- Email: [email protected]
Multiple complex odontoma of the maxilla and the mandible
Vol. 120 No. 1 July 2015
Multiple complex odontoma of the maxilla and the mandible Lisha Sun, PhD,a Zhipeng Sun, MD,b and Xuchen Ma, PhDb Objective. The aim of this study was to present a literature review and case report of multiple odontoma. Methods. A case of multiple odontoma is presented with a review of the English-language literature. The clinical and radiologic features are summarized. Results. In total, 12 cases of multiple odontoma were retrieved from the literature for analysis. The multiple odontomas were localized or extensive and involved two to four quadrants of the jaws. The histologic types were compound or complex. Conclusion. Odontomas can be multiple and involve multiple quadrants of the jaws. (Oral Surg Oral Med Oral Pathol Oral Radiol 2015;120:e11-e16)
Odontoma is one of the most common odontogenic tumors and usually involves the tooth-bearing regions of the jaw.1 There are two basic types: compound and complex. The compound odontoma forms multiple irregular toothlike structures, whereas the complex odontoma forms amorphous calcification, with dysplastic dentin covered by enamel.1 Both compound and complex odontomas mostly occur as solitary lesions in the jaw. Multiple odontoma (MO) is characterized by numerous odontomas involving one to four quadrants of the jaws.2-6 MO also can occur with other malformations, such as stenosis of the esophagus.7,8 The terms “odontomatosis” and “odontoma syndrome” have been suggested to describe MO.2,5 Due to its rarity, little is known about the comprehensive clinical features of MO. Radiologic diagnosis of a single or multiple compound odontoma based on the presence of characteristic toothlike structures is not difficult. However, multiple and massive complex odontomas, exhibiting amorphous calcification, raise many possible differential diagnoses and constitute a diagnostic and therapeutic challenge. Fibrous dysplasia can frequently present as multiple expansive lesions with mixed radiopaque and radiolucent density involving both the maxilla and the mandible. Florid osseous dysplasia lesions can be bilateral and occur in both jaws apical to teeth, with varying densities ranging from an equal mixture of radiolucent and radiopaque regions to almost complete radiopacity. Ossifying fibroma can also occasionally be multiple and massive, showing well-fined tumors with internal calcifications. Gardner syndrome can present with multiple osteomatous jaw lesions,9 dental anomalies, or epidermoid cysts as extracolonic lesions.10 a
Key Laboratory of Oral Pathology, School and Hospital of Stomatology, Peking University, Beijing, China. b Department of Oral and Maxillofacial Radiology, School and Hospital of Stomatology, Peking University, Beijing, China. Received for publication Sep 17, 2014; returned for revision Feb 17, 2015; accepted for publication Feb 20, 2015. Ó 2015 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2015.02.488
We report a case of multiple complex odontoma with a review of the related literature.
CASE REPORT A 14-year-old male complaining of a large oral tumor was referred to our hospital. The tumor was small when it was discovered at birth. It grew slowly and expansively. No treatment had been delivered. Progressive swallowing and masticatory difficulties prompted the patient to seek treatment. The family history was unremarkable. General physical examination showed that the patient was undernourished. Routine blood test indicated anemia with low hemoglobin (117 g/L), low hematocrit (37% L/L), low mean corpuscular volume (77.5 fL), low mean content of hemoglobin (24.6 pg), and high platelet hematocrit (0.37%). A bulbous mass of approximately 6 cm in diameter was noted protruding from the anterior mandible with intact overlying mucosa (Figure 1). The tumor was hard, nontender, and fixed to the mandible. The cheek and the infraorbital areas were expanded with normal facial skin. Intraoral examination showed that the right mandible was expanded from the midline to the ramus. The right maxilla was expanded from the midline to the tuberosity. The lesions in the mandible and the maxilla were hard and nontender, and the mucosa was intact. Ocular examination demonstrated a single elevated inferotemporal limbal mass extending approximately 5 mm into the cornea from the limbus. The patient was referred to an ophthalmologist, and the mass was diagnosed as a limbal dermoid. Panoramic radiography (Figure 2) and spiral computed tomography (CT) (Figure 3) revealed two well-defined adjacent lesions in the right maxilla. The anterior lesion extended around the impacted permanent maxillary right lateral incisor, from the floor of the nasal cavity to the anterior alveolar bone. It was mainly radiolucent, with some calcifications at the inferior aspect. The other maxillary lesion extended around the caudally displaced permanent maxillary left third molar, along the anterior wall of the maxillary sinus to the buccal alveolar bone. The permanent maxillary lateral incisor and the third permanent molar were both located at the peripheral borders of the tumors. The permanent maxillary right canine was also impacted and located outside the maxillary lesions. Three closely adjacent mandibular lesions were observed on CT (Figure 4). The posterior lesion in the ramus appeared as a well-demarcated radiolucency with the impacted permanent
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Fig. 2. Panoramic radiograph of the patient. Two tumors are observed in the right maxilla. The permanent maxillary right lateral incisor (arrow 1) is caudally displaced and surrounded by a radiolucent capsule with a well-defined border and calcification at the bottom. The permanent maxillary right third molar (arrow 2) is displaced and superimposed by a well-defined calcified mass. The mandibular tumors present as multilocular lesions with mixed density. Two permanent mandibular right molars (arrows 3 and 4) and canine (arrow 5) are impacted inside the lesions, and another permanent mandibular molar (arrow 6) is displaced.
