otolaryngologia polska 66 (2012) 359–362
Available online at www.sciencedirect.com
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Case report/Kazuistyka
Ossifying fibroma–cementoma of jaw. Differences in
histopathological nomenclature
Włókniak kostnieja˛ cy – cementoma szcze˛ki. Różnice w nomenklaturze
histopatologicznej
Wiesław Konopka 1,2,*, Małgorzata Śmiechura 1, Małgorzata Strużycka 1,
Marcin Kozakiewicz 3, Monika Dzieniecka 4
1
Department of Otolaryngology Polish Mother's Memorial Hospital-Research Institute, Lodz, Poland
Department of Audiology, Phoniatry and Paediatric Otolaryngology Medical University of Lodz, Poland
Head: Wiesław Konopka
3
Department of Maxillar and Facial Surgery Medical University of Lodz, Poland
Head: Marcin Kozakiewicz
4
Department of Patomorphology Polish Mother's Memorial Hospital-Research Institute, Lodz, Poland
Head: Andrzej Kulig
2
article info
abstract
Article history:
Ossifying fibroma (cementoma) is a tumor of mesenchymal origin which represents about
Received: 25.05.2012
1% of odontogenic tumors. It is commonly found in patients under 25, more often so in
Accepted: 26.06.2012
women. As its growth is slow and painless, it is usually accidentally detected by dental
Published on line: 04.07.2012
radiological examination. The aim of our study was to present the histopathological
dilemma concerning the naming of a rare odontogenic tumor of the jaw. The authors
Keywords:
Cementoma
present a rare jaw tumor, a benign ossifying fibroma, in the maxilla of a 12-year-old girl
Histopathological nomenclature
Jaw tumor
diagnosis. The clinical and histological criteria for identifying this type of tumor are still
treated surgically, and they discuss the difficulty in making a definitive histopathological
uncertain, as the most common sites, that is the tooth-bearing areas of the mandible, are
very rare in the maxilla. The differentiation from the central fibro-osseous lesions in the
Słowa kluczowe:
włókniak kostnieja˛cy
nomenklatura histopatologiczna
guz szcze˛ki
maxilla bones is discussed. The final diagnosis of ossifying fibroma was based on the WHO
classification. A literature search reveals a fundamental flaw in defining a unified classification for this type of change. As there is no clear diagnostic criterion, few repeatable
diagnoses can be found. Although individual researchers tend to use their own means of
classification in their routine work, the WHO classification should be applied.
© 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by
Elsevier Urban & Partner Sp. z o.o. All rights reserved.
* Corresponding author at: Department of Otolaryngology Polish Mother’s Memorial Hospital-Research Institute Rzgowska 281/289, 93-338
Łódź, Poland. Tel.: +48 502510083/422711701.
E-mail address: [email protected] (W. Konopka).
0030-6657/$ – see front matter © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
http://dx.doi.org/10.1016/j.otpol.2012.06.027
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otolaryngologia polska 66 (2012) 359–362
Introduction
Ossifying fibroma (cementoma) is a slow-growing benign
neoplasm composed of fibroblastic and osseous components,
which occurs throughout the skull but most commonly, in the
mandible and maxilla. It is of mesenchymal origin and arises
from the cementum, one of the tissues forming the root of the
tooth, and represents about 1% of odontogenic tumors [1]. It
occurs most often in young people under 25 years of age, more
often in women than in men. According to Eversole [2] in 75–
89% of cases, the tumor occurs in the mandible, especially in
its rear section, near the premolars and molars. It rarely occurs
in episodes or in the vicinity of the front teeth. The tumor
grows very slowly, about 0.5 cm per year, and does not present
any clinical symptoms for a long time. It is often detected by
chance on teeth after a radiological inspection; the greatest
diagnostic value can be attributed to the pantomographic
X-rays often requested by dentists. The jaw bones, paranasal
sinuses, nasopharynx, and orbit are frequently found to be
more active and demonstrate a juvenile variety of ossifying
fibroma (juvenile cemento-ossifying fibroma). The juvenile
variant of the tumor is prone to grow much more rapidly,
tending toward the destruction of surrounding tissue and
penetration of the surrounding space, leading to alveolar
distension and displacement of bone resorption, as well as
paresthesia, pain, swollen facial bones and loose teeth [1].
Although the etiopathogenesis of cementoma is not completely understood, injuries, genetic disorders, hormonal disorders and calcium-phosphate are among the likely factors that
contribute to its development. Four types can be distinguished
based on tissue type: cementoma ossificans – the most
common form, benign cementoma, giant cementoma and
periapical cement dysplasia.
