Accepted Manuscript Psammomatoid and Trabecular Juvenile Ossifying Fibroma: two distinct radiographic entities Adepitan A. Owosho, BChD Marion A. Hughes, MD Joanne L. Prasad, DDS Anitha Potluri, DMD, MDsc Barton Branstetter, MD PII:
S2212-4403(14)01256-5
DOI:
10.1016/j.oooo.2014.09.010
Reference:
OOOO 1011
To appear in:
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Received Date: 26 June 2014 Revised Date:
15 August 2014
Accepted Date: 11 September 2014
Please cite this article as: Owosho AA, Hughes MA, Prasad JL, Potluri A, Branstetter B, Psammomatoid and Trabecular Juvenile Ossifying Fibroma: two distinct radiographic entities, Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology (2014), doi: 10.1016/j.oooo.2014.09.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Title: Psammomatoid and Trabecular Juvenile Ossifying Fibroma: two distinct radiographic entities Authors: Adepitan A. Owosho BChD¹, Marion A. Hughes MD²*, Joanne L. Prasad DDS³, Anitha Potluri DMD, MDsc4, Barton Branstetter, MD5
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Affiliations: 1. Chief resident, Oral and Maxillofacial Pathology, University of Pittsburgh Medical Center. 2. Assistant Professor, Division of Neuroradiology, Department of Radiology, University of Pittsburgh. 3. Clinical Assistant Professor, Department of Diagnostic Sciences, School of Dental
Medicine, University of Pittsburgh. 4. Assistant Professor, Department of Diagnostic Sciences, School of Dental Medicine, University of Pittsburgh. 5. Professor of Radiology, Otolaryngology, and Biomedical
* To whom correspondence should be addressed.
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Email: [email protected]
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Informatics, Division of Neuroradiology, Department of Radiology, University of Pittsburgh.
Telephone: (412) 647-3553
Address: 200 Lothrop Street, Pittsburgh, PA 15261
Disclosure: All authors declare that there are no financial conflicts associated with this study and that the funding source has no role in conceiving and performing the study.
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Word count for the abstract: 144
Word count for the manuscript: 2908 Number of tables: 1
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Number of figures: 8
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ABSTRACT Objective. Evaluate and compare the radiographic features of psammomatoid versus trabecular variants
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of juvenile ossifying fibromas. Study design. Twelve of fifteen cases of juvenile ossifying fibroma of the craniofacial bones, retrieved from our pathology archives for the period between 2002 and 2013, had available preoperative computed tomography scans. The radiographic features of each case were analyzed and compared by two
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neuroradiologists and one oral and maxillofacial radiologist, each blinded to the histopathologic diagnosis.
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Results. Juvenile psammomatoid ossifying fibroma demonstrated a ground-glass pattern either as an outer mantle with central radiolucency, a single mural nodule, or solid homogeneous mass. The trabecular variant presented as a radiolucent lesion with irregular, scattered calcifications. Both variants displayed a well-defined radiographic border.
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Conclusion. This study demonstrates that psammomatoid and trabecular juvenile ossifying fibromas exhibit unique radiographic features that allow for distinction between the two variants on computed
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tomography studies.
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INTRODUCTION
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Juvenile ossifying fibroma (JOF) is a benign fibro-osseous lesion with potential for aggressive growth and a predilection for the sinonasal bones, jaws, and calvarium.1-3 This condition is notably more common in young patients. There are two distinct histopathologic subtypes of JOF that are well-recognized: the psammomatoid and trabecular variants. While the microscopic features of these subtypes have been well-
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described in the literature, little attention has been paid to the distinctive radiographic characteristics of
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each. The purpose of this study is to identify the unique radiographic features of these two entities. Juvenile psammomatoid ossifying fibroma (JPOF) and juvenile trabecular ossifying fibroma (JTOF) are two distinct histopathologic entities. JPOF was first described by Benjamins in 1938,4 who termed it “osteoid fibroma with atypical ossification of the frontal sinus.” Since then, various names have been proposed for this entity. 2, 5, 6 The term “psammomatoid ossifying fibroma” was coined by Gögl in 1949.6
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The trabecular variant of juvenile ossifying fibroma was first described by Reed and Hagy in 1965,7 but it was Makek, in 1983,5 who termed it the “trabecular” variant and presented a clearer distinction between the two histologic subtypes. The appellation juvenile (aggressive) ossifying fibroma was previously used by the World Health Organization, but the current edition refers to the two variants of JOF as JPOF and
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JTOF.3 JOF occurs most frequently in children, but has also been described in adults.1, 2, 5, 8-12 JPOF, in
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particular, has been reported in adults more commonly than JTOF, and shows a wider age distribution (3 months to 72 years),2 while JTOF is seen predominantly in young patients, with an age range of 2 to 33 years and an average age of occurrence between 8 and 10 years.1, 5 Although both variants have a predilection for the craniofacial bones, the preferred site of occurrence of each variant differs. JPOF most commonly involves the paranasal sinuses, although cases of JPOF developing within the jaws and other craniofacial bones have also been reported.2, 8, 13-15 JPOF developing within the sinonasal region often present with aggressive clinical features, such as significant bony
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expansion and a high tendency for recurrence. In contrast, JTOF favors the gnathic bones, with a predilection for the maxilla. JTOF may exhibit rapid aggressive growth and lesions developing in the maxillary sinus may extend into adjacent paranasal sinuses and orbit.