Fig. 1. Clinical facial photo of the patient. Note the oral mass protruding from the anterior mandible and the inferotemporal limbal mass on the right side. third molar and coronal calcification. The right ramus was expanded, and the cortex was continuous and thinned (see Figures 4A and 4B). The middle lesion in the molar and premolar region was made up of circumferentially intermingled radiolucent and radiopaque areas around a peripherally displaced permanent molar (see Figures 4B and 4C). The anterior mental lesion was predominantly radiopaque originating from the base of the anterior mandible and protruding outside the mouth (see Figures 4C and 4D; Figure 5). The right mandibular permanent canine was located at the inferior periphery of this lesion. The radiolucent areas of the lesions showed no significant enhancement after contrast media were administered intravenously. The radiopaque areas of the lesions mostly presented as dense amorphous and gravel-shaped areas with attenuation similar to enamel. Another round and well-defined mass with diameter of 1 cm with internal calcification was attached to the superior aspect of the nasopharyngeal cavity. A biopsy from the mandibular mass revealed odontogenic epithelium with fibrous and myxoid tissue. Taking into account the radiologic and pathologic presentations, a provisional diagnosis of a benign odontogenic tumor with tooth forming capacity was determined. Complex odontoma and ameloblastic fibro-odontoma were considered possible candidates. In consideration that excision or enucleation of all maxillary and mandibular tumors in one operation would result in pathologic fracture, bone defects, and discontinuity and
functional disability, partial excision of the tumors was planned, and the anterior mandibular lesion was removed during the first surgery. The cut surface of the excised specimen was solid, white, and firm. Scattered irregular toothlike hard tissues were observed (Figure 6). Microscopically, the tumor showed irregularly arranged cementum-like hard tissues with areas of cell-rich odontogenic epithelium in the background of fibrous tissue (Figure 7). The diagnosis of a complex odontoma was confirmed. The patient was examined 6 months after the operation. No significant regrowth of the tumor was noted. The patient was requested to make regular follow-up visits to our hospital.
DISCUSSION Although odontoma is a very common odontogenic tumor, MO involving numerous sites of the jaws is not frequently encountered. A review of the Englishlanguage literature found 11 cases (Table I).2,4-8,11-13 In the present case, patients with MO included 5 females and 7 males aged from newborn to 27 years old, with the mean age of 11.5 years. MO can present as a congenital lesion and grow expansively. MO can involve one to four quadrants of the jaws. The tumors may be extensive or composed of several localized lesions. MO can be made up of different histologic types of odontoma, including compound, complex, or both.5 The lesions could be cystic or may develop with increasing enamel and dentin formation within the odontogenic epithelium.12 Stenosis of the esophagus is reported to be associated with MO.7,8 Other related malformations, including stenosis of aortae, sight disorder, and bronchiectasis, have been described.7,8
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Fig. 3. Coronal computed tomography images of two maxillary odontomas (T1 and T2). A, 1.25 mm thickness bone algorithm image. B, 10 mm thickness maximum intensity projection image. A clear boundary is observed between T1 and T2. The permanent lateral incisor (white arrows) and the permanent third molar (white arrowheads) are encapsulated at the caudal periphery of the tumors. The calcifications are heterogeneous and enamel attenuation (black arrowheads) is prominent on the MIP image (B).
