Ossifying Fibroma–Cementoma of Jaw. Differences in Histopathological

otolaryngologia polska 66 (2012) 359–362

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Case report/Kazuistyka Ossifying ﬁbroma–cementoma of jaw. Differences in histopathological nomenclature Wło´kniak kostnieja˛cy – cementoma szcze˛ki. Ro´z˙nice w nomenklaturze histopatologicznej

Wiesław Konopka 1,2,*,Małgorzata S´ miechura 1,Małgorzata Struz˙ycka 1, Marcin Kozakiewicz 3, Monika Dzieniecka 4

1 Department of Otolaryngology Polish Mother's Memorial Hospital-Research Institute, Lodz, Poland 2 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 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: present a rare jaw tumor, a benign ossifying fibroma, in the maxilla of a 12-year-old girl Cementoma treated surgically, and they discuss the difficulty in making a definitive histopathological Histopathological nomenclature diagnosis. The clinical and histological criteria for identifying this type of tumor are still Jaw tumor 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 maxilla bones is discussed. The final diagnosis of ossifying fibroma was based on the WHO Słowa kluczowe: classification. A literature search reveals a fundamental flaw in defining a unified classi- wło´ kniak kostnieja˛cy fication for this type of change. As there is no clear diagnostic criterion, few repeatable nomenklatura histopatologiczna diagnoses can be found. Although individual researchers tend to use their own means of guz szcze˛ki 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 Ło´ dz´, 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.2012.06.027 360 otolaryngologia polska 66 (2012) 359–362

alveolus and hard and soft palate. She had already been Introduction operated on twice at the ages of 6 and 18 months. The disease was observed for several months, gradually Ossifying fibroma (cementoma) is a slow-growing benign expanding and causing a marked asymmetry of the face. neoplasm composed of fibroblastic and osseous components, Enlargement of the tumor caused pain in the area. The child did which occurs throughout the skull but most commonly, in the not report facial sensory disturbances. Laryngological exami- mandible and maxilla. It is of mesenchymal origin and arises nation found facial asymmetry with a lump on the left cheek, from the cementum, one of the tissues forming the root of the asymmetry of the external nose and curvature of the nasal tooth, and represents about 1% of odontogenic tumors [1].It septum on the right side, which partially impaired patency. In occurs most often in young people under 25 years of age, more the vestibule of the mouth, a hard swelling was found on the often in women than in men. According to Eversole [2] in 75– alveolar process on the left side of the maxilla. In the oral cavity 89% of cases, the tumor occurs in the mandible, especially in and pharynx, scars from numerous reconstructive surgeries of its rear section, near the premolars and molars. It rarely occurs the palate were visible; the soft palate was short and in episodes or in the vicinity of the front teeth. The tumor asymmetric. The results of laboratory tests were normal. grows very slowly, about 0.5 cm per year, and does not present A CT scan of the sinuses showed that the left maxillary sinus any clinical symptoms for a long time. It is often detected by was filled mostly by sclerotic tissue with numerous calcifica- chance on teeth after a radiological inspection; the greatest tions, without evidence of malignant bone and without diagnostic value can be attributed to the pantomographic the presence of periosteal reactions. The lesion measured X-rays often requested by dentists. The jaw bones, paranasal 45 Â 43 mm. sinuses, nasopharynx, and orbit are frequently found to be The front part of the normal sinus aeration was separated more active and demonstrate a juvenile variety of ossifying from the tumor by a bone lamella. Hypoplasia of the lower fibroma (juvenile cemento-ossifying fibroma). The juvenile right nasal concha was present, as were signs of a history of variant of the tumor is prone to grow much more rapidly, plastic surgery for cleft palate. The patient was qualified to tending toward the destruction of surrounding tissue and provide material for histopathological examination. penetration of the surrounding space, leading to alveolar Under general anesthesia, the left maxillary sinus was distension and displacement of bone resorption, as well as opened according to standard procedure through the vestibule paresthesia, pain, swollen facial bones and loose teeth [1]. of the oral cavity. The anterior wall of the sinus was thin and Although the etiopathogenesis of cementoma is not comple- brittle. A white tumor fragment was removed; it had a gray, tely understood, injuries, genetic disorders, hormonal disor- solid consistency and did not bleed when touched. The tumor ders and calcium-phosphate are among the likely factors that easily separated from the wall bone. An initial histological contribute to its development. Four types can be distinguished examination (2098/11) indicated that the tumor was an based on tissue type: cementoma ossificans – the most odontogenic fibroma. common form, benign cementoma, giant cementoma and The girl was qualified for surgical treatment. The tumor periapical cement dysplasia. was reached through the side of the oral vestibule; it was The characteristic histopathologic features of the structure dissected from the surrounding tissues and removed. Revision is the presence of a tumor capsule, a rich cellular connective of the postoperative cavity was performed by removing the tissue stroma, spindle cells and an immature trabecular remaining tumor fragments penetrating further into the sinus bone and masses which looks like cement. Radiological images space. The bony walls of the cavity were smooth and did not of cementomas, especially the larger ones, display highly display any signs of postoperative destruction or malignant characteristic features. The typical radiological image is invasion. A visual inspection of the excised tissue revealed composed of dense bone structure around the tooth root in a gray–white, smooth, springy tumor measuring 60 Â 60 mm a relatively regular circular shape, around which bone with no bleeding. An anterior osteoplasty was then performed. rarefaction can be seen, due to resorption, and rather ‘‘fuzzy’’ The size of the postoperative cavity in the upper and lower surrounding tissue. Cementomas should be treated surgically sinus cavity wall and the degree of facial asymmetry were by radical resection of the tumor along with the neighboring minimized by moving the peripheral bone lamellae toward healthy tissue. Although large tumors require the use of the center of the opening in the wall of the maxillary sinus. diagnostic computed tomography (CT), a variation of conical The tumor mass was submitted for histopathological exami- computed tomography, with a much higher resolution than CT, nation. The treatment was well tolerated. is available for use in this type of examination. The postoperative treatment included analgesics and anti- inflammatory drugs. On the seventh postoperative day, a CT scan was performed which showed the increased mucosal Aim edema in the medial upper-left maxillary sinus and the area of more intense contrast on the lateral side, which derived from The aim of our study was to present the histopathological the local postoperative edema. The continuity of the bone wall difficulty faced in naming a rare odontogenic tumor of the jaw. of the left maxillary sinus was broken in a number of places This study addresses the case of an 11-year-old girl who was and unattached fragments of bone left after angioplasty were admitted to the Department of Otolaryngology with a suspected visible. On the eighth day, the girl, being in good overall tumor in the left maxillary sinus, forming a lump in the cheek. condition was discharged and allowed to go home. From the interview, it was known that the child was born with According to the histopathological examination (3681/11), a congenital malformation in the form of median cleft upper lip, the dominant element in the specimen was a proliferation of otolaryngologia polska 66 (2012) 359–362 361 [(Fig._1)TD$FIG]