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Histologically, JPOF typically reveals numerous spherical calcified bodies without osteoblastic rimming. Some of these calcifications show psammoma body-like concentric lamellae with basophilic centers and an eosinophilic osteoid rim. The intervening fibrous stroma consists of loosely to densely collagenized
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tissue containing a dense proliferation of fibroblast-like, spindle-shaped cells with hyperchromatic nuclei (Figure 1A, Case 2).3 Psammomatous calcifications are generally not observed in the trabecular variant.
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In comparison, JTOF is characterized by bony trabeculae of varying degrees of mineralization along with poorly-defined seams of immature osteoid which blend in with the surrounding fibrous stroma. The immature woven bone trabeculae are typically surrounded by plump osteoblasts. A lattice-like pattern may result from interconnected trabeculae (Figure 1B, Case 9).3 As seen in the psammomatoid variant, the background stroma in JTOF is typically quite cellular, with an abundance of spindle-shaped
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fibroblasts. Multinucleated giant cells, mitotic figures, and aneurysmal bone cyst-like changes may be seen in either variant.2, 3, 12, 16, 17 ASSOCITAED ANEURYSMAL BONE CYSTS TYPICALLY APPEAR AS RADIOLUCENT LESIONS WITH MULTIPLE CYSTS AND FLUID-FLUID LEVELS
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FREQUENTLY NOTED ON CT OR MRI.18
Various radiographic features have been reported to describe JOF, but radiographic correlation of each
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variant with microscopic findings has not been well described. A common description is of an expansile, well-defined, corticated, mixed radiopaque-radiolucent mass, OR MULTILOCULAR RADIOLUCENT LESION regardless of the subtype. Few reports provide a more detailed radiographic analysis that describes and distinguishes each variant, yet imaging plays an important role in the diagnosis of benign fibro-osseous lesions. The present study describes the distinctive radiographic features of JPOF versus JTOF. Twelve cases of juvenile ossifying fibroma were analyzed, 8 of the psammomatoid variant and 4 of
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the trabecular variant, in an attempt to radiographically differentiate between these two distinct
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histopathologic entities.
MATERIALS AND METHODS
The study was approved by the Institutional Review Board of the University of Pittsburgh (IRB#:
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PRO14030104). All cases histopathologically diagnosed as juvenile ossifying fibroma involving the craniofacial bones, of either the psammomatoid or trabecular subtype, were retrospectively identified
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from the electronic pathology records of the University of Pittsburgh Medical Center from 2002 to 2013. Fifteen cases (10 JPOF and 5 JTOF) were retrieved and the microscopic diagnosis was re-confirmed by AAO and JLP. Of those fifteen cases, twelve (8 JPOF and 4 JTOF) had preoperative maxillofacial computed tomography (CT) scans at our institution. The typical imaging protocol was a maxillofacial CT with intravenous contrast (LIGHTSPEED VCT AND OPTIMA CT660; GE (GENERAL ELECTRIC)
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HEALTHCARE, BUCKINGHAMSHIRE, UNITED KINGDOM) WITH 120KVP, 240 mA, COLLIMATION 1.25MM, FOV 15-23CM, PITCH FACTOR 0.53.IMAGES WERE REVIEWED IN BOTH BONE WINDOWS (WINDOW WIDTH/WINDOW LEVEL (WW/WL 2000/300)), AND SOFT
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TISSUE WINDOWS (WW/WL 400/40). Coronal and sagittal reformats were performed. Three cases were excluded since imaging was absent for review; these cases were external consults from other
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institutions. The bone and soft tissue windows of the CT scans of each case were reviewed based on the following set of criteria: anatomical site involved, invasion of surrounding structures, periphery (welldefined or poorly-defined border), and the internal structure of the JOF. The imaging studies were reviewed by three separate radiologists BB, MH, and AP each blinded to the histopathologic diagnosis. BB and MH are dedicated head and neck radiologists, certified by the American Board of Radiology with CAQ certification in neuroradiology, with 13 and 8 years of practice experience, respectively. AP is an oral and maxillofacial radiologist certified by the American Board of Oral and Maxillofacial Radiology with 9 years of practice experience.
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RESULTS
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Juvenile psammomatoid ossifying fibroma Eight cases of JPOF were examined. The patients’ sex distribution was balanced (4 males and 4 females) with ages ranging from 2 to 25 years (average of 11.5 years). In five cases, the lesions originated from the
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ethmoid, with extension to other sinonasal bones and the nasal cavity (Cases 1-3, 5, and 7). The
remainder of the cases arose within the maxilla (Cases 6 and 8) and the sphenoid bone (Case 4) (Table 1).
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Radiographically, the lesions all showed a well-defined corticated border, with no soft tissue involvement. In 6 out of 8 cases the internal structure of the lesions consisted of a thick, radiopaque, ground-glass outer mantle with a central radiolucency (Cases 2,3, 5-8) (Figures 2 and 3). One case (Case 1) showed a ground-glass opacity present as a single mural nodule (Figure 4). Another case (Case 4) showed a completely homogeneous ground-glass pattern throughout the lesion, with no central lucency (Figure 5).
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There was complete accord among the 3 reviewing radiologists with regards to the description of the internal structures of the lesions in all eight cases. Central calcifications were absent, no adjacent soft tissue invasion was present, and the borders were well-defined in all cases. Summary of their radiographic
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findings are presented in Table 1.
