CASE REPORTS
J Oral Maxillofac Surg
Calcifying Epithelial Odontogenic
(Pindborg) Tumor: A Series of
4 Distinctive Cases and a Review
of the Literature
Beatriz Patiño, MD,* Javier Fernández-Alba, MD, PhD,†
Alvaro Garcia-Rozado, MD,‡ Roberto Martin, MD,§
José Luis López-Cedrún, MD, PhD,储 and
Begoña Sanromán, MD¶
The calcifying epithelial odontogenic tumor (CEOT)
was first described as an entity by Danish pathologist
Jens J. Pindborg in 1955.1 Also known as Pindborg
tumor, it is an uncommon, locally invasive, benign
odontogenic tumor occurring in individuals over a
wide age range but peaking in incidence in the 40s. It
usually presents as a hard painless mass, generally
affecting the mandible. The characteristic histopatho-
logic description consists of sheets and islands of
polygonal cells that often have distinct intercellular
bridges. The nuclei may be pleomorphic and hyper-
chromatic and bizarre in appearance. Mitoses are very
uncommon. Pale eosinophilic masses (amyloid-like)
may be found within the sheets of tumor cells and can
undergo calcification, often concentrically in the form
of Liesegang rings.2
Our experience consists of 4 cases in the last 4
years. We present these cases and review the series
Received from the Complejo Hospitalario Juan Canalejo, La
Coruña, Spain.
*Resident, Department of Maxillofacial Surgery.
†Consultant Maxillofacial Surgeon, Department of Maxillofacial
Surgery.
‡Consultant Maxillofacial Surgeon, Department of Maxillofacial
Surgery.
§Consultant Maxillofacial Surgeon, Department of Maxillofacial
Surgery.
储Department Chief, Department of Maxillofacial Surgery.
¶Resident, Department of Pathology.
Address correspondence and reprint requests to Dr Patiño:
Complejo Hospitalario Juan Canalejo, La Coruña, Department of
Maxillofacial Surgery, Xubias de Arriba 84, 15006 La Coruña, Spain;
e-mail: 31007bps@comb.es
© 2005 American Association of Oral and Maxillofacial Surgeons
0278-2391/05/6309-0017$30.00/0
doi:10.1016/j.joms.2005.05.302
1361
63:1361-1368, 2005
reported in the literature. Their interest lies not only
in their considerable amount, but also in their clinical
characteristics.
Report of Cases
CASE 1
A 31-year-old man was referred by his general practitioner
for diagnosis of a painless mandibular swelling that had
been present for 9 years and had gradually increased in size.
On examination there was a 3 ⫻ 5 cm exophytic reddish
tumor without bleeding or ulcers. An orthopantomogram
(OPG) revealed a 4 ⫻ 5 cm radiolucent lobular image in
symphysis (Fig 1), without root resorption, right to the
inferior border.
Computed tomography (CT) showed an osseous destruc-
tive lesion (Fig 2). An incisional biopsy was taken and was
consistent with a diagnosis of CEOT.
Surgical treatment was performed that included en bloc
resection of the tumor with clinically normal margins, pre-
serving the inferior border of the symphysis bone. Surgery
was completed with bone distraction to fill in the defect.
Two horizontal distraction devices were placed internally,
emerging from both mandibular angles, after intraoperative
lateralization of both inferior alveolar nerves.
Surgery was partially successful, but after removing these
distraction appliances, another alveolar distraction device
was placed to increase the mandibular vertical length.
Microscopically, the tumor showed strands and nests of
cells with pleomorphic nuclei, prominent nucleoli, uncom-
mon mitoses, and a pronounced eosinophilic cytoplasm,
everything surrounded by a fibrous tissue containing ample
eosinophilic material that stained intensely positive for amy-
loid with Congo red. Many calcified spots were found.
The patient remains tumor-free 2 years after surgery.
CASE 2
The second case is a 67-year-old woman with a slightly
bleeding and painless lesion located in the gum of the
alveolar border of the right superior maxilla that had ap-
peared 1 month before. On examination there was a super-
ficially ulcerated mass, similar to an epulis (Fig 3). The OPG
1362
FIGURE 1. Case 1: An orthopantomogram (OPG) revealed a 4 ⫻ 5
cm radiolucent lobular image in symphysis, without root resorption,
right to the inferior border.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
showed a superficial erosive pattern in the bone adjacent to
the tumor (Fig 4).
