- Email: [email protected]
Clinicopathologic correlation: a mixed radio-opaque and radiolucent lesion of the posterior maxilla
Accepted Manuscript A mixed radio-opaque and radiolucent lesion of the posterior maxilla Chan M. Park, DDS, MD Jacob S. Barber, DDS Nasser Said-Al-Naief, DDS, MS PII:
S2212-4403(14)00371-X
DOI:
10.1016/j.oooo.2014.03.003
Reference:
OOOO 878
To appear in:
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Received Date: 7 August 2013 Revised Date:
4 February 2014
Accepted Date: 9 March 2014
Please cite this article as: Park CM, Barber JS, Said-Al-Naief N, A mixed radio-opaque and radiolucent lesion of the posterior maxilla, Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology (2014), doi: 10.1016/j.oooo.2014.03.003. 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.
ACCEPTED MANUSCRIPT
Clinicopathologic Correlation
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A mixed radio-opaque and radiolucent lesion of the posterior maxilla
Chan M Park DDS, MD1, Jacob S Barber DDS2, and Nasser Said-Al-Naief DDS, MS3
Director, University of the Pacific School of Dentistry, Highland Hospital,
Division of Oral and Maxillofacial Surgery, Oakland CA
Resident, University of the Pacific School of Dentistry, Highland Hospital,
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2Chief
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1 Program
Division of Oral and Maxillofacial Surgery, Oakland CA 3 Associate
Professor, University of the Pacific School of Dentistry, Pacific Oral and
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Maxillofacial Pathology Department, San Francisco CA
Address Correspondence to: Chan M Park DDS, MD Division of Oral and Maxillofacial Surgery Highland Hospital, Alameda County Medical Center 1411 E 31st Street, Oakland, Ca 94602 Telephone: 510-437-8523
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Fax: 510-535-7761 Email: [email protected] Clinical Presentation:
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A 48 year old male was referred by the general dentist to the oral and
maxillofacial surgery clinic at Highland Hospital in Oakland, California for evaluation of an asymptomatic left maxillary swelling and management of an impacted left
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maxillary third molar. The swelling had been present for an unknown duration. On physical examination, there were no extraoral findings or lymphadenopathy.
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Intraorally, there was a firm buccal and palatal expansion in the left posterior maxilla obscuring the visibility of his left maxillary molars (Figure 1). Aside from the notable poor dentition, the rest of the intraoral examination was unremarkable. The medical history was significant for schizophrenia, type II diabetes, and
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asthma. Two months prior to our evaluation of the patient, he was hospitalized for a deep venous thrombosis and a pulmonary embolism. The patient’s medications included lisinopril, Advair (fluticasone/salmeterol), aspirin, Lovenox (enoxaparin),
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risperdone, and bupropion.
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A panoramic radiograph illustrated an impacted grossly deformed left maxillary third molar and an approximately 4x3 centimeter diffuse mixed radiolucent and radio-opaque lesion within the left posterior maxilla (Figure 2). A computed tomograph of the maxillofacial region demonstrated a 4x4x3cm
poorly-circumscribed mixed radiolucent and radio-opaque lesion extending from the distal of the left maxillary second premolar to the pterygoid plates (Figure 3a-b). The maxillary sinus was displaced superiorly.
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Differential Diagnosis: Several entities were entertained in the differential diagnosis of the current
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lesion, based on the physical examination and radiographs. Odontoma is a very common odontogenic tumor. In a study by Buchner et al, 75.9% of all 1,088
odontogenic tumors identified were odontomas.1 There are two classical types:
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compound, which appears tooth-like, and the complex, which are radio-opaque and are a mixture of hard and soft dental tissue.2 Radiographically this case did appear
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to have an impacted tooth, with radio-opacities surrounding it, which could be a compound odontoma. Odontoma is considered in the differential diagnosis because it commonly is involved with impacted teeth or unerupted teeth, and is asymptomatic, just as this case presented. The lowest frequency of odontomas is
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found in the posterior maxilla(13%), with the highest incidence in the anterior maxilla(37%). Odontomas normally are identified in younger patients, which is not
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consistent with this case, although that does not rule it out.
Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg
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tumor, was also on our differential diagnosis. CEOTs are benign odontogenic epithelial tumors, most likely derived from the reduced enamel epithelium, stratum intermedium, or dental lamina epithelium.3 CEOTs may occur as the more common central type or as an extraosseous (peripheral) lesion. The radiographic appearance of an impacted tooth associated with small diffuse surrounding calcifications within a poorly-defined radiolucent lesion is suggestive of CEOT, which is how this case presents radiographically. CEOTs are 3 times more likely to be found in the
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mandible than the maxilla, with Neville, et al., reporting that the posterior maxilla is the location of 21% of CEOTs.4 This lesion is in the posterior maxilla, which makes it less likely to be CEOT. The clinical presentation of a CEOT is a slowly enlarging
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painless mass that occurs in a pericoronal location or prohibits the eruption of a tooth,5 which is consistent with how this patient presented to our clinic.