The characteristic histopathologic features of the structure
is the presence of a tumor capsule, a rich cellular connective
tissue stroma, spindle cells and an immature trabecular
bone and masses which looks like cement. Radiological images
of cementomas, especially the larger ones, display highly
characteristic features. The typical radiological image is
composed of dense bone structure around the tooth root in
a relatively regular circular shape, around which bone
rarefaction can be seen, due to resorption, and rather ‘‘fuzzy’’
surrounding tissue. Cementomas should be treated surgically
by radical resection of the tumor along with the neighboring
healthy tissue. Although large tumors require the use of
diagnostic computed tomography (CT), a variation of conical
computed tomography, with a much higher resolution than CT,
is available for use in this type of examination.
Aim
The aim of our study was to present the histopathological
difficulty faced in naming a rare odontogenic tumor of the jaw.
This study addresses the case of an 11-year-old girl who was
admitted to the Department of Otolaryngology with a suspected
tumor in the left maxillary sinus, forming a lump in the cheek.
From the interview, it was known that the child was born with
a congenital malformation in the form of median cleft upper lip,
alveolus and hard and soft palate. She had already been
operated on twice at the ages of 6 and 18 months.
The disease was observed for several months, gradually
expanding and causing a marked asymmetry of the face.
Enlargement of the tumor caused pain in the area. The child did
not report facial sensory disturbances. Laryngological examination found facial asymmetry with a lump on the left cheek,
asymmetry of the external nose and curvature of the nasal
septum on the right side, which partially impaired patency. In
the vestibule of the mouth, a hard swelling was found on the
alveolar process on the left side of the maxilla. In the oral cavity
and pharynx, scars from numerous reconstructive surgeries of
the palate were visible; the soft palate was short and
asymmetric. The results of laboratory tests were normal.
A CT scan of the sinuses showed that the left maxillary sinus
was filled mostly by sclerotic tissue with numerous calcifications, without evidence of malignant bone and without
the presence of periosteal reactions. The lesion measured
45 43 mm.
The front part of the normal sinus aeration was separated
from the tumor by a bone lamella. Hypoplasia of the lower
right nasal concha was present, as were signs of a history of
plastic surgery for cleft palate. The patient was qualified to
provide material for histopathological examination.
Under general anesthesia, the left maxillary sinus was
opened according to standard procedure through the vestibule
of the oral cavity. The anterior wall of the sinus was thin and
brittle. A white tumor fragment was removed; it had a gray,
solid consistency and did not bleed when touched. The tumor
easily separated from the wall bone. An initial histological
examination (2098/11) indicated that the tumor was an
odontogenic fibroma.
The girl was qualified for surgical treatment. The tumor
was reached through the side of the oral vestibule; it was
dissected from the surrounding tissues and removed. Revision
of the postoperative cavity was performed by removing the
remaining tumor fragments penetrating further into the sinus
space. The bony walls of the cavity were smooth and did not
display any signs of postoperative destruction or malignant
invasion. A visual inspection of the excised tissue revealed
a gray–white, smooth, springy tumor measuring 60 60 mm
with no bleeding. An anterior osteoplasty was then performed.
The size of the postoperative cavity in the upper and lower
sinus cavity wall and the degree of facial asymmetry were
minimized by moving the peripheral bone lamellae toward
the center of the opening in the wall of the maxillary sinus.
The tumor mass was submitted for histopathological examination. The treatment was well tolerated.
The postoperative treatment included analgesics and antiinflammatory drugs. On the seventh postoperative day, a CT
scan was performed which showed the increased mucosal
edema in the medial upper-left maxillary sinus and the area of
more intense contrast on the lateral side, which derived from
the local postoperative edema. The continuity of the bone wall
of the left maxillary sinus was broken in a number of places
and unattached fragments of bone left after angioplasty were
visible. On the eighth day, the girl, being in good overall
condition was discharged and allowed to go home.
According to the histopathological examination (3681/11),
the dominant element in the specimen was a proliferation of
otolaryngologia polska 66 (2012) 359–362
[(Fig._1)TD$IG]
361
revealed areas of round or ovoid, strongly alkaline, calcic noncellular structures which, according to some authors [3–5],
resemble bodies of the cement. A small percentage of the tumor
texture was also made up of pseudo-cystic field structures
(regressive changes), around which clusters of giant polynuclear osteoclast cells were present. Mitotic figures were not found
in the examined tumor specimens (Fig. 1, Fig. 2).