An excisional biopsy was performed; the pathologic ex-
amination showed a tumor composed of strands and nests
of ameloblastic cells with abundant eosinophilic material
that stained occasionally with Congo red techniques. Some
calcifications were seen. No atypias nor mitoses were
found. The final diagnosis was CEOT.
After almost 4 years, the patient is disease-free.
CASE 3
Case 3 is a 24-year old woman with a painless swelling of
the right maxilla. The tumor had been present for 4 years
and had increased its size faster during the last 4 months.
The intraoral examination disclosed a hard submucosal 3 ⫻
4 cm tumor firmly attached to the anterolateral surface of
the right maxilla and the zygoma. The patient did not
complain of any symptoms.
The OPG showed a well-defined mass with occasional
radiopaque foci, ahead of the maxillary sinus, without root
resorption. The CT scan revealed a right maxillary 3.5 ⫻ 2.5
cm tumor arising from the hard palate and causing a broad
FIGURE 2. Case 1: The CT showed an osseous destructive lesion in
symphysis.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
CALCIFYING EPITHELIAL ODONTOGENIC TUMOR
FIGURE 3. Case 2: On examination there was a superficially ulcer-
ated mass, similar to an epulis.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
bone destruction in the anterolateral wall of the maxillary
sinus. The mass was heterogeneous, with cystic and solid
areas and irregular calcifications.
An excisional biopsy identified the mass as a Pindborg
tumor. Partial maxillectomy was performed, from the first
molar tooth to the pterygoid processes. Reconstruction was
accomplished with a non microvascular iliac crest graft
fixed with miniplates.
Microscopically, the tumor contained a large amount of
spherical eosinophilic structures, some of them with concen-
tric lamination and sometimes showing an empty space in
the middle. Between them, there were few star-shaped cells
with scattered atypias. The eosinophilic structures showed
calcified areas. The tumor had infiltrating features without
atipie.
After surgery, the patient suffered an infection of the
surgical wound, so that part of the graft had to be removed
because of necrosis. Four years later, the patient is disease-
free.
CASE 4
A 40-year old man was referred to us after a casual finding
in an OPG. On examination there was a swelling in the right
FIGURE 4. Case 2: The OPG showed a superficial erosive pattern in
the bone adjacent to the tumor.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
PATIÑO ET AL 1363

stratum intermedium of tooth germs, a finding later

FIGURE 5. Case 4: The OPG showed a mixed radiolucent-
radiopaque defect, with “soap bubble” texture, from the second pre-
molar tooth to the mandibular angle.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
mandible. The OPG showed a mixed radiolucent-
radiopaque defect, with “soap bubble” texture, from the
second premolar tooth to the mandibular angle (Fig 5). No
impacted teeth were found. CT scan revealed a 3.2 ⫻ 1.8
cm expansive mass, without extraosseous component
(Fig 6).
An incisional biopsy was performed, and the histologic
description was of strands and nests of polyhedral epithelial
cells with eosinophilic cytoplasm and prominent nuclei.
These cells had distinct intercellular bridges and were irreg-
ular in shape with pleomorphic nuclei. Between cells, there
was an eosinophilic amyloid-like surrounding material. Cal-
cium salts were diffusely deposited, with calcified bodies
showing concentric lamellae.
Segmental mandibulectomy was performed, and recon-
struction with iliac crest free flap was achieved. The patient
is now disease-free after 1 year.
Discussion
CEOT is an uncommon neoplasm accounting for
less than 1% of all odontogenic tumors. It is classified
between the benign epithelial odontogenic tumors.
After being described by Pindborg in 1955, during the
next 20 years only approximately 100 cases were
reported,3 and in a recent article Pflaumer et al4 esti-
mated that more than 150 cases have been reported
to date.
confirmed.7,8
The clinical differences among the CEOT types may
be because of their origin.9-11 Hicks12 distinguishes
the histogenetic origin of Pindborg tumor according
to its clinical presentation, that is, central (intraosse-
ous, 87.8%), peripheral (extraosseous, 6.1%), or as a
hybrid tumor in combination with adenomatoid odon-
togenic tumor (AOT). In his opinion, central tumors
are derived from the stratum intermedium of the
enamel organ. In contrast, the extraosseous form
arises from dental lamina epithelial rests in gingival
and/or basal cells of the gingival surface epithelium.