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Ameloblastic fibro-odontoma (AFO) a painless, slow growing, oftenasymptomatic mixed tumor very similar to the onset in this patient. The
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distribution of these tumors is evenly split between the maxilla and mandible, without gender preference. Radiographically, AFO may appear as a unilocular or multilocular radiolucent lesion with a well-corticated border.1 They contain radioopaque contents of various opacity, and often are associated with an unerupted
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tooth.6,7 AFOs mainly occur in children in the 1st or 2nd decades of life with an average age of nine8. Due to the young age prediliction it makes this lesion less likely to be the culprit in our patient.
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Odontoameloblastoma, previously named the ameloblastic odontoma is an
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extremely rare tumor. It characteristically presents as a locally destructive, slow growing, radiolucent lesion that often contains tooth-like radiopacities.4 There have been few cases documented, and generally, they occur in a wide age range; from infancy up until the 5th decade, with predilection for the posterior mandible.9,10 Based on the similar documented age range at diagnosis, clinical presentation, and radiographic appearance, the odontoameloblastoma must be entertained in our patient.
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Calcifying odontogenic cyst (COC) was also included in the differential diagnosis. COC involves patients with a wide age range and involves upper and lower jaws with equal frequency, with prevalence for the anterior jaw. This
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asymptomatic lesion has a radiopaque outline with calcifications internally and
typically occurs in a pericoronal location or in association with an unerupted teeth.2
jaw makes our case less likely to be a COC.
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Radiographically the COC mimics our lesion; however, its preference for the anterior
Dentinogenic ghost cell tumor (DGCT) is an extremely rare odontogenic
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tumor, which is accepted by most as the solid variant of COC. Histologically these tumors display an infiltrative solid growth pattern composed of odontogenic epithelium mixed with ghost cells and dentinoid ground substance.11 DGCT involves males and females with equal frequency and equally involves the maxilla and
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mandible, with predilection towards the anterior jaws. Additionally, it is typically seen in patients less than 30 years of age. Radiographically, it appears as a well defined or poorly circumscribed unilocular or a multilocular radiolucent lesion with
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various amounts of calcifications.12 Radiographically, the DGCT represents our
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patient’s lesion but the age during presentation and likelihood to be in the anterior jaws makes it lower on our differential list. Adenomatoid odontogenic tumor (AOT) has been reported to be the fifth
most common odontogenic tumor. It is an asymptomatic radiolucent lesion with occasional radio-opacities within the lesion that may cause slow cortical displacement.2 It occurs with a sex predilection for women of nearly 2:1 and is usually in younger patients.13 Our patient is in his 5th decade of life, which makes it
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unlikely to be this tumor and, normally, AOT is found in the anterior jaws of females. Calcification of the tumor consists of irregular calcifications that demonstrate a Liesegang pattern similar to the CEOT.14
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Desmoplastic ameloblastoma (DA) was also considered in our differential, as it has been known to be mistaken for a mixed benign fibro-osseous lesion
radiographically. DA was first described in 1984 by Eversole, et al15 and many
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similar cases have been reported since then. DA can affect people of all ages;
however, it affects people more commonly in the range of 30-40 years of age. They
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are often radiolucent, but most commonly present with a mixed radiolucent/radioopaque pattern and may appear partially calcified, with poorly-defined borders. The presenting sign is often asymptomatic expansion. The lesion has a high tendency to be in the anterior portion of the jaw, with no predilection for either
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jaw.16 Again, the DA radiographically and demographically presents like our case, but the predilection for the anterior jaw makes it less likely. Based on this patient’s clinical presentation, symptoms, and radiographic
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appearance, a benign odontogenic neoplasm was our working diagnosis. The
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duration of the maxillary expansion was unknown, but the diagnosis was strengthened by the fact that it was painless, involved a tooth and had a mixedradio-opaque presentation on radiographs.
Diagnosis and Management An incisional biopsy showed sheets of round and polyhedral cells which exhibited clearly-evident intercellular bridges. Mild but generalized cellular pleomorphism
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and patchy nuclear hyperchromatism was appreciated throughout. Concentric calcifications were also present throughout, and both epithelial and calcified elements were intimately associated with and were supported by a homogeneous
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amorphous material. It stained positively for Congo red special stain and showed
birefringent apple green hue on polarization, confirming amyloid origin (Figures 46). The histopathology of the lesion led to a diagnosis of a calcified epithelial
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odontogenic tumor (CEOT). Subsequently, a left hemi-maxillectomy was performed (Figure 7). The patient was followed post-operatively and was last seen at one
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year, at which there was no evidence of recurrence of disease.
