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Caso clnico

Tumor de Pindborg (tumor odontognico

epitelial calcificante)

Pindborg tumor (Calcifying epithelial odontogenic tumor)

J. Rubio Palau1, C. Bescs Atn2, J. Pamias Romero2, M. Sez Barba2, G. Raspall Martn3,
P. Huguet Redecilla4

Resumen: El objetivo del presente artculo es realizar una revisin de la
literatura del tumor de Pindborg a propsito de un caso. El inters radica
en su baja frecuencia y a la importancia de la anatoma patolgica para su
diagnstico y correcto tratamiento ya que puede confundirse en algunos
casos con otras tumoraciones benignas, a diferencia de las cuales, en este
tumor debe realizarse una reseccin con mrgenes de seguridad para
disminuir la probabilidad de recidiva.
Abstract: The aim of this article is to review the literature
on Pindborg tumor based on a case. In view of its low fre-
quency and the importance of histology for its correct diag-
nosis and treatment as it can be confused with other benign
tumors, appropriate resection is necessary with tumor-free
margins in order to reduce the probability of local recu-
rrence.

Key words: Pindborg tumor; Calcifying epithelial odontogenic

Palabras clave: Tumor de Pindborg; Tumor odontognico epitelial cal-
cificante. tumor.

Recibido: 19.09.06
Aceptado: 18.12.06

1 Mdico Residente.
2 Mdico Adjunto.
3 Jefe de Servicio. Servicio de Ciruga Oral y Maxilofacial.
4 Mdico Adjunto. Servicio de Anatoma Patolgica.
Servicio de Ciruga Oral y Maxilofacial.
Hospital Universitari Vall dHebron. Barcelona, Espaa.

Correspondencia:
Josep Rubio Palau
Paseo Vall dHebron, 119-129
08035 Barcelona, Espaa
Email: jrubiopalau@yahoo.es
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J. Rubio y cols. Rev Esp Cir Oral y Maxilofac 2007;29,5 (septiembre-octubre):342-347 2007 ergon 343

Introduccin Introduction

El tumor de Pindborg o tambin lla- Pindborg tumor, also called

mado tumor odontognico epitelial cal- Calcifying Epithelial Odon-
cificante (TOEC) es una neoplasia benig- togenic Tumor (CEOT) is an
na pero con carcter invasivo local y uncommon, benign neo-
tendencia a la recidiva, de baja fre- plasm with an invasive local
cuencia y que suele asentar en la man- nature. It tends to arise in
dbula. the mandible and to recur.
El TOEC es una neoplasia rara que CEOT is a rare neoplasm
representa entre 0,17 y 1,8% de todos that represents between
los tumores odontognicos1 y de la que 0.17 and 1.8% of all odon-
tan slo se han publicado unos 200 togenic tumors, 1 and only
casos, con una media de 4 casos nue- 200 cases have been pub-
vos por ao en el mundo.2 lished, with an average of 4
Se han descritro dos variantes segn new cases per year in the
su localizacin: intrasea o central (94% world.2
de los casos) y extrasea, perifrica o Two varieties have been
de tejidos blandos (6% de los casos).2 described according to their
Actualmente no hay consenso acerca location: intraosseous or cen-
del origen del tumor y se cree que deri- tral (94% of cases) and
Figura 1. Fotografa de tres cuartos antes de la intervencin qui-
va de elementos epiteliales del rgano rrgica. extraosseous, peripheral or
del esmalte, aunque otros autores creen Figure 1. Three-quarter view before surgery. of soft tissues (6% of cases).2
que deriva de remanentes celulares de There is currently no con-
la lmina basal o a partir del estrato sensus as to the origins of
basal del epitelio gingival.3 the tumor, and it is believed
La afectacin mandibular es en fre- that it is derived from epithe-
cuencia el doble de la maxilar y suele lial elements of the enamel
asentar a nivel premolar-molar,4 aun- organ, although other
que se ha descrito una predileccin en authors believe that it is
el maxilar en asiticos.1 Frecuentemen- derived from cellular remains
te se asocia a dientes no erupcionados, of the basal lamina or from
especialmente molares mandibulares the basal layer of gingival
(52%) y quistes odontgenos.4,5 Tam- epithelium.3
bin se ha descrito la afectacin bima- Mandibular involvement is
xilar y la afectacin bilateral.3 Figura 2. Ortopantomografa antes de la intervencin quirrgica. commonly twice that of the
No hay una predileccin de sexo en Figure 2. Orthopantomography before surgery. maxilla, and it tends to arise
la variante central y ms de dos tercios in the premolar-molar area4
de las lesiones aparecen entre la terce- although a predilection has
ra y quinta dcadas de la vida,2 con una been described for the maxil-
media de edad de 40,3 aos en el central y 31,8 en el perifrico.4 la in orientals.1 It is commonly associated with unerupted
teeth, especially mandibular molars (52%) and odontogenic
cysts.4,5 Bimaxillary and bilateral involvement has also been
Caso clnico described.3
There is no sex predilection in the central variety and
El paciente es un varn de 38 aos que acudi al Servicio de more than two thirds of the lesions appear between the
Ciruga Oral y Maxilofacial del Hospital Universitari Vall dHebron, third and fifth decades in life.2 The mean age is 40.3 years
remitido de otro centro, por tumoracin mandibular de 1 ao de for central tumors and 31.8 for peripheral ones.4
evolucin (Fig. 1).
Como antecedentes patolgicos destaca el ser exadicto a dro-
gas por va parenteral, ser VHC positivo y VIH positivo en trata- Case Report
miento con antiretrovirales. A la exploracin se apreciaba una tumo-
racin mandibular derecha de aproximadamente 5 cms de tama- The patient was a 38 year old male that attended the
o, fija y de consistencia dura mantenindose la integridad de la Department of Oral and Maxillofacial Surgery of the Hos-
mucosa oral. En la ortopantomografa apareca una lesin mixta pital Universitari Vall dHebron, having been referred by
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344 Rev Esp Cir Oral y Maxilofac 2007;29,5 (septiembre-octubre):342-347 2007 ergon Tumor de Pindborg (tumor odontognico epitelial calcificante)