With the hybrid CEOT/AOT, the AOT portion arises
from all 3 components of the enamel organ
(preameloblasts, stellate reticulum, and stratum inter-
medium). Apparently, in rare cases of AOT, induction
of CEOT formation occurs from the stratum interme-
dium.
EPIDEMIOLOGY AND CLINICAL PRESENTATION
CEOT is considered a tumor of adults, with most
patients in the age range of 30 to 50 years.13 The
mean age is slightly greater in central CEOT (40.3
years, 31.8 years in peripheral CEOT).12 However,
there is a wide age range reported from 8 to 92 years.3
There are no differences according to gender6 in
central CEOT. With regard to localization, most cases
(68%) occur in the mandible, around the premolar-
molar region.6,14 Our patients (case nos. 1 and 3)
presented with fairly uncommon localizations, 1 in
the symphysis and the other in the maxilla. Fifty-two
percent of the cases of CEOT are associated with an

HISTOGENESIS
The CEOT (a tumor derived from the odontogenic
epithelium without odontogenic ectomesenchyme) is
thought to be related histogenetically to the rests of
the odontogenic epithelium, specifically to the stra-
tum intermedium of the enamel organ. In an article
from 1966, Pindborg suggested that the tumor arises
from the reduced enamel organ because it is often
associated with an embedded tooth.5 A histochemical
study of the CEOT has shown intense alkaline phos- FIGURE 6. Case 4: The CT scan revealed a 3.2 ⫻ 1.8 cm expan-
phatase activity in the epithelial cells.6 The histo- sive mass, without extraosseous component.
chemical pattern of this activity resembles the alka- Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
line phosphatase appearance that occurs in the Maxillofac Surg 2005.
1364
unerupted or embedded tooth.6 The typical clinical
presentation is a slowly enlarging intraosseous mass
that causes expansion of the affected mandible and is
asymptomatic.12 Although the CEOT is a benign neo-
plasm, it has a variable biologic behavior ranging from
very mild to moderate invasiveness.2 The tumor
grows by infiltration and may produce cortical expan-
sion, tooth movement, and root resorption.13 Maxil-
lary tumors often involve the sinus, but there are few
sinus symptoms.13 Like other benign odontogenic tu-
mors, it will not cause an alteration of nerve sensa-
tion.
Peripheral CEOTs are typically smaller than 2 cm.
They grow from the gum or from the edentulous
alveolar mucosa, as in case no. 2, generally in the
anterior region (incisor-premolar). They are painless
and firm, without osseous invasiveness of the maxilla.
Nevertheless, the bone adjacent to the tumor typically
shows a superficial erosive pattern.12 Its behavior is
less aggressive clinically than the central one, and
therefore the treatment could be more conservative.
The gender ratio in peripheral CEOTs is 1 to 2 (male:
female).12
None of the patients in our series have shown, until
now, clinical or histopathologic evidence of recur-
rence, although the follow-up is no longer than 4
years. Invasiveness was variable: case no. 2, periph-
eral CEOT, presented with a small tumor, without
affectation of the surrounding tissues. Case no. 3 had
a tumor located in the maxilla, but limited to the hard
palate and dentoalveolar process. Case no. 4, the most
characteristic one, consisted of a mass in the right
mandibular angle that caused cortical expansion with-
out breaking it. Case 1, as well as case no. 4, were
quite invasive tumors.
RADIOLOGY
Because the invasiveness of this neoplasm is vari-
able and will influence treatment, a panoramic radio-
graph and CT scan are recommended.
An early tumor may be completely radiolucent. As
the tumor matures and becomes larger, most will
become mixed radiolucent-radiopaque, although
some larger tumors will remain radiolucent.2 More-
over, the radiographic picture will present a spectrum
ranging from a unilocular radiolucency to a very mul-
tilocular one, suggestive of a “soap bubble” appear-
ance.2 Most are associated with the crown of an
impacted tooth; in this case, the tumor can be con-
fused with an odontogenic cyst. Smaller tumors may
appear uniloculated, but this is misleading because all
are infiltrating.13 Characteristically, multiple radio-
pacities of varying size develop within the radiolucent
area and occasionally there are extensive areas of
calcification, which cause the lesion to become ra-
diopaque.15 Peripheral tumors are typically radiolu-
CALCIFYING EPITHELIAL ODONTOGENIC TUMOR
cent, although the bone adjacent to the tumor char-
acteristically shows a superficial erosive pattern,12 as
we said before and as is reported in case no. 2.