Fig. 4. A, Axial bone algorithm image (1.25 mm thickness) shows the two maxillary tumors (T1 and T2) and the boundary between them. A well-demarcated mass (T3) encapsulating the permanent mandibular third molar with coronal calcification is observed in the right ramus. The buccal and lingual cortices are expanded and thinned, but still continuous. BeD, Multi-planar reformatted maximum intensity projection image (10 mm thickness). Closely adjacent to T3 is another lesion (T4) with a permanent mandibular molar at the distal peripheral border and circuitous shell-like calcification (black arrowheads in B and C). C and D, Encapsulating the mandibular canine is the mental mass (T5), protruding anteriorly with circumferential whorled calcification. The calcifications in T3-T5 are heterogeneous, and enamel attenuation can be observed.
Although the etiology of odontoma is still not fully understood, it is presumed to be a hamartoma deriving from aberrance or abnormal invagination of the ectodermal epithelium during embryogenesis.1 Limbal
dermoid, which resulted from abnormal invagination of the developmental epithelium in the present case also, is presumed to share some etiologic association with ipsilateral MO. The primitive maxillary process,
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Fig. 5. Three-dimensional views of the expanded maxilla and deformed mandible. Thinned and perforated cortex and continuous mandibular base are observed.
Fig. 6. Cut surface of the specimen. Note the tumor is solid and mixed with irregular toothlike hard structures (black arrows).
mandibular process, and the optic pit are contiguous in the first branchial arch during the first 3 to 4 weeks of embryogenesis. Local aberrance of ectodermic epithelium could presumably grow into multiple organs and give rise to numerous developmental lesions. The radiologic appearances of odontoma are associated with the stages of development and mineralization.14 With histologic differentiation to enamel and dentin, the correspondingly mixed radiopaque attenuation will appear and increase.15 Completely radiolucent odontomas without calcification are rare. Partial calcification within a cystic radiolucency is frequently observed, indicating a developing lesion. In the mature stage, the calcification occupies most of the tumor, surrounded by a narrow radiolucency.15
The present case adds to our experience with unilateral occurrence in the mandible and the maxilla concurrent with ipsilateral limbal dermoid. CT images, especially the multi-planar reformatted images, help delineate the boundary and spatial relationship of individual tumors; maximum intensity projection images help identify and differentiate enamel attenuations from amorphous calcification; massive sizes and proximity of the tumors may result in holistic merging into a multilocular appearance on panoramic radiographs. MO should be differentiated from bone-related lesions and odontogenic tumors with hard tissue formation. Identification of enamel attenuation is important because it is not present in bone-related lesions, such as fibrous dysplasia and ossifying fibroma. Interference with the impacted and displaced tooth at the peripheral border of the tumor also indicates an odontogenic origin. Malignant lesions, such as osteosarcoma, can be excluded because of the long history, limited and slow growth behavior, and well-demarcated borders of the lesions in the present case. Odontogenic tumors with hard tissue formation, such as the adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, ameloblastic fibro-odontoma, and odontoameloblastoma, may not be easily distinguishable from odontoma. The adenomatoid odontogenic tumor shows a prominent association with unerupted permanent teeth, especially the canines. The radiopacities inside the adenomatoid odontogenic tumor are frequently small, less radiodense, and arranged in circular or irregular clusters. The most characteristic finding of a calcifying epithelial odontogenic tumor is the appearance of radiopacities close to the embedded tooth inside a cystlike radiolucency. Mineralized dental tissues are also formed in ameloblastic fibro-odontoma and odontoameloblastoma, which sometimes cannot be
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Fig. 7. Strands or islands of odontogenic epithelium are observed (A, H&E; 200, arrows; B, H&E; 100, arrows) with cementum-like hard tissue formation (B, arrowheads). The cementum-like hard tissue is arranged in a haphazard pattern (C, arrowheads, H&E; 100) and decalcified enamel-like tissue (D, arrow, H&E; 400) and cementum-like hard tissue (D, arrowhead) are also observed.
Table I. Summary of multiple odontomas in the literature Author(year)
Gender/age
Site
Bader (1967)
F/0-5
4 quadrants
Malik (1974)
F/7
4 quadrants
Mani (1974)
M/19
4 quadrants
Schimidseder (1975)
M/4
4 quadrants
M/newborn
4 quadrants
F/1
Melnick (1975)
M/20
Iwamoto (1999)
F/15
Ajike (2000) Bordini (2008)
F/15 M/17
Erdogan (2014)
M/27
Sun (present study) M/13
Type Compound and complex Compound
Compound and complex Compound
Extensive Extensive in maxilla; localized in mandible Localized Extensive
Developing Localized complex Bilateral maxilla Developing Localized and right ameloblastic mandible fibro-odontoma Right mandibular Compound Localized molar region Bilateral Compound and Localized mandible developing complex 4 quadrants Compound Extensive 4 quadrants Compound Extensive in right maxilla, localized in other 3 quadrants 4 quadrants Compound Extensive
Right maxilla and mandible
Complex
Other abnormality
Size
Extensive
Treatment
Stenosis of esophagus No
Two-staged operation
No
Surgery refused.