Juvenile trabecular ossifying fibroma
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Four cases of JTOF were included, affecting 3 males and one female, with ages ranging from 4 to 15 years (average of 12 years). Two lesions arose in the maxilla and extended to the ethmoid sinus and orbit (Cases 9 and 10), while one lesion was noted to originate from the ethmoid sinus with growth of the tumor into the sphenoid sinus (Case 11). The fourth case (Case 12) of JTOF was located in the mandible (Table 1). Radiographically, the lesions appeared predominantly radiolucent with a well-defined thin corticated margin and scattered, irregular calcifications (Cases 9, 10, 11, and 12) (Figures 6-8). In all 4 cases, the 3 reviewing radiologists agreed that central calcifications were present and none of the JTOFs
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demonstrated any areas with a ground glass pattern of calcification and also agreed that no adjacent soft tissue invasion was present and the borders were well defined in all four cases. A summary of the
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radiographic findings is presented in Table 1.
DISCUSSION
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JOF is a benign fibro-osseous lesion presently classified as a variant of ossifying fibroma. The lesion is termed “juvenile” because it develops primarily within the craniofacial bones of children, although some
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cases of JOF have been reported in adult patients. THE OCCURRENCE OF JOF IN ADULTS IS THE EXCEPTION TO THE RULE. IT SHOULD BE EMPHASIZED THAT THE DIAGNOSTIC CRITERIA APPLIED IN JOF OCCURRING IN ADULTS SHOULD BE STRICTLY APPLIED IN ORDER TO PREVENT CONFUSION WITH THE COMMON "ADULT" TYPE OF OSSIFYING FIBROMA. Radiographic studies play an important role in the diagnosis of fibro-osseous lesions such as fibrous
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dysplasia, ossifying fibroma, and osseous dysplasia. Waldron stated that “in the absence of good clinical and radiologic information, a pathologist can only state that a given biopsy is consistent with a fibroosseous lesion” but “with adequate clinical and radiologic information, most lesions can be assigned with
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reasonable certainty into one of several categories.”19 While the histologic features of the two variants of JOF have been well-established, the imaging characteristics of each subtype have not previously been
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well-defined. To date, imaging studies, while useful in determining the LOCATION and extent of the lesion, have failed to assist the clinician in rendering an accurate diagnosis. In this study, three radiologists independently reviewed the CT scans of 8 patients with JPOF and 4 patients with JTOF, in order to determine whether these variants have distinguishing radiographic features. A ground-glass radiopaque structure consisting of either an outer mantle with a central radiolucent core, a single mural nodule, or a completely homogeneous radiotexture was indicative of JPOF. Similar radiographic features have also been described in a few published case reports of JPOF.1, 8,
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10, 13, 20
Of course, ground-glass opacity is not unique to JPOF. It is a radiographic feature traditionally
associated with fibrous dysplasia. However, these two conditions should be easily distinguished from one another on the basis of other radiographic features. For example, fibrous dysplasia exhibits a diffuse
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border that blends imperceptibly with normal surrounding bone, whereas JPOF presents with a welldefined border. Furthermore, fibrous dysplasia retains the overall configuration of the involved bone, whereas JPOF has a roughly spherical configuration. Distinguishing JPOF from fibrous dysplasia is
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important, given the differing treatment implications. In JTOF, the ground glass opacity is absent. Instead, the internal structure is primarily radiolucent with irregular and scattered calcifications. This radiographic appearance was observed in all trabecular variants presented in this study and has also been reported in
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other cases of JTOF in the published literature.1, 21-23 TABLE 2 HIGHLIGHTS DISTINGUISHING RADIOGRAPHIC FEATURES OF BENIGN FIBRO-OSSEOUS LESIONS OF THE CRANIOFACIAL BONES.
It is important to point out that JOF lesions can occur anywhere in the craniofacial skeleton, regardless of
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subtype. All cases of JPOF in our study did develop within the sinonasal bones (ethmoid, maxilla, and sphenoid), which corroborates well with frequently reported sites of occurrence in other case series.2, 5, 9, 10 While most JPOF do favor these sites, cases of JPOF originating within the gnathic and other craniofacial
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bones have also been reported.2, 5, 8, 13 In contrast, JTOF favors gnathic bones, with a predilection for the maxilla,5, 12 although extragnathic cases arising in sinonasal bones were reported by Makek.5 In our case
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series, two cases of JTOF arose within the maxilla, one case was located in the ethmoid, and one developed within the mandible. Therefore, location should not be used as a reliable indicator of JOF subtype. Recognition of the distinct imaging characteristics of these lesions is more salient and useful in rendering an accurate radiographic diagnosis. In conclusion, our study demonstrates that the two histologic variants of JOF have a different and distinctive appearance on CT scans. These findings are not only useful in differentiating between these
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two variants, but can also help to distinguish JOF from other benign fibro-osseous lesions OF THE CRANIOFACIAL BONES.
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Banu K, Palikat SM. Juvenile trabecular ossifying fibroma of the mandible. J Maxillofac Oral Surg 2010;9:87-90.
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Espinosa SA, Villanueva J, Hampel H, Reyes D. Spontaneous regeneration after juvenile ossifying fibroma resection: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:e32-35.
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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;128:317-320.