As the plain radiographs are rather unspecific, the
CT of Pindborg tumor usually shows a well defined
mass that thins both plates of the mandible and con-
tains scattered radiopaque areas.15 Magnetic reso-
nance imaging (MRI) reveals a lesion that is predom-
inantly hyperintense on T2-weighted and hypointense
on T1-weighted images. These features distinguish
the lesions from more aggressive neoplasms. Regard-
ing the requirement of further imaging other than
plain radiographs, we refer to the article reported in
2000 by Cross et al.15 CT and MRI images are not of
primary diagnostic importance. However, to be able
to see the internal structures of the lesion and the
involvement of neighboring structures gives useful
information in the surgical plan. There are some spe-
cific questions that influence the selection of a surgi-
cal approach, such as whether there are both cortical
plates of the mandible involved or whether there is
extraosseous extension of soft tissue. Plain films are
unreliable in detecting the degree of cortical bone
involvement or the presence of a soft tissue mass and
are unable to visualize the inferior alveolar canal sat-
isfactorily.
All our patients (except case 2) underwent CT
exploration. The images showed radiolucent lesions
with a calcification pattern inside. None of the pa-
tients underwent MRI exploration. We think MRI has
a lesser usefulness because the bony structures are
better represented in CT images. The potential advan-
tages of MRI include absence of ionizing radiation and
superior contrast resolution of soft tissues. Because of
its radiologic characteristics, Pindborg tumor needs to
be distinguished from other radiolucent or mixed jaw
lesions, such as odontogenic keratocysts, aneurysmal
bone cyst, ameloblastomas, and odontogenic myxo-
mas. Table 1 summarizes the difference between the
imaging features of this lesion and those of other
lesions of the jaw.
HISTOPATHOLOGY
Histopathology is probably the most variable aspect
of this tumor. CEOTs are unencapsulated, infiltrating
tumors (Fig 7). The characteristic epithelial compo-
nent consists of sheets and nests of polyhedral epi-
thelial cells (Fig 8) with well-defined cell borders,
often displaying intercellular bridges (Fig 9).12 These
neoplastic cells show a moderate degree of pleomor-
phism, but only rare typical mitoses. Nuclei are cen-
trally located and often contain a large nucleolus. The
nuclei may be pleomorphic, hyperchromatic, and bi-
zarre in appearance.2 Binucleated cells may be fre-
quent.
PATIÑO ET AL 1365

Table 1. COMPARISON OF THE IMAGING FEATURES WITH THOSE OF OTHER JAW LESIONS15

Magnetic Resonance
Lesion Plain Film Radiography Computed Tomography Imaging

Pindborg tumor Unilocular or multilocular radiolucent Well-defined, expands bone, cortical High signal on T2W1, low
defect, may be radiopaque, well or thinning, multilocular, bony septa, signal on T1W1; low
poorly defined, “honeycomb” radiopacities, tooth embedded signal areas in keeping
texture, scattered radiopacities, with bony septa
may contain tooth
Ameloblastoma Unilocular or multilocular radiolucent Expansion of bone, cortical Multilocular, solid and
defect; sharp scalloped margin, destruction, soft tissue extension cystic components,
“soap bubble” or “honeycomb” irregular tic walls,
texture, may contain tooth papillary projections,
high intensity spots on
T1W1, enhancement
with Gd-DTPA.
Myxoma Unilocular or multilocular radiolucent Expansion and thinning of bone, High signal on T2W1,
defect, irregular scalloped margin, internal trabeculation, soft tissue solid and cystic
between roots of teeth, “soap extension, cystic mass components
bubble” texture, bone cortex intact
Aneurysmal Expands bone, well-defined, Expansion, internal septations, Multiloculated, fluid levels
bone cyst trabeculations smooth margins, no bone
destruction
Odontogenic Unilocular, well-defined, displaced Well-defined, thinning of cortex
keratocysts mandibular canal which may be perforated
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral Maxillofac Surg 2005.