Stenosis of esophagus Stenosis of esophagus No
Two-session surgical excision Excision Curettage
No
Excision
No
Excision
No No
Excision Excision
No description
Mild mental Partial excision retardation, sight disorder, severe myopia Limbal dermoid Partial excision Nasal pharyngeal mass
ORAL AND MAXILLOFACIAL RADIOLOGY e16 Sun, Sun and Ma
differentiated from developing complex odontoma radiologically or pathologically. In this consideration, biopsy is needed to consolidate the diagnosis and to exclude the potential malignant transformation. The surgical management of the odontoma should be conservative, and enucleation of the tumor is the first treatment of choice. For massive tumors, two-staged removal can also be considered to preserve mandibular function and decrease the risk of pathologic fracture.7,8,16 CT helps with evaluation of the remnant bone continuity and volume for surgical considerations. Cone beam CT is also recommended because of its relatively low radiation dose and higher spatial resolution.
CONCLUSIONS Multiple odontomas can be local or extensive, involving numerous quadrants of the jaws. CT helps identify enamel attenuation in the amorphous calcification of complex multiple odontoma. REFERENCES 1. Prætorius F, Piattelli A. Odontoma, complex type. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon, France: IARC; 2005:310. 2. Malik SA. Odontomatosis (multiple odontomas)da case report. Br J Oral Surg. 1974;11:262-264. 3. Crincoli V, Scivetti M, Di Bisceglie MB, Lucchese A, Favia G. Odontoma: retrospective study and confocal laser scanning microscope analysis of 52 cases. Minerva Stomatol. 2007;56: 611-620. 4. Melnick M. Odontomatosis. Oral Surg Oral Med Oral Pathol. 1975;40:163. 5. Mani NJ. Odontoma syndrome: report of an unusual case with multiple multiform odontomas of both jaws. J Dent. 1974;2: 149-152. 6. Erdogan O, Keceli O, Oztunc H, Evlice B, Altug HA, Gunhan O. Compound odontoma involving the four quadrants of the jaws: a
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7. 8.
9.
10.
11.
12.
13. 14. 15.
16.
case report and review of the literature. Quintessence Int. 2014;45:341-344. Bader G. Odontomatosis (multiple odontomas). Oral Surg Oral Med Oral Pathol. 1967;23:770-773. Schmidseder R, Hausamen JE. Multiple odontogenic tumors and other anomalies. An autosomal dominantly inherited syndrome. Oral Surg Oral Med Oral Pathol. 1975;39:249-258. Lee BD, Lee W, Oh SH, Min SK, Kim EC. A case report of Gardner syndrome with hereditary widespread osteomatous jaw lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107:e68-e72. Ramaglia L, Morgese F, Filippella M, Colao A. Oral and maxillofacial manifestations of Gardner’s syndrome associated with growth hormone deficiency: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;103:e30-e34. Bordini J Jr, Contar CM, Sarot JR, Fernandes A, Machado MA. Multiple compound odontomas in the jaw: case report and analysis of the literature. J Oral Maxillofac Surg. 2008;66:2617-2620. Iwamoto O, Harada H, Kusukawa J, Kameyama T. Multiple odontomas of the mandible: a case report. J Oral Maxillofac Surg. 1999;57:338-341. Ajike SO, Adekeye EO. Multiple odontomas in the facial bones. A case report. Int J Oral Maxillofac Surg. 2000;29:443-444. An SY, An CH, Choi KS. Odontoma: a retrospective study of 73 cases. Imaging Sci Dent. 2000;42:77-81. White SC, Pharoah MJ. Benign tumors of the jaws. In: White SC, Pharoah MJ, eds. Oral Radiology Principles and Interpretation. St. Louis, MO: Mosby; 2009:378-381. Chrcanovic BR, Jaeger F, Freire-Maia B. Two-stage surgical removal of large complex odontoma. Oral Maxillofac Surg. 2010;14:247-252.