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Owosho AA, Potluri A, Bilodeau E. Osseous dysplasia (Cemento-osseous dysplasia) of the jaw
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Figure Legends
1. Figure 1. Photomicrographs of juvenile psammomatoid ossifying fibroma (Case 2) (A) and juvenile trabecular ossifying fibroma (Case 9) (B) (H&E, x200)
2. Figure 2. JPOF (Case 2). Axial CT image demonstrates a lesion involving the ethmoid and sphenoid bones showing a ground-glass opacity with areas of central radiolucency (A) and a
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sagittal view (B)
3. Figure 3. JPOF (Case 5). Axial CT image demonstrates a lesion involving the ethmoid bone and extending to the nasal bone showing a ground-glass opacity with central radiolucency (A) and a
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sagittal view showing the lesion extending to the frontal sinus (B) 4. Figure 4. JPOF (Case 1). Axial CT image demonstrates a lesion involving the ethmoid bone
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showing a ground-glass single mural nodule (A) and a sagittal view (B) 5. Figure 5. JPOF (Case 4). Axial CT image demonstrates a lesion involving the ethmoid and sphenoid bones showing a completely homogeneous ground-glass opacity (A) and a sagittal view (B)
6. Figure 6. JTOF (Case 9). Coronal CT image demonstrates a LESION involving the maxillary bone and extending to the ethmoid bone showing irregular scattered calcifications (A) and an axial view (B)
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7. Figure 7. JTOF (Case 10). Axial CT image demonstrates a lesion involving the maxillary bone showing irregular scattered calcifications (A) and a coronal view showing the lesion extending to the ethmoid bone and orbit (B)
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8. Figure 8. JTOF (Case 11). Axial CT image demonstrates a lesion involving the ethmoid bone
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showing subtle, irregular scattered calcifications (A) and a coronal view (B)
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F
JPOF
3
15
F
JPOF
4
2
M
JPOF
5
13
M
JPOF
6
9
F
JPOF
7
7
F
JPOF
8
10
M
JPOF
9
15
M
JTOF
10
14
M
JTOF
11
15
M
JTOF
12
4
F
JTOF
NSTI = No soft tissue involvement
Size (mm) 57 x 46 x 30
Ethmoid and maxillary sinuses, nasal Maxillary and ethmoid sinuses Maxillary and ethmoid sinuses, orbit Maxillary and ethmoid sinuses, orbit Ethmoid and sphenoid sinuses Mandible
60 x 41 x 40
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Table 1. Summary of the clinical and radiographic features
Effect on surrounding structures Expansile, NSTI
68 x 44 x 40
Expansile, NSTI
46 x 46 x 34
Expansile, NSTI
43 x 34 x 27
Expansile, NSTI
46 x 46 x 26
Expansile, NSTI
28 x 25 x 22
Expansile, NSTI
48 x 45 x 35 51 x 51 x 46 53 x 44 x 39
Expansile, NSTI Expansile, NSTI Expansile, NSTI Expansile, NSTI
55 x 53 x 45
Expansile, NSTI
21 x 15 x 13
Expansile, NSTI
Periphery Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border
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25
Anatomical site Ethmoid and maxillary sinuses, nasal Ethmoid and sphenoid sinuses Ethmoid, maxillary, frontal sinuses, nasal Sphenoid, ethmoid sinuses and skull base Ethmoid and frontal sinuses, orbit, nasal Maxillary sinus
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Histologic variant JPOF
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Sex M
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Age 11
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Case no. 1
Internal structure Ground-glass opaque single mural nodule Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Homogeneous ground-glass opacity Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications
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Table 2. Distinguishing radiographic features of benign fibro-osseous lesions of the craniofacial bones. Fibrous dysplasia
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Depending on the stage of maturity, an ossifying fibroma may present as a unilocular radiolucency, a mixed radiolucent-radiopaque lesion, or a radiopaque mass. A ground-glass pattern is not observed. Mixed radiolucentradiopaque lesions can generally be distinguished from JTOF by the site (usually located at the tooth bearing region of the jaws) and age of the patient. Lesions exhibit a discrete well-defined border. When large, associated bony expansion exhibits a spherical configuration.
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Ossifying fibroma
Depending on the stage of maturity, fibrous dysplasia may present as a completely radiolucent lesion, a groundglass opacity, or homogenously sclerotic mass. In contrast to JPOF and JTOF, fibrous dysplasia typically shows an ill-defined border that blends imperceptibly with normal surrounding bone. Bony expansion maintains the overall shape and configuration of the involved bone (any bone in the craniofacial skeleton can be affected).
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Juvenile trabecular ossifying fibroma Primarily radiolucent with Ground-glass radiopaque lesion irregular and scattered presenting in one of three ways: calcifications. In contrast to (1) as an outer thick mantle with JPOF and fibrous dysplasia, a central radiolucent core, (2) a ground-glass opacity is single ground glass mural absent. nodule, or (3) a solid, JTOF also exhibits a wellhomogeneous radiopacity. defined border, discrete from Lesions exhibit well-defined, the normal surrounding discrete borders. Associated bone. Associated bony bony expansion demonstrates a expansion is similar to spherical configuration (has a JPOF, with a spherical predilection for sinonasal bones). configuration (has a predilection for gnathic bones).
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Juvenile psammomatoid ossifying fibroma
Osseous dysplasia Depending on the stage of maturity, osseous dysplasia may present as a completely radiolucent lesion, mixed radiolucent-radiopaque lesion with globular masses of calcified tissue, or as a radiopaque lesion with a radiolucent rim separating it from the normal surrounding bone and adjacent tooth roots (usually located at the periapical region of the jaws).24 The lesion is not associated with bone expansion.