Discussion:
Calcified epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, was
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first described by Pindborg in 1955.17 Pindborg went on to publish with Franklin in 1976 a paper characterizing 113 CEOTs that had been reported since 1946.18 They found CEOT to be twice as likely to occur in the mandible than the maxilla, and
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usually in the molar-premolar region. The current literature states there is a ratio of
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3:1 in the mandible to the maxilla.8,19 They found the mean age to be 40 years, with equal gender predilection.18 The typical clinical presentation of a CEOT is a locally aggressive, painless, and slowly-enlarging mass. Radiographically, they appear radiolucent with flecks of radio-opaque calcifications.3 The patient in this report described a similar slow onset of a painless maxillary mass, and his age is near the mean age. The radiographic appearance also led us to be suspicious of a CEOT.
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CEOT presents with distinct histomorphologic features: solid sheets, trabeculae or nests of monotonous-appearing epithelial cells with well-formed intercellular bridges. They may potentially show generalized cellular
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pleomorphism; however, mitotic activity is typically not observed. A variable
amount of concentric calcification referred to as “Liesegang rings” is seen. However, tumors may lack any evidence of calcifications.20 Of significance is the presence of
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amyloid deposits within these tumors, which can be confirmed by special stain
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Congo red21 or crystal violet22 and immunohistochemical staining for thioflavin T.23
There has been debate in the literature regarding the proper treatment of CEOTs. Some authors feel it represents a locally aggressive lesion,24 while others feel it is an expansile tumor that does not invade the trabeculation of medullary
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bone25. Demian, et al., in 2010 published a case report of a CEOT with malignant transformation and discussed 7 others previously reported in the literature.26 Enucleation and curettage may only be a reasonable option for very small tumors,
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less than 1.5 cm. There are no studies examining what is an adequate bony margin to take. Based on the aggressive and infiltrative nature of the tumor, resection with
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a rim of normal tissue is currently recommended.8,19 If there are clear cells present, larger surgical margins are indicated, because clear cell odontogenic tumors are thought to behave more aggressively than the conventional CEOT27. If intraoperative frozen studies are performed, a smaller excision margin is acceptable, assuming margins are negative.
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References 1. Buchner A, Phillip MW, Carpenter WM. Relative frequency of central odontogenic tumors: a study of 1088 cases from northern California and
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comparison to studies from other parts of the world. J Oral Maxillofac Surg 2006; 64(9):1343-52.
2. White DK, Street CC, Jenkins WS, Clark AR, Ford JE. Panoramic radiograph in
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pathology. Atlas Oral Maxillofacial Surg Clin N Am 2003;11(1): 1-53.
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3. Sapp J, Eversole L, Wysocki G. Odontogenic Tumors. In: Rudolph P, Alvis K, editors. Contemporary Oral and Maxillofacial Pathology. St. Louis, Missouri Mosby; 2004. p. 143-5.
4. Neville B, Damm D Allen CM, Bouquot JE. Odontogenic Cysts and Tumors. In:
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Rudolph P, Alvis K, Forest E, editors. Oral and Maxillofacial Pathology. Philadelphia, Pennsylvania Saunders Company; 2002 p. 623-625.
5. Nakano H, Ota Y, Yura Y. Calcifying epithelial odontogenic tumor of the maxilla
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with ulcerative stomatitis: a case report. Br J Oral Maxillofac Surg 2009;47(3):222-4.
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6. De Riu G, Meloni SM, Contini M, Tullio A. Ameloblastic fibro-odontoma: case report and review of the literature. J Craniomaxillofac Surg 2010;38(2):141-4. 7. Zouhary KJ, Said-Al-Naief N, Waite PD. Ameloblastic fibro-odontoma: expansile mixed radiolucent lesion in the posterior maxilla: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106(4):15-21. 8. Hu Y, Liu B, Su T, Zhang W, Zhao. A huge ameloblastic fibro-odontoma of the maxilla. Oral Oncol 2006;42(4):160-2.
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9. Sapru BL, Dasgupta D, Rajaram. Odontoameloblastoma: a rare odontogenic tumor. Med J Arm Forc India 2001;57(4):333-4.
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10. Mosqueda-Taylor A, Carlos-Bregni R, Ramirez-Amador V, Palma-Guzman JM,
Esquivel-Bonilla D, Hernandez-Rojas LA. Odontoameloblastoma. Clinico-pathologic study of three cases and critical review of the literature. Oral Oncol
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2002;38(8):800-5.