The most characteristic findings are the presence of loss of electron density and become degraded into
amyloid-like substance and calcified concentric Liese- fine filaments, having similar thickness and electron
gang rings.12 Rounded, pale eosinophilic masses density to those forming the filamentous masses. It is
(amyloid-like) may be found within the sheets of tu- concluded that the fine filamentous material is a form
mor cells and, because of its affinity with mineral of amyloid which results from degradation of lamina
salts, it can undergo calcification, often having the densa material.17
concentric appearance of lamellar bodies or Lieseg- CEOT has been classified as one of the epithelial
ang rings (Fig 10).6 The surrounding tissue may also odontogenic tumors, and no structures reminiscent to
contain large clumps of this homogeneous eosino- dental hard tissues have been identified. However,
philic material (Fig 8). Calcium salts are often dif- dentin18 and bone or cementum-like components
fusely deposited within these areas. The eosinophilic
material has an apple-green birefringence under po-
larized light after staining with Congo red (Fig 11).13
It also stains positive for crystal violet and thioflavine
T.2 As the globules mineralize, the amyloid-like mate-
rial loses its positivity for Congo red16 and changes
from PAS-negative to strongly PAS-positive.
There is some controversy as to whether the ho-
mogeneous substance is a degeneration product or is
actively secreted, and whether it is extracellular or
intracellular in origin.6 Although the exact origin is
not known, the amyloid in Pindborg tumor is proba-
bly derived from degradation of lamina densa material
(basal lamina), secreted by the tumor epithelial
cells.17 An ultrastructural study of the eosinophilic
masses has demonstrated 2 types of structures that
are probably related. The first type appears as sheets
FIGURE 7. Case 4: CEOTs are unencapsulated, infiltrating tumors. In
of fine filaments measuring 10 to 12 nm in diameter. this case the epithelial tumor invaded locally the osseous trabeculae.
The second type is in the form of aggregates of lamina (Hematoxylin-eosin stain; magnification ⫻ 100.)
densa fragments, probably secreted by the tumor ep- Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
ithelium. These fragments appear to undergo some Maxillofac Surg 2005.
1366 CALCIFYING EPITHELIAL ODONTOGENIC TUMOR

FIGURE 8. Case 4: The characteristic epithelial component consists FIGURE 10. Case 4: Calcifications having the concentric appear-
of sheets and nests of polyhedral epithelial cells. In this photograph, the ance of lamellar bodies or Liesegang rings may be found within the
tumoral cells arranged in solid nests surrounded by a dense eosino- sheets of tumor cells. (Hematoxylin-eosin stain; magnification ⫻ 200.)
philic stroma are seen. (Hematoxylin-eosin stain; magnification ⫻
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
100.)
Maxillofac Surg 2005.
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
as just as odontogenic as the epithelium instead of
being neglected as a simple fibrovascular tumor sup-
have been identified.16,19 The mineralized amyloid
porting tissue.16
appears to stimulate the adjacent stroma to the pro-
An ultrastructural study19 has shown that, in addi-
duction of a collagenous matrix of collagen. Ultra-
tion to the polyhedral epithelial cells, there is another
structurally, the banded collagen fibrils are arranged
cell type in the CEOT, a cell having the ultrastructural
perpendicular to the surface of the calcified lamellar
characteristics of myoepithelial cells. These cells
bodies.17 Calcification of this latter material will de-
show a lamina densa that is continuous with that in
velop acellular areas resembling cementum, as well as
relation to the basal plasma membrane of the tumor
cellular areas, resembling bone. Unmineralized Congo
epithelial cells and also a large number of hemides-
red-positive material did not appear to evoke such a
mosomes is seen between the myoepithelial cells and
stromal reaction. It is interesting to speculate whether
tumor epithelial cells.
the fibrous stroma of CEOT should not be considered
In 1982, Ai-Ru et al20 published a study in which
they described 4 different patterns of the CEOT based

FIGURE 9. Case 4: The cellularity is characterized by large eosino-

philic cytoplasms, often displaying intercellular bridges. Nuclei are
pleomorphic and often have a prominent nucleolus. Binucleated and
multinucleated cells may be frequent. (Hematoxylin-eosin stain; mag-
nification ⫻ 400.)
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
FIGURE 11. Case 4: The dense eosinophilic material surrounding
the tumoral nests is seen after staining with Congo red. (Congo red;
magnification x 200.)
Patiño et al. Calcifying Epithelial Odontogenic Tumor. J Oral
Maxillofac Surg 2005.