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Statement of clinical relevance
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Juvenile ossifying fibroma has two histologic variants, it is important to be able to radiographically differentiate between these variants, in order to provide an accurate diagnosis.
S2212-4403(14)01256-5
DOI:
10.1016/j.oooo.2014.09.010
Reference:
OOOO 1011
To appear in:
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Received Date: 26 June 2014 Revised Date:
15 August 2014
Accepted Date: 11 September 2014
Please cite this article as: Owosho AA, Hughes MA, Prasad JL, Potluri A, Branstetter B, Psammomatoid and Trabecular Juvenile Ossifying Fibroma: two distinct radiographic entities, Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology (2014), doi: 10.1016/j.oooo.2014.09.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Title: Psammomatoid and Trabecular Juvenile Ossifying Fibroma: two distinct radiographic entities Authors: Adepitan A. Owosho BChD¹, Marion A. Hughes MD²*, Joanne L. Prasad DDS³, Anitha Potluri DMD, MDsc4, Barton Branstetter, MD5
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Affiliations: 1. Chief resident, Oral and Maxillofacial Pathology, University of Pittsburgh Medical Center. 2. Assistant Professor, Division of Neuroradiology, Department of Radiology, University of Pittsburgh. 3. Clinical Assistant Professor, Department of Diagnostic Sciences, School of Dental
Medicine, University of Pittsburgh. 4. Assistant Professor, Department of Diagnostic Sciences, School of Dental Medicine, University of Pittsburgh. 5. Professor of Radiology, Otolaryngology, and Biomedical
* To whom correspondence should be addressed.
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Email: [email protected]
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Informatics, Division of Neuroradiology, Department of Radiology, University of Pittsburgh.
Telephone: (412) 647-3553
Address: 200 Lothrop Street, Pittsburgh, PA 15261
Disclosure: All authors declare that there are no financial conflicts associated with this study and that the funding source has no role in conceiving and performing the study.
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Word count for the abstract: 144
Word count for the manuscript: 2908 Number of tables: 1
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Number of figures: 8
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ABSTRACT Objective. Evaluate and compare the radiographic features of psammomatoid versus trabecular variants
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of juvenile ossifying fibromas. Study design. Twelve of fifteen cases of juvenile ossifying fibroma of the craniofacial bones, retrieved from our pathology archives for the period between 2002 and 2013, had available preoperative computed tomography scans. The radiographic features of each case were analyzed and compared by two
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neuroradiologists and one oral and maxillofacial radiologist, each blinded to the histopathologic diagnosis.
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Results. Juvenile psammomatoid ossifying fibroma demonstrated a ground-glass pattern either as an outer mantle with central radiolucency, a single mural nodule, or solid homogeneous mass. The trabecular variant presented as a radiolucent lesion with irregular, scattered calcifications. Both variants displayed a well-defined radiographic border.
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Conclusion. This study demonstrates that psammomatoid and trabecular juvenile ossifying fibromas exhibit unique radiographic features that allow for distinction between the two variants on computed
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INTRODUCTION
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Juvenile ossifying fibroma (JOF) is a benign fibro-osseous lesion with potential for aggressive growth and a predilection for the sinonasal bones, jaws, and calvarium.1-3 This condition is notably more common in young patients. There are two distinct histopathologic subtypes of JOF that are well-recognized: the psammomatoid and trabecular variants. While the microscopic features of these subtypes have been well-
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described in the literature, little attention has been paid to the distinctive radiographic characteristics of
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each. The purpose of this study is to identify the unique radiographic features of these two entities. Juvenile psammomatoid ossifying fibroma (JPOF) and juvenile trabecular ossifying fibroma (JTOF) are two distinct histopathologic entities. JPOF was first described by Benjamins in 1938,4 who termed it “osteoid fibroma with atypical ossification of the frontal sinus.” Since then, various names have been proposed for this entity. 2, 5, 6 The term “psammomatoid ossifying fibroma” was coined by Gögl in 1949.6
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The trabecular variant of juvenile ossifying fibroma was first described by Reed and Hagy in 1965,7 but it was Makek, in 1983,5 who termed it the “trabecular” variant and presented a clearer distinction between the two histologic subtypes. The appellation juvenile (aggressive) ossifying fibroma was previously used by the World Health Organization, but the current edition refers to the two variants of JOF as JPOF and
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JTOF.3 JOF occurs most frequently in children, but has also been described in adults.1, 2, 5, 8-12 JPOF, in
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particular, has been reported in adults more commonly than JTOF, and shows a wider age distribution (3 months to 72 years),2 while JTOF is seen predominantly in young patients, with an age range of 2 to 33 years and an average age of occurrence between 8 and 10 years.1, 5 Although both variants have a predilection for the craniofacial bones, the preferred site of occurrence of each variant differs. JPOF most commonly involves the paranasal sinuses, although cases of JPOF developing within the jaws and other craniofacial bones have also been reported.2, 8, 13-15 JPOF developing within the sinonasal region often present with aggressive clinical features, such as significant bony
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expansion and a high tendency for recurrence. In contrast, JTOF favors the gnathic bones, with a predilection for the maxilla. JTOF may exhibit rapid aggressive growth and lesions developing in the maxillary sinus may extend into adjacent paranasal sinuses and orbit.