con calcificaciones en su interior que another center because of

afectaba la hemimandbula derecha (Fig. a tumor-like mass in the
2). mandible that had been
En la TC se observaba una lesin evolving for a year. (Fig. 1)
mixta expansiva en cuerpo de mand- His medical history includ-
bula derecha que destrua las corticales ed an ex-addiction to par-
de 4,5x3x3,3 cm (Fig. 3). enteral drug use. He was
La biopsia realizada informaba de HCV and HIV positive and
tumor odontognico epitelial calcifican- undergoing antiretroviral
te. therapy. On examination a
El paciente fue sometido a una hemi- right-sided mandibular
mandibulectoma derecha con recons- tumor-like mass could be
truccin microquirrgica con peron appreciated that measured
(Figs. 4 y 5). 5 cms, which was attached
La anatoma patolgica revel una and hard in consistency.
masa de 4 x 2,5 cm de superficie irre- The oral mucosa was
gular con aspecto heterogneo, reas Figura 3. TC antes de la intervencin quirrgica. intact. The orthopanto-
congestivas y restos de piezas dentarias, Figure 3. CT scan before surgery. mography showed a mixed
que confirm el diagnstico de tumor lesion with calcifications in
odontognico epitelial calcificante de its interior that was affect-
mandbula y que infiltraba partes blan- ing the right side of the
das peristicas. El nervio mentoniano no mandible. (Fig. 2) The CAT
presentaba invasin y la biopsia intrao- scan showed an expansive
peratoria de ganglio de cadena facial mixed lesion in the right
izquierda no presentaba metstasis (Figs. mandibular body that was
6 y 7). destroying cortical bone,
La evolucin postoperatoria fue favo- which measured 4.5x3x3.3
rable y fue dado de alta tolerando dieta cm. (Fig. 3) The biopsy car-
y con control de las heridas en consultas ried out reported a calcify-
externas (Figs. 8, 9 y 10). ing epithelial odontogenic
tumor. The patient under-
went a mandibulectomy of
Discusin the right half which was
reconstructed microsurgi-
Clnicamente este tipo de tumores se Figura 4. Fotografa macroscpica de la pieza de extirpacin del cally with fibula. (Figs. 4 y
tumor.
caracterizan por ser asintomticos en el Figure 4. Macroscopic photograph of the resected tumor specimen. 5) The anatomic patholo-
momento del diagnstico, aunque el gy report described a mass
lento crecimento del tumor puede pro- measuring 4x2.5 cm with
ducir efectos mecnicos y a pesar de que an irregular surface and a
se trata de una neoplasia benigna, puede heterogeneous appearance,
invadir localmente.3 El tumor crece por congestive areas and
infiltracin y puede producir una expan- remains of teeth. This con-
sin de la cortical, movimiento dentario firmed the diagnosis of cal-
y resorcin de las races. En algunos casos cifying epithelial odonto-
puede producir dolor, obstruccin nasal, genic tumor of the
epistaxis, cefalea e incluso sangrado.3 A mandible that was infil-
nivel maxilar puede afectar a los senos. trating the soft tissue of the
La tasa de recurrencia oscila entre 10 y Figura 5. Ortopantomografa despus de la intervencin quirr- periosteum. The mental
gica.
14%.1, 4 Figure 5. Orthopantomography after surgery.
nerve showed no invasion,
La variante extrasea suele localizar- and the intraoperative biop-
se en la enca anterior como una masa sy of the left facial system
indolora parecida a un pulis,2 y sin afectacin sea del maxilar aun- lymph node showed no signs of metastasis (Figs. 6 y 7).
que el hueso adyacente suele mostrar una erosin superficial. Su The postoperative period was favorable and he was dis-
comportamiento es menos agresivo que la variante central, lo que charged able to tolerate food. His wounds were checked
permite realizar un tratamiento ms conservador.4 on an outpatient basis (Figs. 8, 9 y 10).
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J. Rubio y cols. Rev Esp Cir Oral y Maxilofac 2007;29,5 (septiembre-octubre):342-347 2007 ergon 345