PATIÑO ET AL
on only 9 cases, which gives an idea of the enormous
variability of this tumor. Afterwards, a clear cell vari-
ant was described as a fifth pattern.13,12,21 Overall, the
clear cell variant of CEOT represents 6% of all CEOT
cases reported.12 In this variant, clear cells may com-
prise anything from a minor to a major component of
the neoplasm. The cells contain glycogen and are
mucin-negative.13
The cases presented in this article match the classic
description of the Pindborg tumor: there were no clear
cell variants or hybrid tumors in combination with
adenomatoid odontogenic tumors; the characteristic
polyhedral cells displaying intercellular bridges as
well as the amyloid substance, calcified or not, were
found in all the cases (see Figs 7-11).
TREATMENT AND PROGNOSIS
The CEOT, as seen before, has a variable biologic
behavior ranging from very mild to moderate invasive-
ness.2 Therefore, the literature has varied regarding
its treatment. Because it is an uncommon and slow-
growing tumor and follow-up is often lost or only
minimal, too few reports of long-term outcomes are
available.
The CEOT is generally managed surgically. Because
even small CEOTs are infiltrating tumors, treatment
should include removal with a border of clinically and
radiographically normal bone. A margin of about 1 cm
is appropriate. Margins placed too close are likely to
be compromised.13 Peripheral tumors may be treated
with a smaller margin of 0.5 cm, because of its lesser
aggressiveness. Although CEOT is considered less ag-
gressive clinically than typical infiltrating ameloblas-
toma, treatment results have not been as satisfactory
as expected. It seems that those treated with enucle-
ation and curettage procedures show a recurrence
rate ranging from 15% to 30% after just 2 to 4 years.
An overall recurrence rate of 14% was recorded.3
Those treated with resection approaches have few if
any recurrences. Therefore, the CEOT is best treated
with a resection using 1.0⫺ to 1.5⫺ cm margins in
bone.2 A minimum of 5 years and as many as 10 years
may be necessary because of the very slow growth
rate of this tumor.
Hicks et al12 described perineural invasion by clear
cells in a case of clear cells variant. A relatively high
recurrence rate of 22% was stated in these variants.
Because clear cell change in odontogenic tumors may
portend low-grade malignant behavior, this feature
should be regarded. Extended follow-up of these pa-
tients appears to be indicated to assess local recur-
rence and early initiation of therapy.
Recently, Veness et al22 reported a case of mandib-
ular Pindborg tumor with malignant transformation
and metastatic spread. After initial excision, the sur-
gical margins were involved. The tumor was diag-
1367
nosed as a CEOT. Afterwards, the tumor recurred, and
it was re-excised, and the tumor reached the surgical
margin again. The histopathologic analysis showed a
tumoral evolution, with an increase in cellularity and
mitoses and with loss of amyloid-like material and
calcification. Invasion of small blood vessels was a
new finding. A further re-excision was undertaken,
with a supraomohyoid neck dissection. Histopatho-
logic examination confirmed the diagnosis of odonto-
genic carcinoma and revealed infiltration into skeletal
muscle and metastatic spread to 1 level-3 lymph node.
In the literature there is only 1 other case of Pindborg
tumor with lymphatic metastatic spread before,23 but
in that case there is not a previous diagnosis of benig-
nity.
Regarding our casuistry, only case no. 2 could be
treated through conservative resection and direct clo-
sure. The other 3 cases, in view of the volume of the
tumoral mass and the need to include tumor-free
margins in the resected segment, created the dilemma
of the defect reconstruction. In case no. 1, as indi-
cated before, we opted for bone distraction. Case no.
3, a tumor situated in right maxilla that needed max-
illectomy performed, was treated with an iliac crest
graft, and case no. 4, whose follow-up is the briefest,
underwent surgical reconstruction with a microsurgi-
cal iliac crest free flap. None of the patients have
presented with any evidence of tumoral recurrence.
Although we acknowledge that longer follow-up is
necessary to consider them completely disease-free.
The CEOT shows a characteristic variation in its
histologic and clinical presentation. The clinical pre-
sentation, as can be seen in our series, is quite vari-
able, as much relating to location as to the size and
degree of local invasion. The histopathology presents
a wide variety of subtypes, something surprising com-
ing from a tumor so unusual. This circumstance
makes the diagnosis more difficult. Nevertheless,
achieving a correct diagnosis is extremely important
because adequate treatment, that is, an appropriate re-
section with accurate tumor-free margins for a benign
neoplasm with an invasive behavior, depends on it.