11. Stone CH, Gaba AR, Benninger MS, Zarbo RJ. Odontogenic ghost cell tumor: a
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case report with cytological findings. Diagn Cytopathol 1998;18(2):199-203. 12. Chindasombatjaroen J, Kakimoto N, Akiyama H, Kubo K, Murakami S, Furukawa S, Kishino M. Computerized tomography observation of a calcifying cystic odontogenic tumor with an odontoma: case report. Oral Surg Oral Med Oral Pathol
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Oral Radiol Endod 2007;104:52-7.
13. Suzukia H, Hashimoto K. Adenomatoid odontogenic tumour of the maxilla: immunohistochemical study. Asian J Oral Maxillofac Surg 2005;17(4):267-71.
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14. Philipsen HP, Reichart PA. Adenomatoid odontogenic tumour: facts and figures.
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Oral Oncol 1999;35(2):125-131. 15. Eversole LR, Leider AS, Strub D. Radiographic characteristics of cystogenic ameloblastoma. Oral Surg Oral Med Oral Pathol 1984;57(5):572-7.
16. Said-Al-Naief N. Updates in Diagnostic Pathology. Odontogenic Tumors for General Pathologists. Adv Exp Med Biol. 2005;563:148-64. 17.. Pindborg JJ: Calcifying epithelial odontogenic tumor. Acta Path Micro Stand. 1955;111:71.
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18. Franklin CD, Pindborg JJ. The calcifying epithelial odontogenic tumor: A review and analysis of 113 Cases. Oral Surg 1976;42(7):53.
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19. Marciani RD, Carlson ER, Braun TW. Trauma Surgical Pathology Temporomandibular Disorders. Oral and Maxillofacial Surgery 2nd Ed Vol II; 2009. p. 473-475.
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20. Takata T, Ogawa I, Miyauchi M, Ijuhin N, Nikai H, Fujita M. Non-calcifying
pindborg tumor with langerhans cells. J Oral Pathol Med. 1993;22(8):378-83
Cytochem 1962;10:355.
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21.. Holde P, Sweat F. On the binding of congo red by amyloid. J Histochem
22.. Askanas V, Engel WK, Alvarez RB. Light and electron microscopic localization of beta-amyloid protein in muscle biopsies of patients with inclusion-body myositis.
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Am J Pathol 1992;141(1):31-6.
23. LeVine III H. Quantification of B-sheet amyloid fibril structures with thioflavin T. Methods Enzymol 1999;309:274-84.
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43.
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24. Pindborg JJ. A calcifying epithelial odontogenic tumor. Cancer 1958;11:838-
25. Vap DR, Dahlin DC, Turlington EG. Pindborg tumor: the so-called calcifying epithelial odontogenic tumor. Cancer 1970;25:629-36. 26. Demian N, Harris RJ, Abramovitch K, Wilson JW, Vigneswaran N. Malignant transformation of calcifying epithelial odontogenic tumor is associated with loss of p53 transcriptional activity: a case report with review of the literature. J Oral Maxillofac Surg 2010;68(8):1964-73.
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27. Hicks MJ, Flaitz CM, Wong MEK, McDaniel RK, Cagle PT. Clear cell variant of calcifying epithelial odontogenic tumor: case report and review of the literature.
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Head Neck 1994;16(3):272-7.
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Legends
Figure 1. The left maxillary expansile lesion extending from the buccal vestibule
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into the palate.
Figure 2. At the initial examination a panoramic radiographic shows a left maxillary
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posterior mixed lesion.
Figure 3.
3a. An axial computed tomogram (CT) slice at the level of the maxillary mid root
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radiolucency.
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demonstrates a thinned cortical border with radio-opacities present within the
3b. A sagittal CT slice of the same area.
Figure 4. A low power photomicrograph demonstrates the presence of an epithelial tumor, composed of large sheets of cells (black solid arrow), interspersed by generalized noticeable amount of calcification (hollow arrow) (hematoxylin and eosin stain; original magnification X100).
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Figure 5. 5a. This highlights the presence of clusters and sheets of epithelial cells intimately
(hematoxylin and eosin stain; original magnification X200).
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juxtaposed withe concentric calcifications and homogenous pink-staining material
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5b. A higher power photomicrograph demonstrates dense sheets of epithelium which exhibit well-formed intercellular bridges and mild generalized cellular
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pleomorphism (white arrow) and intimately surrounding concentric type calcifications (black arrow); referred to as “Liesegang rings” (hematoxylin and eosin stain; original magnification X400).
5c. A lower power view shows homogeneous acellular material (arrow); proven to
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be amyloid in origin (hematoxylin and eosin stain; original magnification X200). 5d. Positive staining of the homogeneous acellular material (arrow) with Congo red stain, characteristic of and confirming the presence of amyloid
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(Special stain; Congo red, original magnification X400).