El diagnstico suele producirse al rea- Discussion

lizar una radiografa rutinaria, por lo que
debido a su potencial carcter invasivo Clinically these types of
local debe realizarse una TC para deter- tumors are typically asymp-
minar la extensin y el tratamiento.3,4 tomatic at diagnosis, and
A nivel radiolgico el tumor pasa por although they grow slowly,
distintas fases evolutivas. Inicialmente el the tumor can have mechan-
tumor es radiolcido como un quiste ical effects. In spite of being
odontgeno (sobre todo cuando est a benign neoplasm, it can be
en relacin con un diente incluido) o un locally invasive.3 The tumor
ameloblastoma qustico. A continuacin grows by means of infiltra-
sigue un patrn mixto aunque algunos tion and there can be expan-
tumores grandes pueden permanecer Figura 6. Fotografa histolgica hematoxilina-eosina (250x): Clu- sion of the cortical bone, den-
las epiteliales regulares sin atipias y presencia de concreciones eosi-
radiolcidos,4 y aparecen calcificaciones nfilas formando estructuras anulares (anillos de Liesegang) y cal- tal movement and root
intralesionales.3 Finalmente aparece un cificaciones. resorption. In some cases
patrn irregular, con reas uni o multi- Figure 6. Histological photograph with hematoxylin-eosin (250x): there may be pain, nasal
Regular epithelial cells showing no atypia can be observed together
loculares como un panal de abejas debi- with eosinophilic deposits forming ring-like structures (Liesegang rings) obstruction, epistaxis,
do a la destruccin y calcificacin tumo- and calcifications. headaches and even bleed-
ral.2,3 El tumor suele estar bien circuns- ing.3 In the maxilla the sinus-
crito radiolgicamente a pesar que los es may be affected. The
mrgenes esclerticos pueden no ser recurrence rate is between
evidentes.2 10 and 14%.1,4
Los tumores perifricos suelen ser The extraosseous type tends
radiolcidos aunque el hueso adyacen- to be located in the anteri-
te puede mostrar erosiones superficia- or gingiva presenting as a
les.4 painless mass similar to an
La TC es til para mostrar las calcifi- epulis.2 There is no involve-
caciones, dientes no erupcionados y las ment of the jaw although the
erosiones seas,1 y suele mostrar una adjacent bone tends to show
lesin bien delimitada, que expande el superficial erosion. Its behav-
hueso, con adelgazamiento de la cor- ior is less aggressive than the
tical, multilocular, con septos seos, Figura 7. Tincin rojo Congo y examen con luz polarizada (250x): central variety, which permits
radiopacidades y dientes incluidos, aun- Se observa birrefringencia de color verde manzana de los anillos more conservative treat-
que en este caso apareca una lesin de de Liesegang. ment.4
carcter infiltrante dado su avanzado Figure 7. Congo red stain and examination with polarized light (250x): The diagnosis tends to arise
Birefringence of an apple-green color can be observed in Liesegangs
estado evolutivo. La resonancia mag- rings. after routine radiography
ntica muestra una lesin hiperintensa and, given its potentially
en T2 e hipointensa en T1, estas carac- invasive nature, a CAT scan
tersticas distinguen al tumor de otras neoplasias ms agresivas.4 should be carried out in order to determine its extension and
Las ventajas de la RM son la ausencia de radiaciones ionizantes y treatment.3,4
una mayor definicin de los tejidos blandos. Radiologically the tumor has different stages of devel-
La histologa es el aspecto ms variable de este tumor. El crite- opment. Initially the tumor is radiolucent, like an odonto-
rio caracterstico para diagnosticarlo son unas masas de grandes genic cyst (especially when it is by an unerupted tooth) or
clulas epiteliales poligonales con bordes bien definidos y puentes like a cystic ameloblastoma. A mixed pattern will then fol-
intercelulares.2 El citoplasma es abundante y eosinoflico. A menu- low, although some larger tumors may remain radiolucent4
do hay pleomorfismo celular y los ncleos suelen ser prominentes and intralesional calcifications will appear.3 Finally, an irreg-
y con una gran variabilidad de tamao, forma y nmero. No sue- ular pattern will be displayed, with uni- or multilocular areas,
len haber mitosis. A nivel extracelular suelen haber acumulaciones like a honeycomb, due to destruction and tumor calcifica-
de material de tipo amiloide con birrefringencia de color verde man- tions.2,3 The tumor tends to be well-circumscribed radio-
zana bajo luz polarizada despus de ser teida con rojo Congo.4 Un logically in spite of having sclerotic margins that may not be
hallazgo muy importante es la calcificacin concntrica de este visible.2 The peripheral tumors tend to be radiolucent although
material amiliode formando los anillos de Liesegang, que son patog- the adjacent bone tends to have superficial erosion.4
nomnicos de este tumor.2 Este material amiloide calcificado pro- The CAT scan is useful for showing calcification, unerupt-
duce un estmulo sobre el estroma tumoral que lo lleva a segregar ed teeth and bone erosion.1 It will reveal a well-defined mul-
una matriz de colgeno posteriormente calcificable.3 tilocular lesion expanding the bone, together with thinning of
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346 Rev Esp Cir Oral y Maxilofac 2007;29,5 (septiembre-octubre):342-347 2007 ergon Tumor de Pindborg (tumor odontognico epitelial calcificante)