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Histologically, JPOF typically reveals numerous spherical calcified bodies without osteoblastic rimming. Some of these calcifications show psammoma body-like concentric lamellae with basophilic centers and an eosinophilic osteoid rim. The intervening fibrous stroma consists of loosely to densely collagenized
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tissue containing a dense proliferation of fibroblast-like, spindle-shaped cells with hyperchromatic nuclei (Figure 1A, Case 2).3 Psammomatous calcifications are generally not observed in the trabecular variant.
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In comparison, JTOF is characterized by bony trabeculae of varying degrees of mineralization along with poorly-defined seams of immature osteoid which blend in with the surrounding fibrous stroma. The immature woven bone trabeculae are typically surrounded by plump osteoblasts. A lattice-like pattern may result from interconnected trabeculae (Figure 1B, Case 9).3 As seen in the psammomatoid variant, the background stroma in JTOF is typically quite cellular, with an abundance of spindle-shaped
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fibroblasts. Multinucleated giant cells, mitotic figures, and aneurysmal bone cyst-like changes may be seen in either variant.2, 3, 12, 16, 17 ASSOCITAED ANEURYSMAL BONE CYSTS TYPICALLY APPEAR AS RADIOLUCENT LESIONS WITH MULTIPLE CYSTS AND FLUID-FLUID LEVELS
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FREQUENTLY NOTED ON CT OR MRI.18
Various radiographic features have been reported to describe JOF, but radiographic correlation of each
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variant with microscopic findings has not been well described. A common description is of an expansile, well-defined, corticated, mixed radiopaque-radiolucent mass, OR MULTILOCULAR RADIOLUCENT LESION regardless of the subtype. Few reports provide a more detailed radiographic analysis that describes and distinguishes each variant, yet imaging plays an important role in the diagnosis of benign fibro-osseous lesions. The present study describes the distinctive radiographic features of JPOF versus JTOF. Twelve cases of juvenile ossifying fibroma were analyzed, 8 of the psammomatoid variant and 4 of
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the trabecular variant, in an attempt to radiographically differentiate between these two distinct
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histopathologic entities.
MATERIALS AND METHODS
The study was approved by the Institutional Review Board of the University of Pittsburgh (IRB#:
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PRO14030104). All cases histopathologically diagnosed as juvenile ossifying fibroma involving the craniofacial bones, of either the psammomatoid or trabecular subtype, were retrospectively identified
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from the electronic pathology records of the University of Pittsburgh Medical Center from 2002 to 2013. Fifteen cases (10 JPOF and 5 JTOF) were retrieved and the microscopic diagnosis was re-confirmed by AAO and JLP. Of those fifteen cases, twelve (8 JPOF and 4 JTOF) had preoperative maxillofacial computed tomography (CT) scans at our institution. The typical imaging protocol was a maxillofacial CT with intravenous contrast (LIGHTSPEED VCT AND OPTIMA CT660; GE (GENERAL ELECTRIC)
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HEALTHCARE, BUCKINGHAMSHIRE, UNITED KINGDOM) WITH 120KVP, 240 mA, COLLIMATION 1.25MM, FOV 15-23CM, PITCH FACTOR 0.53.IMAGES WERE REVIEWED IN BOTH BONE WINDOWS (WINDOW WIDTH/WINDOW LEVEL (WW/WL 2000/300)), AND SOFT
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TISSUE WINDOWS (WW/WL 400/40). Coronal and sagittal reformats were performed. Three cases were excluded since imaging was absent for review; these cases were external consults from other
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institutions. The bone and soft tissue windows of the CT scans of each case were reviewed based on the following set of criteria: anatomical site involved, invasion of surrounding structures, periphery (welldefined or poorly-defined border), and the internal structure of the JOF. The imaging studies were reviewed by three separate radiologists BB, MH, and AP each blinded to the histopathologic diagnosis. BB and MH are dedicated head and neck radiologists, certified by the American Board of Radiology with CAQ certification in neuroradiology, with 13 and 8 years of practice experience, respectively. AP is an oral and maxillofacial radiologist certified by the American Board of Oral and Maxillofacial Radiology with 9 years of practice experience.
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RESULTS
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Juvenile psammomatoid ossifying fibroma Eight cases of JPOF were examined. The patients’ sex distribution was balanced (4 males and 4 females) with ages ranging from 2 to 25 years (average of 11.5 years). In five cases, the lesions originated from the
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ethmoid, with extension to other sinonasal bones and the nasal cavity (Cases 1-3, 5, and 7). The
remainder of the cases arose within the maxilla (Cases 6 and 8) and the sphenoid bone (Case 4) (Table 1).
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Radiographically, the lesions all showed a well-defined corticated border, with no soft tissue involvement. In 6 out of 8 cases the internal structure of the lesions consisted of a thick, radiopaque, ground-glass outer mantle with a central radiolucency (Cases 2,3, 5-8) (Figures 2 and 3). One case (Case 1) showed a ground-glass opacity present as a single mural nodule (Figure 4). Another case (Case 4) showed a completely homogeneous ground-glass pattern throughout the lesion, with no central lucency (Figure 5).
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There was complete accord among the 3 reviewing radiologists with regards to the description of the internal structures of the lesions in all eight cases. Central calcifications were absent, no adjacent soft tissue invasion was present, and the borders were well-defined in all cases. Summary of their radiographic
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findings are presented in Table 1.