Figure 6. A low power photomicrograph demonstrates the presence of generalized apple-green birefringent pattern, characteristic of polarized amyloid material (Congo red special stain; polarized light, original magnification X100).
Figure 7. The gross excised specimen.
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S2212-4403(14)00371-X
DOI:
10.1016/j.oooo.2014.03.003
Reference:
OOOO 878
To appear in:
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Received Date: 7 August 2013 Revised Date:
4 February 2014
Accepted Date: 9 March 2014
Please cite this article as: Park CM, Barber JS, Said-Al-Naief N, A mixed radio-opaque and radiolucent lesion of the posterior maxilla, Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology (2014), doi: 10.1016/j.oooo.2014.03.003. 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.
ACCEPTED MANUSCRIPT
Clinicopathologic Correlation
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A mixed radio-opaque and radiolucent lesion of the posterior maxilla
Chan M Park DDS, MD1, Jacob S Barber DDS2, and Nasser Said-Al-Naief DDS, MS3
Director, University of the Pacific School of Dentistry, Highland Hospital,
Division of Oral and Maxillofacial Surgery, Oakland CA
Resident, University of the Pacific School of Dentistry, Highland Hospital,
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2Chief
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1 Program
Division of Oral and Maxillofacial Surgery, Oakland CA 3 Associate
Professor, University of the Pacific School of Dentistry, Pacific Oral and
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Maxillofacial Pathology Department, San Francisco CA
Address Correspondence to: Chan M Park DDS, MD Division of Oral and Maxillofacial Surgery Highland Hospital, Alameda County Medical Center 1411 E 31st Street, Oakland, Ca 94602 Telephone: 510-437-8523
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Fax: 510-535-7761 Email: [email protected] Clinical Presentation:
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A 48 year old male was referred by the general dentist to the oral and
maxillofacial surgery clinic at Highland Hospital in Oakland, California for evaluation of an asymptomatic left maxillary swelling and management of an impacted left
SC
maxillary third molar. The swelling had been present for an unknown duration. On physical examination, there were no extraoral findings or lymphadenopathy.
M AN U
Intraorally, there was a firm buccal and palatal expansion in the left posterior maxilla obscuring the visibility of his left maxillary molars (Figure 1). Aside from the notable poor dentition, the rest of the intraoral examination was unremarkable. The medical history was significant for schizophrenia, type II diabetes, and
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asthma. Two months prior to our evaluation of the patient, he was hospitalized for a deep venous thrombosis and a pulmonary embolism. The patient’s medications included lisinopril, Advair (fluticasone/salmeterol), aspirin, Lovenox (enoxaparin),
EP
risperdone, and bupropion.
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A panoramic radiograph illustrated an impacted grossly deformed left maxillary third molar and an approximately 4x3 centimeter diffuse mixed radiolucent and radio-opaque lesion within the left posterior maxilla (Figure 2). A computed tomograph of the maxillofacial region demonstrated a 4x4x3cm
poorly-circumscribed mixed radiolucent and radio-opaque lesion extending from the distal of the left maxillary second premolar to the pterygoid plates (Figure 3a-b). The maxillary sinus was displaced superiorly.
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Differential Diagnosis: Several entities were entertained in the differential diagnosis of the current
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lesion, based on the physical examination and radiographs. Odontoma is a very common odontogenic tumor. In a study by Buchner et al, 75.9% of all 1,088
odontogenic tumors identified were odontomas.1 There are two classical types:
SC
compound, which appears tooth-like, and the complex, which are radio-opaque and are a mixture of hard and soft dental tissue.2 Radiographically this case did appear
M AN U
to have an impacted tooth, with radio-opacities surrounding it, which could be a compound odontoma. Odontoma is considered in the differential diagnosis because it commonly is involved with impacted teeth or unerupted teeth, and is asymptomatic, just as this case presented. The lowest frequency of odontomas is
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found in the posterior maxilla(13%), with the highest incidence in the anterior maxilla(37%). Odontomas normally are identified in younger patients, which is not
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consistent with this case, although that does not rule it out.
Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg
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tumor, was also on our differential diagnosis. CEOTs are benign odontogenic epithelial tumors, most likely derived from the reduced enamel epithelium, stratum intermedium, or dental lamina epithelium.3 CEOTs may occur as the more common central type or as an extraosseous (peripheral) lesion. The radiographic appearance of an impacted tooth associated with small diffuse surrounding calcifications within a poorly-defined radiolucent lesion is suggestive of CEOT, which is how this case presents radiographically. CEOTs are 3 times more likely to be found in the
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mandible than the maxilla, with Neville, et al., reporting that the posterior maxilla is the location of 21% of CEOTs.4 This lesion is in the posterior maxilla, which makes it less likely to be CEOT. The clinical presentation of a CEOT is a slowly enlarging
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painless mass that occurs in a pericoronal location or prohibits the eruption of a tooth,5 which is consistent with how this patient presented to our clinic.