Reprint requests: Zhipeng Sun, MD Department of Oral and Maxillofacial Radiology School and Hospital of Stomatology Peking University 22 South Zhongguancun Avenue Haidian District Beijing 100081 P. R. China [email protected]
Multiple complex odontoma of the maxilla and the mandible Lisha Sun, PhD,a Zhipeng Sun, MD,b and Xuchen Ma, PhDb Objective. The aim of this study was to present a literature review and case report of multiple odontoma. Methods. A case of multiple odontoma is presented with a review of the English-language literature. The clinical and radiologic features are summarized. Results. In total, 12 cases of multiple odontoma were retrieved from the literature for analysis. The multiple odontomas were localized or extensive and involved two to four quadrants of the jaws. The histologic types were compound or complex. Conclusion. Odontomas can be multiple and involve multiple quadrants of the jaws. (Oral Surg Oral Med Oral Pathol Oral Radiol 2015;120:e11-e16)
Odontoma is one of the most common odontogenic tumors and usually involves the tooth-bearing regions of the jaw.1 There are two basic types: compound and complex. The compound odontoma forms multiple irregular toothlike structures, whereas the complex odontoma forms amorphous calcification, with dysplastic dentin covered by enamel.1 Both compound and complex odontomas mostly occur as solitary lesions in the jaw. Multiple odontoma (MO) is characterized by numerous odontomas involving one to four quadrants of the jaws.2-6 MO also can occur with other malformations, such as stenosis of the esophagus.7,8 The terms “odontomatosis” and “odontoma syndrome” have been suggested to describe MO.2,5 Due to its rarity, little is known about the comprehensive clinical features of MO. Radiologic diagnosis of a single or multiple compound odontoma based on the presence of characteristic toothlike structures is not difficult. However, multiple and massive complex odontomas, exhibiting amorphous calcification, raise many possible differential diagnoses and constitute a diagnostic and therapeutic challenge. Fibrous dysplasia can frequently present as multiple expansive lesions with mixed radiopaque and radiolucent density involving both the maxilla and the mandible. Florid osseous dysplasia lesions can be bilateral and occur in both jaws apical to teeth, with varying densities ranging from an equal mixture of radiolucent and radiopaque regions to almost complete radiopacity. Ossifying fibroma can also occasionally be multiple and massive, showing well-fined tumors with internal calcifications. Gardner syndrome can present with multiple osteomatous jaw lesions,9 dental anomalies, or epidermoid cysts as extracolonic lesions.10 a
Key Laboratory of Oral Pathology, School and Hospital of Stomatology, Peking University, Beijing, China. b Department of Oral and Maxillofacial Radiology, School and Hospital of Stomatology, Peking University, Beijing, China. Received for publication Sep 17, 2014; returned for revision Feb 17, 2015; accepted for publication Feb 20, 2015. Ó 2015 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2015.02.488
We report a case of multiple complex odontoma with a review of the related literature.
CASE REPORT A 14-year-old male complaining of a large oral tumor was referred to our hospital. The tumor was small when it was discovered at birth. It grew slowly and expansively. No treatment had been delivered. Progressive swallowing and masticatory difficulties prompted the patient to seek treatment. The family history was unremarkable. General physical examination showed that the patient was undernourished. Routine blood test indicated anemia with low hemoglobin (117 g/L), low hematocrit (37% L/L), low mean corpuscular volume (77.5 fL), low mean content of hemoglobin (24.6 pg), and high platelet hematocrit (0.37%). A bulbous mass of approximately 6 cm in diameter was noted protruding from the anterior mandible with intact overlying mucosa (Figure 1). The tumor was hard, nontender, and fixed to the mandible. The cheek and the infraorbital areas were expanded with normal facial skin. Intraoral examination showed that the right mandible was expanded from the midline to the ramus. The right maxilla was expanded from the midline to the tuberosity. The lesions in the mandible and the maxilla were hard and nontender, and the mucosa was intact. Ocular examination demonstrated a single elevated inferotemporal limbal mass extending approximately 5 mm into the cornea from the limbus. The patient was referred to an ophthalmologist, and the mass was diagnosed as a limbal dermoid. Panoramic radiography (Figure 2) and spiral computed tomography (CT) (Figure 3) revealed two well-defined adjacent lesions in the right maxilla. The anterior lesion extended around the impacted permanent maxillary right lateral incisor, from the floor of the nasal cavity to the anterior alveolar bone. It was mainly radiolucent, with some calcifications at the inferior aspect. The other maxillary lesion extended around the caudally displaced permanent maxillary left third molar, along the anterior wall of the maxillary sinus to the buccal alveolar bone. The permanent maxillary lateral incisor and the third permanent molar were both located at the peripheral borders of the tumors. The permanent maxillary right canine was also impacted and located outside the maxillary lesions. Three closely adjacent mandibular lesions were observed on CT (Figure 4). The posterior lesion in the ramus appeared as a well-demarcated radiolucency with the impacted permanent
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Fig. 2. Panoramic radiograph of the patient. Two tumors are observed in the right maxilla. The permanent maxillary right lateral incisor (arrow 1) is caudally displaced and surrounded by a radiolucent capsule with a well-defined border and calcification at the bottom. The permanent maxillary right third molar (arrow 2) is displaced and superimposed by a well-defined calcified mass. The mandibular tumors present as multilocular lesions with mixed density. Two permanent mandibular right molars (arrows 3 and 4) and canine (arrow 5) are impacted inside the lesions, and another permanent mandibular molar (arrow 6) is displaced.