Juvenile trabecular ossifying fibroma
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Four cases of JTOF were included, affecting 3 males and one female, with ages ranging from 4 to 15 years (average of 12 years). Two lesions arose in the maxilla and extended to the ethmoid sinus and orbit (Cases 9 and 10), while one lesion was noted to originate from the ethmoid sinus with growth of the tumor into the sphenoid sinus (Case 11). The fourth case (Case 12) of JTOF was located in the mandible (Table 1). Radiographically, the lesions appeared predominantly radiolucent with a well-defined thin corticated margin and scattered, irregular calcifications (Cases 9, 10, 11, and 12) (Figures 6-8). In all 4 cases, the 3 reviewing radiologists agreed that central calcifications were present and none of the JTOFs
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demonstrated any areas with a ground glass pattern of calcification and also agreed that no adjacent soft tissue invasion was present and the borders were well defined in all four cases. A summary of the
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radiographic findings is presented in Table 1.
DISCUSSION
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JOF is a benign fibro-osseous lesion presently classified as a variant of ossifying fibroma. The lesion is termed “juvenile” because it develops primarily within the craniofacial bones of children, although some
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cases of JOF have been reported in adult patients. THE OCCURRENCE OF JOF IN ADULTS IS THE EXCEPTION TO THE RULE. IT SHOULD BE EMPHASIZED THAT THE DIAGNOSTIC CRITERIA APPLIED IN JOF OCCURRING IN ADULTS SHOULD BE STRICTLY APPLIED IN ORDER TO PREVENT CONFUSION WITH THE COMMON "ADULT" TYPE OF OSSIFYING FIBROMA. Radiographic studies play an important role in the diagnosis of fibro-osseous lesions such as fibrous
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dysplasia, ossifying fibroma, and osseous dysplasia. Waldron stated that “in the absence of good clinical and radiologic information, a pathologist can only state that a given biopsy is consistent with a fibroosseous lesion” but “with adequate clinical and radiologic information, most lesions can be assigned with
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reasonable certainty into one of several categories.”19 While the histologic features of the two variants of JOF have been well-established, the imaging characteristics of each subtype have not previously been
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well-defined. To date, imaging studies, while useful in determining the LOCATION and extent of the lesion, have failed to assist the clinician in rendering an accurate diagnosis. In this study, three radiologists independently reviewed the CT scans of 8 patients with JPOF and 4 patients with JTOF, in order to determine whether these variants have distinguishing radiographic features. A ground-glass radiopaque structure consisting of either an outer mantle with a central radiolucent core, a single mural nodule, or a completely homogeneous radiotexture was indicative of JPOF. Similar radiographic features have also been described in a few published case reports of JPOF.1, 8,
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10, 13, 20
Of course, ground-glass opacity is not unique to JPOF. It is a radiographic feature traditionally
associated with fibrous dysplasia. However, these two conditions should be easily distinguished from one another on the basis of other radiographic features. For example, fibrous dysplasia exhibits a diffuse
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border that blends imperceptibly with normal surrounding bone, whereas JPOF presents with a welldefined border. Furthermore, fibrous dysplasia retains the overall configuration of the involved bone, whereas JPOF has a roughly spherical configuration. Distinguishing JPOF from fibrous dysplasia is
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important, given the differing treatment implications. In JTOF, the ground glass opacity is absent. Instead, the internal structure is primarily radiolucent with irregular and scattered calcifications. This radiographic appearance was observed in all trabecular variants presented in this study and has also been reported in
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other cases of JTOF in the published literature.1, 21-23 TABLE 2 HIGHLIGHTS DISTINGUISHING RADIOGRAPHIC FEATURES OF BENIGN FIBRO-OSSEOUS LESIONS OF THE CRANIOFACIAL BONES.
It is important to point out that JOF lesions can occur anywhere in the craniofacial skeleton, regardless of
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subtype. All cases of JPOF in our study did develop within the sinonasal bones (ethmoid, maxilla, and sphenoid), which corroborates well with frequently reported sites of occurrence in other case series.2, 5, 9, 10 While most JPOF do favor these sites, cases of JPOF originating within the gnathic and other craniofacial
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bones have also been reported.2, 5, 8, 13 In contrast, JTOF favors gnathic bones, with a predilection for the maxilla,5, 12 although extragnathic cases arising in sinonasal bones were reported by Makek.5 In our case
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series, two cases of JTOF arose within the maxilla, one case was located in the ethmoid, and one developed within the mandible. Therefore, location should not be used as a reliable indicator of JOF subtype. Recognition of the distinct imaging characteristics of these lesions is more salient and useful in rendering an accurate radiographic diagnosis. In conclusion, our study demonstrates that the two histologic variants of JOF have a different and distinctive appearance on CT scans. These findings are not only useful in differentiating between these
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two variants, but can also help to distinguish JOF from other benign fibro-osseous lesions OF THE CRANIOFACIAL BONES.
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References
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Espinosa SA, Villanueva J, Hampel H, Reyes D. Spontaneous regeneration after juvenile ossifying fibroma resection: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:e32-35.