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Ameloblastic fibro-odontoma (AFO) a painless, slow growing, oftenasymptomatic mixed tumor very similar to the onset in this patient. The
M AN U
distribution of these tumors is evenly split between the maxilla and mandible, without gender preference. Radiographically, AFO may appear as a unilocular or multilocular radiolucent lesion with a well-corticated border.1 They contain radioopaque contents of various opacity, and often are associated with an unerupted
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tooth.6,7 AFOs mainly occur in children in the 1st or 2nd decades of life with an average age of nine8. Due to the young age prediliction it makes this lesion less likely to be the culprit in our patient.
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Odontoameloblastoma, previously named the ameloblastic odontoma is an
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extremely rare tumor. It characteristically presents as a locally destructive, slow growing, radiolucent lesion that often contains tooth-like radiopacities.4 There have been few cases documented, and generally, they occur in a wide age range; from infancy up until the 5th decade, with predilection for the posterior mandible.9,10 Based on the similar documented age range at diagnosis, clinical presentation, and radiographic appearance, the odontoameloblastoma must be entertained in our patient.
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Calcifying odontogenic cyst (COC) was also included in the differential diagnosis. COC involves patients with a wide age range and involves upper and lower jaws with equal frequency, with prevalence for the anterior jaw. This
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asymptomatic lesion has a radiopaque outline with calcifications internally and
typically occurs in a pericoronal location or in association with an unerupted teeth.2
jaw makes our case less likely to be a COC.
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Radiographically the COC mimics our lesion; however, its preference for the anterior
Dentinogenic ghost cell tumor (DGCT) is an extremely rare odontogenic
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tumor, which is accepted by most as the solid variant of COC. Histologically these tumors display an infiltrative solid growth pattern composed of odontogenic epithelium mixed with ghost cells and dentinoid ground substance.11 DGCT involves males and females with equal frequency and equally involves the maxilla and
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mandible, with predilection towards the anterior jaws. Additionally, it is typically seen in patients less than 30 years of age. Radiographically, it appears as a well defined or poorly circumscribed unilocular or a multilocular radiolucent lesion with
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various amounts of calcifications.12 Radiographically, the DGCT represents our
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patient’s lesion but the age during presentation and likelihood to be in the anterior jaws makes it lower on our differential list. Adenomatoid odontogenic tumor (AOT) has been reported to be the fifth
most common odontogenic tumor. It is an asymptomatic radiolucent lesion with occasional radio-opacities within the lesion that may cause slow cortical displacement.2 It occurs with a sex predilection for women of nearly 2:1 and is usually in younger patients.13 Our patient is in his 5th decade of life, which makes it
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unlikely to be this tumor and, normally, AOT is found in the anterior jaws of females. Calcification of the tumor consists of irregular calcifications that demonstrate a Liesegang pattern similar to the CEOT.14
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Desmoplastic ameloblastoma (DA) was also considered in our differential, as it has been known to be mistaken for a mixed benign fibro-osseous lesion
radiographically. DA was first described in 1984 by Eversole, et al15 and many
SC
similar cases have been reported since then. DA can affect people of all ages;
however, it affects people more commonly in the range of 30-40 years of age. They
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are often radiolucent, but most commonly present with a mixed radiolucent/radioopaque pattern and may appear partially calcified, with poorly-defined borders. The presenting sign is often asymptomatic expansion. The lesion has a high tendency to be in the anterior portion of the jaw, with no predilection for either
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jaw.16 Again, the DA radiographically and demographically presents like our case, but the predilection for the anterior jaw makes it less likely. Based on this patient’s clinical presentation, symptoms, and radiographic
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appearance, a benign odontogenic neoplasm was our working diagnosis. The
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duration of the maxillary expansion was unknown, but the diagnosis was strengthened by the fact that it was painless, involved a tooth and had a mixedradio-opaque presentation on radiographs.
Diagnosis and Management An incisional biopsy showed sheets of round and polyhedral cells which exhibited clearly-evident intercellular bridges. Mild but generalized cellular pleomorphism
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and patchy nuclear hyperchromatism was appreciated throughout. Concentric calcifications were also present throughout, and both epithelial and calcified elements were intimately associated with and were supported by a homogeneous
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amorphous material. It stained positively for Congo red special stain and showed
birefringent apple green hue on polarization, confirming amyloid origin (Figures 46). The histopathology of the lesion led to a diagnosis of a calcified epithelial
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odontogenic tumor (CEOT). Subsequently, a left hemi-maxillectomy was performed (Figure 7). The patient was followed post-operatively and was last seen at one
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year, at which there was no evidence of recurrence of disease.