Fig. 1. Clinical facial photo of the patient. Note the oral mass protruding from the anterior mandible and the inferotemporal limbal mass on the right side. third molar and coronal calcification. The right ramus was expanded, and the cortex was continuous and thinned (see Figures 4A and 4B). The middle lesion in the molar and premolar region was made up of circumferentially intermingled radiolucent and radiopaque areas around a peripherally displaced permanent molar (see Figures 4B and 4C). The anterior mental lesion was predominantly radiopaque originating from the base of the anterior mandible and protruding outside the mouth (see Figures 4C and 4D; Figure 5). The right mandibular permanent canine was located at the inferior periphery of this lesion. The radiolucent areas of the lesions showed no significant enhancement after contrast media were administered intravenously. The radiopaque areas of the lesions mostly presented as dense amorphous and gravel-shaped areas with attenuation similar to enamel. Another round and well-defined mass with diameter of 1 cm with internal calcification was attached to the superior aspect of the nasopharyngeal cavity. A biopsy from the mandibular mass revealed odontogenic epithelium with fibrous and myxoid tissue. Taking into account the radiologic and pathologic presentations, a provisional diagnosis of a benign odontogenic tumor with tooth forming capacity was determined. Complex odontoma and ameloblastic fibro-odontoma were considered possible candidates. In consideration that excision or enucleation of all maxillary and mandibular tumors in one operation would result in pathologic fracture, bone defects, and discontinuity and
functional disability, partial excision of the tumors was planned, and the anterior mandibular lesion was removed during the first surgery. The cut surface of the excised specimen was solid, white, and firm. Scattered irregular toothlike hard tissues were observed (Figure 6). Microscopically, the tumor showed irregularly arranged cementum-like hard tissues with areas of cell-rich odontogenic epithelium in the background of fibrous tissue (Figure 7). The diagnosis of a complex odontoma was confirmed. The patient was examined 6 months after the operation. No significant regrowth of the tumor was noted. The patient was requested to make regular follow-up visits to our hospital.
DISCUSSION Although odontoma is a very common odontogenic tumor, MO involving numerous sites of the jaws is not frequently encountered. A review of the Englishlanguage literature found 11 cases (Table I).2,4-8,11-13 In the present case, patients with MO included 5 females and 7 males aged from newborn to 27 years old, with the mean age of 11.5 years. MO can present as a congenital lesion and grow expansively. MO can involve one to four quadrants of the jaws. The tumors may be extensive or composed of several localized lesions. MO can be made up of different histologic types of odontoma, including compound, complex, or both.5 The lesions could be cystic or may develop with increasing enamel and dentin formation within the odontogenic epithelium.12 Stenosis of the esophagus is reported to be associated with MO.7,8 Other related malformations, including stenosis of aortae, sight disorder, and bronchiectasis, have been described.7,8
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Fig. 3. Coronal computed tomography images of two maxillary odontomas (T1 and T2). A, 1.25 mm thickness bone algorithm image. B, 10 mm thickness maximum intensity projection image. A clear boundary is observed between T1 and T2. The permanent lateral incisor (white arrows) and the permanent third molar (white arrowheads) are encapsulated at the caudal periphery of the tumors. The calcifications are heterogeneous and enamel attenuation (black arrowheads) is prominent on the MIP image (B).