Se han descrito distintas variantes de the cortical layer, bony sep-

TOEC: de clulas claras (el cual suele tener tum, radio-opacity and
un comportamiento ms agresivo y tiene unerupted teeth, although in
una tasa de recurrencia mayor),2 de clu- this case an infiltrative type of
las de Langerhans, de clulas mioepite- lesion was shown given its
liales o formador de cemento. advanced stage. Magnetic res-
Segn la radiologa debe realizarse onance will show a hyperin-
el diagnstico diferencial con el amelo- tense lesion on T2 weighted
blastoma, el mixoma, el quiste seo sequences and hypointense
aneurismtico y los quistes odontge- on T1. These characteristics
nos.4 Tambin deben desacartarse el distinguish the tumor from
granuloma de clulas gigantes, el fibro- Figura 8. Fotografa de frente antes y despus de la intervencin other neoplasms that are
quirrgica.
ma osificante-cementificante, el fibro- more aggressive.4 RMI has the
Figure 8. Face-on photograph before and after surgery.
dontoma ameloblstico y el fibroma advantage of not using ioniz-
ameloblstico. Si la lesin presenta un ing radiation and soft tissue is
sangrado importante debe realizarse el better defined.
diagnstico diferencial con el angio- The more changeable aspect
ma central mandibular.3 Si hay presen- of this tumor is its histology.
cia de clulas claras se deben conside- The characteristic criteria for
rar el carcinoma odontgeno de clu- diagnosing it are large polyg-
las claras, el ameloblastoma de clulas onal epithelial cells with well-
claras, el carcinoma intraseo, las neo- defined borders and intercel-
plasias de glndulas salivares y el oste- lular bridges.2 The cytoplasm
oblastoma agresivo.5 is abundant and eosinophilic.
La base del tratamiento es la ciruga There is often cellular pleo-
y sta debe individualizarse en cada caso morphism and the nuclei
segn la extensin, localizacin y carac- tend to be prominent and
tersticas del paciente. Si se opta por un Figura 9. Fotografa de perfil antes y despus de la intervencin with a great variety of sizes,
quirrgica.
tratamiento conservador mediante enu- shapes and numbers. Mito-
Figure 9. Photograph showing profile before and after surgery.
cleacin o curetaje incluyendo un mar- sis tends not to be seen. At
gen de tejido sano junto con un pro- an extracellular level there
longado seguimiento, debe considerarse tends to be accumulation of
que la tasa de recidiva es del 15-30% a an amyloid type of material
los 2-4 aos.4 Por lo tanto, debido a su with birefringence of an
carcter infiltrativo, es preferible la resec- apple-green color under
cin con un cm de tejido sano clnica y polarized light after Congo
radiolgicamente (0,5 en los perifricos red staining.4 A very impor-
por su menor agresividad) consiguien- tant finding is concentric cal-
do as una ausencia de recidiva. En los cification in the amyloid
casos en que afecte al maxilar o sea la material that will form
variante de clulas claras debe tratarse Liesegang rings, which are
de manera ms agresiva.5 Consecuen- pathognomonic for this
temente la mejor forma de tratamien- tumor.2 This calcified amyloid
to es incluir unos mrgenes de 1-1,5 cm Figura 10. Fotografa basal antes y despus de la intervencin qui- material stimulates the tumor
rrgica.
junto con un seguimiento de 5-10 aos.4 stroma which leads it to seg-
Figure 10. Bottom-up photograph before and after surgery.