Discussion:
Calcified epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, was
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first described by Pindborg in 1955.17 Pindborg went on to publish with Franklin in 1976 a paper characterizing 113 CEOTs that had been reported since 1946.18 They found CEOT to be twice as likely to occur in the mandible than the maxilla, and
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usually in the molar-premolar region. The current literature states there is a ratio of
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3:1 in the mandible to the maxilla.8,19 They found the mean age to be 40 years, with equal gender predilection.18 The typical clinical presentation of a CEOT is a locally aggressive, painless, and slowly-enlarging mass. Radiographically, they appear radiolucent with flecks of radio-opaque calcifications.3 The patient in this report described a similar slow onset of a painless maxillary mass, and his age is near the mean age. The radiographic appearance also led us to be suspicious of a CEOT.
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CEOT presents with distinct histomorphologic features: solid sheets, trabeculae or nests of monotonous-appearing epithelial cells with well-formed intercellular bridges. They may potentially show generalized cellular
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pleomorphism; however, mitotic activity is typically not observed. A variable
amount of concentric calcification referred to as “Liesegang rings” is seen. However, tumors may lack any evidence of calcifications.20 Of significance is the presence of
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amyloid deposits within these tumors, which can be confirmed by special stain
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Congo red21 or crystal violet22 and immunohistochemical staining for thioflavin T.23
There has been debate in the literature regarding the proper treatment of CEOTs. Some authors feel it represents a locally aggressive lesion,24 while others feel it is an expansile tumor that does not invade the trabeculation of medullary
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bone25. Demian, et al., in 2010 published a case report of a CEOT with malignant transformation and discussed 7 others previously reported in the literature.26 Enucleation and curettage may only be a reasonable option for very small tumors,
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less than 1.5 cm. There are no studies examining what is an adequate bony margin to take. Based on the aggressive and infiltrative nature of the tumor, resection with
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a rim of normal tissue is currently recommended.8,19 If there are clear cells present, larger surgical margins are indicated, because clear cell odontogenic tumors are thought to behave more aggressively than the conventional CEOT27. If intraoperative frozen studies are performed, a smaller excision margin is acceptable, assuming margins are negative.
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References 1. Buchner A, Phillip MW, Carpenter WM. Relative frequency of central odontogenic tumors: a study of 1088 cases from northern California and
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comparison to studies from other parts of the world. J Oral Maxillofac Surg 2006; 64(9):1343-52.
2. White DK, Street CC, Jenkins WS, Clark AR, Ford JE. Panoramic radiograph in
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pathology. Atlas Oral Maxillofacial Surg Clin N Am 2003;11(1): 1-53.
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3. Sapp J, Eversole L, Wysocki G. Odontogenic Tumors. In: Rudolph P, Alvis K, editors. Contemporary Oral and Maxillofacial Pathology. St. Louis, Missouri Mosby; 2004. p. 143-5.
4. Neville B, Damm D Allen CM, Bouquot JE. Odontogenic Cysts and Tumors. In:
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Rudolph P, Alvis K, Forest E, editors. Oral and Maxillofacial Pathology. Philadelphia, Pennsylvania Saunders Company; 2002 p. 623-625.
5. Nakano H, Ota Y, Yura Y. Calcifying epithelial odontogenic tumor of the maxilla
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with ulcerative stomatitis: a case report. Br J Oral Maxillofac Surg 2009;47(3):222-4.
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6. De Riu G, Meloni SM, Contini M, Tullio A. Ameloblastic fibro-odontoma: case report and review of the literature. J Craniomaxillofac Surg 2010;38(2):141-4. 7. Zouhary KJ, Said-Al-Naief N, Waite PD. Ameloblastic fibro-odontoma: expansile mixed radiolucent lesion in the posterior maxilla: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106(4):15-21. 8. Hu Y, Liu B, Su T, Zhang W, Zhao. A huge ameloblastic fibro-odontoma of the maxilla. Oral Oncol 2006;42(4):160-2.
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9. Sapru BL, Dasgupta D, Rajaram. Odontoameloblastoma: a rare odontogenic tumor. Med J Arm Forc India 2001;57(4):333-4.
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10. Mosqueda-Taylor A, Carlos-Bregni R, Ramirez-Amador V, Palma-Guzman JM,
Esquivel-Bonilla D, Hernandez-Rojas LA. Odontoameloblastoma. Clinico-pathologic study of three cases and critical review of the literature. Oral Oncol
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2002;38(8):800-5.
11. Stone CH, Gaba AR, Benninger MS, Zarbo RJ. Odontogenic ghost cell tumor: a
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case report with cytological findings. Diagn Cytopathol 1998;18(2):199-203. 12. Chindasombatjaroen J, Kakimoto N, Akiyama H, Kubo K, Murakami S, Furukawa S, Kishino M. Computerized tomography observation of a calcifying cystic odontogenic tumor with an odontoma: case report. Oral Surg Oral Med Oral Pathol
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Oral Radiol Endod 2007;104:52-7.