According to the current WHO classification [6], ossifying
fibroma was diagnosed. The girl remains under constant ENT
observation. Three months after the surgery the asymmetry of
the face and the bulge in the vestibule of the mouth have
significantly decreased. There has been no recurrence of the
tumor.
Discussion
Fig. 1 – In the centre can be seen degenerative changes in
the form of pseudocystic structures of blood clots and
numerous giant multi-nucleate osteoclasts. In the lower
right corner can be seen a large calcification and
numerous small, spherical calcifications. H&E,
magnification 40T
fibrous tissue, within which were visible fragments of compact
bone and a number of ‘‘scattered balls’’ highly saturated with
calcium salts, which may reflect the composition of the
cement. The image corresponds to the morphological diagnosis of odontogenic fibroma–cementoma. Given the different
definitions of this type of tumor pathology encountered in the
literature, anatomical pathologists were once again requested
to interpret the test results. It was found that the dominant
element within the microscopic image of the tumor was a rich,
fibrous connective tissue separated by less numerous areas
built mainly of collagen bundles. Changes in the weaving of
the trabecular bone were found; some of them were
surrounded by a layer of osteoblasts at the periphery
(‘‘osteoblastic rimming’’). A histological analysis of the tumor
[(Fig._2)TD$IG]
Fig. 2 – Stroma rich in fibrous tissue from the trabecula
bone formation and scattered calcification H&E,
magnification 100T
Ossifying fibroma (ossifying fibroma–cementoma) is a benign
tumor rarely located in the jaw. Its etiology is unclear but is
mentioned in the literature as a post-traumatic etiology
initiating the process of lesion development [7]. The growth
of the tumor is often slow without symptoms. It most
frequently occurs around the premolar area of the mandible,
however, it also appears in the maxilla in 33% of cases. Its
occurrence is usually associated with the arrested development of a tooth. Ossifying fibroma should be differentiated
from a Pindborg tumor, which is a form of infiltrative tumor
causing localized destruction [8]. It is derived from the stratum
intermedium and although it has less growth potential than
an ameloblastoma, local recurrence is not uncommon after
non-radical removal.
Three stages of clinical and radiological dynamics are
mentioned in tumor development. The first is characterized by
a process of destructive osteolysis and fibrous degeneration of
bones, the second, by partial bone calcification and the third,
by total calcification [7, 9, 10]. The location and size of the
maxillary tumor described in this study are unusual, especially in children. The patient’s medical history and existing
developmental disorders including occlusal abnormalities
seem to be potential factors exacerbating symptoms of the
growth of the tumor.
Tumors arising from tooth germs or particular dental tissues
are classified histologically as benign odontogenic tumors
associated with tissue components. Pindborg and Kramer
[in [8]] are the creators of a histological and radiological
classification of tumors. The clinical, microscopic and radiological characteristics of the tumors encountered in practice
depends on their phase of odontogenesis, their location,
severity and development, as well as the mineralization of
the tissue constituting the tumor.
In contrast to most types of odontogenic tumors, it is
believed that tumors of this type (Ossifying fibroma) are
formed alongside, not instead of regular teeth; initial damage
and later fibrosis around the developing tooth germs contribute to tumor growth, as well as the initial and the
subsequent growth of periodontal fibers [1, 8, 11, 12]. Ossifying
fibroma is usually described as a periapical osteofibroma,
cementifying fibroma, cementoblastoma, sclerosing cementoma, fibrocementoma, periapical fibrous dysphasia or odontogenic fibroma, depending on the morphology [4, 6, 9, 13]. As
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otolaryngologia polska 66 (2012) 359–362
in this case, cementomas are the most capsulated form of
tumor and are well demarcated from the environment; mature
tumors are usually highly mineralized, and surgery may be
required to remove a tumor even with the bone fragment.
Two types of cementoma can be distinguished based on
their histopathology: fibrocementoma and sclerosis cementoma. An additional type known as a ‘‘limited growth tumor’’
is derived from cementoblasts of the periodontal membrane
surrounding the root apex [9, 10].
According to the WHO classification above, [3] two
variants of this type of tumor exist: the juvenile trabecular
ossifying fibroma and the juvenile psammomatoid ossifying
fibroma. The diagnostic criteria of these two variants have
much in common, therefore large disparities exist in
the diagnoses of these tumors among pathologists [4, 6].
In the case of the tumor described in this study, subsequent
microscopic images from the same sample fulfilled the
criteria for both variants of ossifying fibroma, according to
the WHO classification [3]. Similar reports of the coexistence
of both variants in a single tumor (juvenile trabecular and
psammomatoid variant of ossifying fibroma) can be found in
the literature [14, 15]. Such internal diversity of the
morphology of the tumor makes it difficult to determine
the correct diagnosis.