Fig. 4. A, Axial bone algorithm image (1.25 mm thickness) shows the two maxillary tumors (T1 and T2) and the boundary between them. A well-demarcated mass (T3) encapsulating the permanent mandibular third molar with coronal calcification is observed in the right ramus. The buccal and lingual cortices are expanded and thinned, but still continuous. BeD, Multi-planar reformatted maximum intensity projection image (10 mm thickness). Closely adjacent to T3 is another lesion (T4) with a permanent mandibular molar at the distal peripheral border and circuitous shell-like calcification (black arrowheads in B and C). C and D, Encapsulating the mandibular canine is the mental mass (T5), protruding anteriorly with circumferential whorled calcification. The calcifications in T3-T5 are heterogeneous, and enamel attenuation can be observed.
Although the etiology of odontoma is still not fully understood, it is presumed to be a hamartoma deriving from aberrance or abnormal invagination of the ectodermal epithelium during embryogenesis.1 Limbal
dermoid, which resulted from abnormal invagination of the developmental epithelium in the present case also, is presumed to share some etiologic association with ipsilateral MO. The primitive maxillary process,
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Fig. 5. Three-dimensional views of the expanded maxilla and deformed mandible. Thinned and perforated cortex and continuous mandibular base are observed.
Fig. 6. Cut surface of the specimen. Note the tumor is solid and mixed with irregular toothlike hard structures (black arrows).
mandibular process, and the optic pit are contiguous in the first branchial arch during the first 3 to 4 weeks of embryogenesis. Local aberrance of ectodermic epithelium could presumably grow into multiple organs and give rise to numerous developmental lesions. The radiologic appearances of odontoma are associated with the stages of development and mineralization.14 With histologic differentiation to enamel and dentin, the correspondingly mixed radiopaque attenuation will appear and increase.15 Completely radiolucent odontomas without calcification are rare. Partial calcification within a cystic radiolucency is frequently observed, indicating a developing lesion. In the mature stage, the calcification occupies most of the tumor, surrounded by a narrow radiolucency.15
The present case adds to our experience with unilateral occurrence in the mandible and the maxilla concurrent with ipsilateral limbal dermoid. CT images, especially the multi-planar reformatted images, help delineate the boundary and spatial relationship of individual tumors; maximum intensity projection images help identify and differentiate enamel attenuations from amorphous calcification; massive sizes and proximity of the tumors may result in holistic merging into a multilocular appearance on panoramic radiographs. MO should be differentiated from bone-related lesions and odontogenic tumors with hard tissue formation. Identification of enamel attenuation is important because it is not present in bone-related lesions, such as fibrous dysplasia and ossifying fibroma. Interference with the impacted and displaced tooth at the peripheral border of the tumor also indicates an odontogenic origin. Malignant lesions, such as osteosarcoma, can be excluded because of the long history, limited and slow growth behavior, and well-demarcated borders of the lesions in the present case. Odontogenic tumors with hard tissue formation, such as the adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, ameloblastic fibro-odontoma, and odontoameloblastoma, may not be easily distinguishable from odontoma. The adenomatoid odontogenic tumor shows a prominent association with unerupted permanent teeth, especially the canines. The radiopacities inside the adenomatoid odontogenic tumor are frequently small, less radiodense, and arranged in circular or irregular clusters. The most characteristic finding of a calcifying epithelial odontogenic tumor is the appearance of radiopacities close to the embedded tooth inside a cystlike radiolucency. Mineralized dental tissues are also formed in ameloblastic fibro-odontoma and odontoameloblastoma, which sometimes cannot be
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Fig. 7. Strands or islands of odontogenic epithelium are observed (A, H&E; 200, arrows; B, H&E; 100, arrows) with cementum-like hard tissue formation (B, arrowheads). The cementum-like hard tissue is arranged in a haphazard pattern (C, arrowheads, H&E; 100) and decalcified enamel-like tissue (D, arrow, H&E; 400) and cementum-like hard tissue (D, arrowhead) are also observed.
Table I. Summary of multiple odontomas in the literature Author(year)
Gender/age
Site
Bader (1967)
F/0-5
4 quadrants
Malik (1974)
F/7
4 quadrants
Mani (1974)
M/19
4 quadrants
Schimidseder (1975)
M/4
4 quadrants
M/newborn
4 quadrants
F/1
Melnick (1975)
M/20
Iwamoto (1999)
F/15
Ajike (2000) Bordini (2008)
F/15 M/17
Erdogan (2014)
M/27
Sun (present study) M/13
Type Compound and complex Compound
Compound and complex Compound
Extensive Extensive in maxilla; localized in mandible Localized Extensive
Developing Localized complex Bilateral maxilla Developing Localized and right ameloblastic mandible fibro-odontoma Right mandibular Compound Localized molar region Bilateral Compound and Localized mandible developing complex 4 quadrants Compound Extensive 4 quadrants Compound Extensive in right maxilla, localized in other 3 quadrants 4 quadrants Compound Extensive
Right maxilla and mandible
Complex
Other abnormality
Size
Extensive
Treatment
Stenosis of esophagus No
Two-staged operation
No
Surgery refused.