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Figure Legends
1. Figure 1. Photomicrographs of juvenile psammomatoid ossifying fibroma (Case 2) (A) and juvenile trabecular ossifying fibroma (Case 9) (B) (H&E, x200)
2. Figure 2. JPOF (Case 2). Axial CT image demonstrates a lesion involving the ethmoid and sphenoid bones showing a ground-glass opacity with areas of central radiolucency (A) and a
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sagittal view (B)
3. Figure 3. JPOF (Case 5). Axial CT image demonstrates a lesion involving the ethmoid bone and extending to the nasal bone showing a ground-glass opacity with central radiolucency (A) and a
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sagittal view showing the lesion extending to the frontal sinus (B) 4. Figure 4. JPOF (Case 1). Axial CT image demonstrates a lesion involving the ethmoid bone
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showing a ground-glass single mural nodule (A) and a sagittal view (B) 5. Figure 5. JPOF (Case 4). Axial CT image demonstrates a lesion involving the ethmoid and sphenoid bones showing a completely homogeneous ground-glass opacity (A) and a sagittal view (B)
6. Figure 6. JTOF (Case 9). Coronal CT image demonstrates a LESION involving the maxillary bone and extending to the ethmoid bone showing irregular scattered calcifications (A) and an axial view (B)
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7. Figure 7. JTOF (Case 10). Axial CT image demonstrates a lesion involving the maxillary bone showing irregular scattered calcifications (A) and a coronal view showing the lesion extending to the ethmoid bone and orbit (B)
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8. Figure 8. JTOF (Case 11). Axial CT image demonstrates a lesion involving the ethmoid bone
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showing subtle, irregular scattered calcifications (A) and a coronal view (B)
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F
JPOF
3
15
F
JPOF
4
2
M
JPOF
5
13
M
JPOF
6
9
F
JPOF
7
7
F
JPOF
8
10
M
JPOF
9
15
M
JTOF
10
14
M
JTOF
11
15
M
JTOF
12
4
F
JTOF
NSTI = No soft tissue involvement
Size (mm) 57 x 46 x 30
Ethmoid and maxillary sinuses, nasal Maxillary and ethmoid sinuses Maxillary and ethmoid sinuses, orbit Maxillary and ethmoid sinuses, orbit Ethmoid and sphenoid sinuses Mandible
60 x 41 x 40
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Table 1. Summary of the clinical and radiographic features
Effect on surrounding structures Expansile, NSTI
68 x 44 x 40
Expansile, NSTI
46 x 46 x 34
Expansile, NSTI
43 x 34 x 27
Expansile, NSTI
46 x 46 x 26
Expansile, NSTI
28 x 25 x 22
Expansile, NSTI
48 x 45 x 35 51 x 51 x 46 53 x 44 x 39
Expansile, NSTI Expansile, NSTI Expansile, NSTI Expansile, NSTI
55 x 53 x 45
Expansile, NSTI
21 x 15 x 13
Expansile, NSTI
Periphery Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border Well-defined border
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Anatomical site Ethmoid and maxillary sinuses, nasal Ethmoid and sphenoid sinuses Ethmoid, maxillary, frontal sinuses, nasal Sphenoid, ethmoid sinuses and skull base Ethmoid and frontal sinuses, orbit, nasal Maxillary sinus
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Histologic variant JPOF
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Sex M
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Age 11
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Case no. 1
Internal structure Ground-glass opaque single mural nodule Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Homogeneous ground-glass opacity Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Ground-glass opaque mantle with central radiolucency Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications Radiolucency with irregular scattered calcifications
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Table 2. Distinguishing radiographic features of benign fibro-osseous lesions of the craniofacial bones. Fibrous dysplasia
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Depending on the stage of maturity, an ossifying fibroma may present as a unilocular radiolucency, a mixed radiolucent-radiopaque lesion, or a radiopaque mass. A ground-glass pattern is not observed. Mixed radiolucentradiopaque lesions can generally be distinguished from JTOF by the site (usually located at the tooth bearing region of the jaws) and age of the patient. Lesions exhibit a discrete well-defined border. When large, associated bony expansion exhibits a spherical configuration.
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Ossifying fibroma
Depending on the stage of maturity, fibrous dysplasia may present as a completely radiolucent lesion, a groundglass opacity, or homogenously sclerotic mass. In contrast to JPOF and JTOF, fibrous dysplasia typically shows an ill-defined border that blends imperceptibly with normal surrounding bone. Bony expansion maintains the overall shape and configuration of the involved bone (any bone in the craniofacial skeleton can be affected).
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Juvenile trabecular ossifying fibroma Primarily radiolucent with Ground-glass radiopaque lesion irregular and scattered presenting in one of three ways: calcifications. In contrast to (1) as an outer thick mantle with JPOF and fibrous dysplasia, a central radiolucent core, (2) a ground-glass opacity is single ground glass mural absent. nodule, or (3) a solid, JTOF also exhibits a wellhomogeneous radiopacity. defined border, discrete from Lesions exhibit well-defined, the normal surrounding discrete borders. Associated bone. Associated bony bony expansion demonstrates a expansion is similar to spherical configuration (has a JPOF, with a spherical predilection for sinonasal bones). configuration (has a predilection for gnathic bones).
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Juvenile psammomatoid ossifying fibroma
Osseous dysplasia Depending on the stage of maturity, osseous dysplasia may present as a completely radiolucent lesion, mixed radiolucent-radiopaque lesion with globular masses of calcified tissue, or as a radiopaque lesion with a radiolucent rim separating it from the normal surrounding bone and adjacent tooth roots (usually located at the periapical region of the jaws).24 The lesion is not associated with bone expansion.
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Statement of clinical relevance
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Juvenile ossifying fibroma has two histologic variants, it is important to be able to radiographically differentiate between these variants, in order to provide an accurate diagnosis.