regate a collagenous matrix
which will calcify.3
Conclusiones Different varieties of CEOT have been described: clear
cell (which tends to be more aggressive and that has a greater
El tumor de Pindborg es una lesin benigna de muy baja fre- recurrence rate),2 Langerhans cell, myoepithelial cell or
cuencia pero que debido a su comportamiento impredecible y a su cementum-forming.
radiologa inespecfica obliga a la realizacin de un estudio anato- The differential diagnosis should include ameloblastoma,
mopatolgico y una ciruga resectiva para minimizar la probabili- myxoma, aneurismatic bone cyst and odontogenic cysts,
dad de recidiva, junto con un seguimiento postoperatorio de al depending on the radiographs.4 Giant cell granuloma should
menos cinco aos. also be ruled out, together with cemento-ossifying fibro-
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J. Rubio y cols. Rev Esp Cir Oral y Maxilofac 2007;29,5 (septiembre-octubre):342-347 2007 ergon
Bibliografa
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2. Germanier Y, Bornstein MM, Stauffer E, Buser D, Calcifying epithelial odonto-
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3. Belmonte-Caro R, Torres-Lagares D, Mayorga-Jimnez F, Garca-Perla Garca A,
Infante-Cossio P, Gutirrez-Prez JL, Calcifying epithelial odontogenic tumor
(Pindborg tumor). Med Oral 2002;7:309-15.
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romn B, Calcifying epithelial odontogenic (pindborg) tumor: a series of 4
distinctive cases and a review of the literature. J Oral Maxilofac Surg 2005;63:1361-
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5. Maiorano E, Renne G, Tradati N, Viale G. Cytogical features of calcifying epit-
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rial, Virchows Arch 2003;442:107-10.
347
ma, ameloblastic fibro-odontoma and ameloblastic fibroma.
If there is considerable bleeding the differential diagnosis
should include central angioma of the mandible.3 If there
are clear cells, the clear cell odontogenic carcinoma should
also be considered together with clear cell ameloblastoma,
intraosseous carcinoma, salivary gland neoplasms and aggres-
sive osteoblastoma.5
The treatment is basically surgical and this should be
tailored in each case according to the extension, location
and characteristics of the patient. If conservative treatment
is elected entailing enucleation, or curettage with a healthy
tissue margin, together with a prolonged follow-up, the recur-
rence rate of 15-30% over 2-4 years should be taken into
account.4 Therefore, due to its infiltrative nature, a clinical
and radiological resection with a one centimeter margin of
healthy tissue is preferable (0.5 for peripheral tumors as they
are less aggressive) and a recurrence-free outcome will in
this way be achieved. In cases affecting the jaw, in other
words the clear cell variety, a more aggressive approach
should be taken.5 As a result the best treatment method is
to include 1-1.5 cm margins together with a follow-up peri-
od of 5-10 years.4
Conclusions
Pindborg tumor is a rare benign lesion. Given its unpre-
dictable behavior and its unspecific radiological findings, car-
rying out an anatomopathologic study is necessary, as is
resective surgery in order to minimize the probability of recur-
rence. Also necessary is a postoperative follow-up of at least
five years.