Stenosis of esophagus Stenosis of esophagus No
Two-session surgical excision Excision Curettage
No
Excision
No
Excision
No No
Excision Excision
No description
Mild mental Partial excision retardation, sight disorder, severe myopia Limbal dermoid Partial excision Nasal pharyngeal mass
ORAL AND MAXILLOFACIAL RADIOLOGY e16 Sun, Sun and Ma
differentiated from developing complex odontoma radiologically or pathologically. In this consideration, biopsy is needed to consolidate the diagnosis and to exclude the potential malignant transformation. The surgical management of the odontoma should be conservative, and enucleation of the tumor is the first treatment of choice. For massive tumors, two-staged removal can also be considered to preserve mandibular function and decrease the risk of pathologic fracture.7,8,16 CT helps with evaluation of the remnant bone continuity and volume for surgical considerations. Cone beam CT is also recommended because of its relatively low radiation dose and higher spatial resolution.
CONCLUSIONS Multiple odontomas can be local or extensive, involving numerous quadrants of the jaws. CT helps identify enamel attenuation in the amorphous calcification of complex multiple odontoma. REFERENCES 1. Prætorius F, Piattelli A. Odontoma, complex type. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon, France: IARC; 2005:310. 2. Malik SA. Odontomatosis (multiple odontomas)da case report. Br J Oral Surg. 1974;11:262-264. 3. Crincoli V, Scivetti M, Di Bisceglie MB, Lucchese A, Favia G. Odontoma: retrospective study and confocal laser scanning microscope analysis of 52 cases. Minerva Stomatol. 2007;56: 611-620. 4. Melnick M. Odontomatosis. Oral Surg Oral Med Oral Pathol. 1975;40:163. 5. Mani NJ. Odontoma syndrome: report of an unusual case with multiple multiform odontomas of both jaws. J Dent. 1974;2: 149-152. 6. Erdogan O, Keceli O, Oztunc H, Evlice B, Altug HA, Gunhan O. Compound odontoma involving the four quadrants of the jaws: a
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7. 8.
9.
10.
11.
12.
13. 14. 15.
16.
case report and review of the literature. Quintessence Int. 2014;45:341-344. Bader G. Odontomatosis (multiple odontomas). Oral Surg Oral Med Oral Pathol. 1967;23:770-773. Schmidseder R, Hausamen JE. Multiple odontogenic tumors and other anomalies. An autosomal dominantly inherited syndrome. Oral Surg Oral Med Oral Pathol. 1975;39:249-258. Lee BD, Lee W, Oh SH, Min SK, Kim EC. A case report of Gardner syndrome with hereditary widespread osteomatous jaw lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107:e68-e72. Ramaglia L, Morgese F, Filippella M, Colao A. Oral and maxillofacial manifestations of Gardner’s syndrome associated with growth hormone deficiency: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;103:e30-e34. Bordini J Jr, Contar CM, Sarot JR, Fernandes A, Machado MA. Multiple compound odontomas in the jaw: case report and analysis of the literature. J Oral Maxillofac Surg. 2008;66:2617-2620. Iwamoto O, Harada H, Kusukawa J, Kameyama T. Multiple odontomas of the mandible: a case report. J Oral Maxillofac Surg. 1999;57:338-341. Ajike SO, Adekeye EO. Multiple odontomas in the facial bones. A case report. Int J Oral Maxillofac Surg. 2000;29:443-444. An SY, An CH, Choi KS. Odontoma: a retrospective study of 73 cases. Imaging Sci Dent. 2000;42:77-81. White SC, Pharoah MJ. Benign tumors of the jaws. In: White SC, Pharoah MJ, eds. Oral Radiology Principles and Interpretation. St. Louis, MO: Mosby; 2009:378-381. Chrcanovic BR, Jaeger F, Freire-Maia B. Two-stage surgical removal of large complex odontoma. Oral Maxillofac Surg. 2010;14:247-252.
Reprint requests: Zhipeng Sun, MD Department of Oral and Maxillofacial Radiology School and Hospital of Stomatology Peking University 22 South Zhongguancun Avenue Haidian District Beijing 100081 P. R. China [email protected]