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agn Pathol 16:271, 1999
14. Veness MJ, Morgan G, Collins AP, et al: Calcifying epithelial
odontogénico (Pindborg) tumor with malignant transformation
and metastatic spread. Head Neck 23:692, 2001
J Oral Maxillofac Surg
63:1368-1371, 2005
Severe Isolated Temporomandibular
Joint Involvement in Juvenile
Idiopathic Arthritis
Paolo Scolozzi, MD, DMD,* Geraldine Bosson, MD, DMD,† and
Bertrand Jaques, MD, DMD‡
First described by Meyer Diamant-Berger in 1892 and
by Frederic Still in 1897, juvenile rheumatoid arthritis
represents a heterogeneous group of chronic inflam-
matory arthritis that begins in childhood and is dis-
tinct from adult rheumatoid arthritis.1 In 1997, the
International League of Associations for Rheumatol-
Received from the Department of Otolaryngology/Head and Neck
Surgery, Division of Oral and Maxillofacial Surgery, Centre Hospi-
talier Universitaire Vaudois, Lausanne, Switzerland.
*Chief Resident.
†Resident.
‡Professor.
Address correspondence and reprint requests to Dr Scolozzi: De-
partment of Otolaryngology/Head and Neck Surgery, Division of Oral
and Maxillofacial Surgery, Centre Hospitalier Universitaire Vaudois,
1011 Lausanne, Switzerland; e-mail: scolozzi@hotmail.com
© 2005 American Association of Oral and Maxillofacial Surgeons
0278-2391/05/6309-0018$30.00/0
doi:10.1016/j.joms.2005.05.300
SEVERE ISOLATED TMJ INVOLVEMENT IN JIA
15. Cross JJ, Pilkington RJ, Antoun NM, et al: Value of computed
tomography and magnetic resonance imaging in the treatment
of a calcifying epithelial odontogenic (Pindborg) tumour. Br J
Oral Maxillofac Surg 38:154, 2000
16. Slootweb PJ: Bone and cementum as stromal features in Pind-
borg tumor. J Oral Pathol Med 20:93, 1991
17. El-Labban NG: Cementum-like material in a case of Pindborg
tumor. J Oral Pathol Med 19:166, 1990
18. Chomette G, Auriol M, Guilbert F: Histoenzymological and ultra-
structural study of a bifocal calcifying epithelial odontogenic tu-
mor. Characteristics of epithelial cells and histogénesis of amyloid-
like material. Virchows Arch (Pathol Anat) 403:67, 1984
19. El-Labban NG, Lee KW, Kramer IRH: The duality of the cell
population in a calcifying odontogenic tumor (CEOT). Histo-
pathology 8:679, 1984
20. Ai-Ru L, Zhen L, Jian S: Calcifying epithelial odontogenic tu-
mors: A clinicopathologic study of nine cases. J Oral Pathol
11:399, 1982
21. Maiorano E, Altini M, Favia G: Clear cell tumors of the
salivary glands, jaws and oral mucosa. Semin Diagn Pathol
14:203, 1997
22. Veness MJ, Morgan G, Collins AP, et al: Calcifying epithelial
odontogénico (Pindborg) tumor with malignant transformation
and metastatic spread. Head Neck 23:692, 2001
23. Basu MK, Matthews JB, Sear AJ, et al: Calcifying epithelial
odontogenic tumor: A case showing features of malignancy.
J Oral Pathol 13:310, 1984
ogy proposed the more universal term of “juvenile
idiopathic arthritis” (JIA) and recognized 8 categories:
systemic arthritis, oligoarthritis (arthritis affecting 1 to
4 joints in the first 6 months of the disease), extended
oligoarthritis, polyarthritis (more than 4 joints in the
first 6 months of disease with 2 subgroups, rheuma-
toid factor (positive or negative), enthesitis related
arthritis (largely made up of HLA B-27 related disease),
psoriatic arthritis, and “other arthritis.” Juvenile idio-
pathic arthritis refers to arthritis of unknown cause in
1 or more joints of at least 6 weeks’ duration occur-
ring in children less than 16 years old.2
Juvenile idiopathic arthritis commonly afflicts the
temporomandibular joint (TMJ) leading to the destruc-
tion of the condylar growth center and subsequent man-
dibular growth disturbances in severe cases.3-6 This can
result in unpleasant facial deformity such as asymme-
try, micro- and retrognathia, as well as secondary
abnormal dental occlusion development. The ulti-
mate and dramatic consequence of the joint destruc-