald–Giemsa, 3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

Fig. 4. (a) Gross photograph-cut surface showed a well circumscribed, gray brown, homogenous tumor mass. (b) Microscopy revealed irregular deposits of pink cementum like material with central calcification, lying within variably cellular fibrous stroma (Hematoxylin and Eosin, 3100). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

consideration. Presence of numerous fibroblasts along with acellular calcified material was suggestive of fibroosseous lesion in our case. Fibro-osseous lesion is a broad category which includes FD, COD, and ossifying fibroma. FD is a very close differential morphologically, but radiologically is a poorly defined expansile lesion with dense homogeneous ground-glass appearance, and was thus excluded. The curvilinear trabeculae of woven bone in FD typically lack osteoblast rimming.5 COD are small, poorly defined lesions commonly seen in middle aged females and occupy the periapical region of the toothbearing jaw areas, which was not the scenario in our case. Well demarcated lesion in maxilla in a 16-year-old

girl with a variable radiolucent to radiopaque appearance and the cytomorphological features clinched the diagnosis of JOF. JOF is different from conventional ossifying fibroma, which commonly occurs in second to fourth decade of life with female preponderance and shows characteristic osteoblastic rimming.6 Because of the presence of acellular pink cementum like material, JOF was previously termed as cemento-ossifying fibroma. Fibro-osseous lesions involving the maxillo-facial region are characterized by replacement of bone by cellular fibroblastic tissue containing foci of mineralization that vary in amount and appearance. JOF is a rare tumor, accounting for 2% of all oral tumors in children.7 It Diagnostic Cytopathology, Vol. 43, No 1

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Fig. 5. Higher magnification showed (a) and (b) psammomatoid bodies, (c) curved ossicles, and (d) woven bone rimmed by osteoblasts (Hematoxylin and Eosin, 3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

mostly occurs in first to second decade of life without any sex predilection.7 Common sites include the facial bones (85%), calvarium (12%), and mandibular region (10%).1 The term JOF in WHO classification (2005) includes two histopathological variants—psammomatous and trabecular.6 The tumor originates from mesodermal cells of odontogenic origin, most probably the periodontal ligament.7 It comprises of calcified psammomatoid bodies/ossicles admixed within variable amount of fibrous stroma, reflecting the multipotential capability of the progenitor cells. Osteoblastic rimming around the bone trabeculae may be seen.7 As these lesions are very firm and difficult to aspirate, scant or inadequate aspirates often pose a major limitation for diagnosis in fibro-osseous lesions and a biopsy may be required.8 Thinning or destruction of cortical bone usually permits the penetration of fine needle for aspiration as in our case. Sivasubramaniam et al.9 emphasized the role of FNA in early diagnosis of JOF and excluded the clinical differential diagnosis of Burkitt’s lymphoma in a 6-year-old boy. Ramzy et al. emphasized the controversial role of FNA in the diagnosis of fibro-osseous lesions of bone due to variability in quantity and quality of the aspirated material.5,8 August et al.10 reviewed 32 jaw lesions including odontogenic cysts, ameloblastomas, fibroosseous, and giant cell lesions. They stated that inadequate or nonrepresentative sampling was the main limitation in discrepant cases.10 However, with adequate aspirated material, FNAC can be diagnostic at times and 78

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obviate the need of open biopsy which is much more painful, requires local anaesthesia and is associated with risk of bleeding. FNA is a simple, quick, and cost effective procedure, which can be easily performed preoperatively in an outpatient department. The present case highlights a rare case of maxillary fibro-osseous lesion in an adolescent girl diagnosed and categorized as JOF preoperatively on cytology and confirmed on histopathology. FNAC excluded the clinicoradiological differential diagnoses comprising of various odontogenic epithelial and mesenchymal lesions and paved way for surgical management in our case. To conclude, although fibro-osseous lesions usually yield paucicellular aspirates as described in literature, but whenever adequate material is obtained one should look for the presence of plump fibroblastic cells, osteoblasts, and acellular calcified material in cytosmears, which may guide further categorization of these lesions and a definitive diagnosis may be possible in light of clinicoradiological correlation.

References 1. Patil RS, Chakravarthy C, Sunder S, Shekar R. Psammomatoid type juvenile ossifying fibroma of mandible. Ann Maxillofac Surg 2013; 3:100–103. 2. Logrono R, Kurtycz DF, Wojtowycz M, Inhorn SL. Fine needle aspiration cytology of fibrous dysplasia: A case report. Acta Cytol 1998;42:1172–1176. 3. Huening MA, Reddy S, Dodd LG. Fine-needle aspiration of fibrous dysplasia of bone: A worthwhile endeavor or not? Diagn Cytopathol 2008;36:325–330.

Diagnostic Cytopathology DOI 10.1002/dc

JUVENILE OSSIFYING FIBROMA ON FNAC 4. G€unhan O, Demirel D, Seng€ un O, Celasun B, Finci R. Cementifying fibroma diagnosed by fine needle aspiration cytology: A case report. Acta Cytol 1992;36:98–100. 5. Gupta N, Gupta R, Bakshi J, Rajwanshi A. Fine needle aspiration cytology in a case of fibrous dysplasia of jaw. Diagn Cytopathol 2009;37:920–922. 6. Barnes L, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours. pathology and genetics of head and neck tumours. Lyon: IARC Press; 2005. p 283– 328. 7. Smith SF, Newman L, Walker DM, Papadopoulos H. Juvenile aggressive psammomatoid ossifying fibroma: An interesting, chal-

lenging and unusual case report and review of the literature. J Oral Maxillofac Surg 2009;67:200–206. 8. Ramzy I, Aufdemorte TB, Duncan DL. Diagnosis of radiolucent lesions of the jaw by fine needle aspiration biopsy. Acta Cytol 1985;29:419–424. 9. Sivasubramaniam V, Faduyile F, Zarka MA, Deery A, Field A. Maxillary cementifying fibroma: A case report with fine-needle aspiration cytology highlighting the expanding role of e-medicine in cytopathologist collaborations. Diagn Cytoathol 2012;40:810–814. 10. August M, Faquin WC, Ferraro NF, Kaban LB. Fine-needle aspiration biopsy of intraosseous jaw lesions. J Oral Maxillofac Surg 1999;57:1282–1286.

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Juvenile ossifying fibroma diagnosed on fine needle aspiration cytology: a diagnostic challenge.

Preoperative diagnosis of jaw lesions is not always possible on the basis of clinico-radiological findings alone and needs to be confirmed before atte...
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