According to Stachura and Domagala [10], bone lesions
made of fibrous connective tissue and bone tissue can be
placed on a continuum with fibrous dysplasia at one end and
ossifying fibroma at the other. Since these entities have
similar microscopic images, a correct diagnosis therefore
requires a joint analysis of histological, radiological and
clinical data (clinical and pathological diagnosis).
Conclusion
A query of the literature reveals a fundamental flaw in
a unified classification of this type of tumor. There appears to
be no clear diagnostic criteria and, therefore, repeatable
diagnoses are few and far between. Most commonly, diagnoses established as part of everyday practice are based on the
classifications used by the individual physician as part of his
job; however the WHO classification should be used.
Authors’ contributions/Wkład autorów
W. Konopka, M. Śmiechura – study design, data collection,
data interpretation, acceptance of final manuscript version,
literature search, funds collection; M. Strużycka – study
design, data collection, data interpretation, literature search,
funds collection; M. Kozakiewicz – study design, literature
search; M. Dzieniecka – data interpretation.
Conflict of interest/Konflikt interesu
None declared.
r e f e r e n c e s / p i ś m i e n n i c t w o
[1] Hermes D, Krger S, Entenmann A. Odontogenic tumors in
childhood – case report and review of literature. Klinische
Padiatrie 2007;219(4):234–239.
[2] Eversole LR, Leider AS, Nelson K. Ossifying fibroma:
a clinicopathologic study of sixty four cases. Oral Surgery
Oral Medicine Oral Pathology 1985;60:505–511.
[3] World Health Organization Classification of Tumours,
International Agency for Research on Cancer (IARC). In:
Barnes L, Eveson JW, Sidransky PD, editors. Pathology and
genetics of head and neck tumours. Lyon: IARC Press; 2005.
[4] Juan Rosai Surgical Pathology, wyd. Mosby red. Rosai and
Ackerman’s, 2004;I:283–284.
[5] Summerlin DJ, Tomich CE. Focal cemento-osseous
dysplasia: a clinicopathologic study of 221 cases. Oral
Surgery Oral Medicine Oral Pathology 1994;78:611–620.
[6] Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE.
Gigantiform cementoma: Clinicopathologic presentation of
3 cases. Oral Surgery Oral Medicine Oral Pathology Oral
Radiology and Endodontics 2001;91:438–444.
[7] Burduk PK, Bilewicz R, Galke K, Marszałek A.
Fibrocementoma of the right maxilla – a case report.
Medical and Biological Sciences 2010;24.3:51–54.
[8] Kaczmarzyk T, Stypułkowska J, Tomaszewska R, Czopek J,
Nowotwory ze˛bopochodne i guzy nowotworopodobne kości
szcze˛kowych. Wyd. Kwintesencja, Warszawa 2009.
[9] Chang CC, Hung HY, Yu-Fong Chang J, Yu CH, Wang YP, Liu
BY, et al. Central ossifying fibromas: a clinicopathologic
study of 28 cases. Journal of the Formosan Medical
Association 2008;107(4):288–294.
[10] Stachura J, Domagała W, Patologia T. II wyd. Polska
Akademia Umieje˛tności, Kraków 2005.
[11] Su L, Weathers DR, Waldron CA. Distinguishing features of
focal cemento-osseous dysplasias and cemento-ossifying
fibromas: I. A pathologic spectrum of 316 cases. Oral Surgery
Oral Medicine Oral Pathology Oral Radiology and
Endodontics 1997;84:301–309.
[12] Voytek TM, Ro JY, Edeiken J, Ayala AG. Fibrous dysplasia
and cement-ossifying fibroma. American Journal of
Surgical Pathology 1995;7:775–781.
[13] Bielecki I, Mniszek J, Zielińska-Pa jak E, Pajak J, Kluczewska E,
Zygan L. Otolaryngol Pol Cemento-ossifying fibroma – opis
dwóch przypadków i przegla˛d literatury 2005;59(1):109–114.
[14] Emerson LP, Alexander M, Job A. Juvenile trabecular and
psamomatoid variant of ossifying of the maxilla with
secondary aneurysmal bone cyst. The Internet Journal of
Otorhinolaryngology 2012;14:1.
[15] Toyosawa S, Yuki M, Kishino M, Ogawa Y, Ueda T,
Murakami S, et al. Ossifying fibroma vs fibrous dysplasia of
the jaw: molecular and immunological characterization.
Modern Pathology 2007;20:389–396.