13. Suzukia H, Hashimoto K. Adenomatoid odontogenic tumour of the maxilla: immunohistochemical study. Asian J Oral Maxillofac Surg 2005;17(4):267-71.
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14. Philipsen HP, Reichart PA. Adenomatoid odontogenic tumour: facts and figures.
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Oral Oncol 1999;35(2):125-131. 15. Eversole LR, Leider AS, Strub D. Radiographic characteristics of cystogenic ameloblastoma. Oral Surg Oral Med Oral Pathol 1984;57(5):572-7.
16. Said-Al-Naief N. Updates in Diagnostic Pathology. Odontogenic Tumors for General Pathologists. Adv Exp Med Biol. 2005;563:148-64. 17.. Pindborg JJ: Calcifying epithelial odontogenic tumor. Acta Path Micro Stand. 1955;111:71.
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18. Franklin CD, Pindborg JJ. The calcifying epithelial odontogenic tumor: A review and analysis of 113 Cases. Oral Surg 1976;42(7):53.
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19. Marciani RD, Carlson ER, Braun TW. Trauma Surgical Pathology Temporomandibular Disorders. Oral and Maxillofacial Surgery 2nd Ed Vol II; 2009. p. 473-475.
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20. Takata T, Ogawa I, Miyauchi M, Ijuhin N, Nikai H, Fujita M. Non-calcifying
pindborg tumor with langerhans cells. J Oral Pathol Med. 1993;22(8):378-83
Cytochem 1962;10:355.
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21.. Holde P, Sweat F. On the binding of congo red by amyloid. J Histochem
22.. Askanas V, Engel WK, Alvarez RB. Light and electron microscopic localization of beta-amyloid protein in muscle biopsies of patients with inclusion-body myositis.
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Am J Pathol 1992;141(1):31-6.
23. LeVine III H. Quantification of B-sheet amyloid fibril structures with thioflavin T. Methods Enzymol 1999;309:274-84.
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43.
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24. Pindborg JJ. A calcifying epithelial odontogenic tumor. Cancer 1958;11:838-
25. Vap DR, Dahlin DC, Turlington EG. Pindborg tumor: the so-called calcifying epithelial odontogenic tumor. Cancer 1970;25:629-36. 26. Demian N, Harris RJ, Abramovitch K, Wilson JW, Vigneswaran N. Malignant transformation of calcifying epithelial odontogenic tumor is associated with loss of p53 transcriptional activity: a case report with review of the literature. J Oral Maxillofac Surg 2010;68(8):1964-73.
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27. Hicks MJ, Flaitz CM, Wong MEK, McDaniel RK, Cagle PT. Clear cell variant of calcifying epithelial odontogenic tumor: case report and review of the literature.
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Head Neck 1994;16(3):272-7.
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Legends
Figure 1. The left maxillary expansile lesion extending from the buccal vestibule
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into the palate.
Figure 2. At the initial examination a panoramic radiographic shows a left maxillary
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posterior mixed lesion.
Figure 3.
3a. An axial computed tomogram (CT) slice at the level of the maxillary mid root
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radiolucency.
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demonstrates a thinned cortical border with radio-opacities present within the
3b. A sagittal CT slice of the same area.
Figure 4. A low power photomicrograph demonstrates the presence of an epithelial tumor, composed of large sheets of cells (black solid arrow), interspersed by generalized noticeable amount of calcification (hollow arrow) (hematoxylin and eosin stain; original magnification X100).
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Figure 5. 5a. This highlights the presence of clusters and sheets of epithelial cells intimately
(hematoxylin and eosin stain; original magnification X200).
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juxtaposed withe concentric calcifications and homogenous pink-staining material
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5b. A higher power photomicrograph demonstrates dense sheets of epithelium which exhibit well-formed intercellular bridges and mild generalized cellular
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pleomorphism (white arrow) and intimately surrounding concentric type calcifications (black arrow); referred to as “Liesegang rings” (hematoxylin and eosin stain; original magnification X400).
5c. A lower power view shows homogeneous acellular material (arrow); proven to
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be amyloid in origin (hematoxylin and eosin stain; original magnification X200). 5d. Positive staining of the homogeneous acellular material (arrow) with Congo red stain, characteristic of and confirming the presence of amyloid
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(Special stain; Congo red, original magnification X400).
Figure 6. A low power photomicrograph demonstrates the presence of generalized apple-green birefringent pattern, characteristic of polarized amyloid material (Congo red special stain; polarized light, original magnification X100).
Figure 7. The gross excised specimen.
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