revealed areas of round or ovoid, strongly alkaline, calcic non- cellular 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 polynuc- lear 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 Ossifying fibroma (ossifying fibroma–cementoma) is a benign the form of pseudocystic structures of blood clots and tumor rarely located in the jaw. Its etiology is unclear but is numerous giant multi-nucleate osteoclasts. In the lower mentioned in the literature as a post-traumatic etiology right corner can be seen a large calcification and initiating the process of lesion development [7]. The growth numerous small, spherical calcifications. H&E, of the tumor is often slow without symptoms. It most magnification 40T 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 develop- fibrous tissue, within which were visible fragments of compact ment of a tooth. Ossifying fibroma should be differentiated bone and a number of ‘‘scattered balls’’ highly saturated with from a Pindborg tumor, which is a form of infiltrative tumor calcium salts, which may reflect the composition of the causing localized destruction [8]. It is derived from the stratum cement. The image corresponds to the morphological diagno- intermedium and although it has less growth potential than sis of odontogenic fibroma–cementoma. Given the different an ameloblastoma, local recurrence is not uncommon after definitions of this type of tumor pathology encountered in the non-radical removal. literature, anatomical pathologists were once again requested Three stages of clinical and radiological dynamics are to interpret the test results. It was found that the dominant mentioned in tumor development. The first is characterized by element within the microscopic image of the tumor was a rich, a process of destructive osteolysis and fibrous degeneration of fibrous connective tissue separated by less numerous areas bones, the second, by partial bone calcification and the third, built mainly of collagen bundles. Changes in the weaving of by total calcification [7, 9, 10]. The location and size of the the trabecular bone were found; some of them were maxillary tumor described in this study are unusual, espe- surrounded by a layer of osteoblasts at the periphery cially in children. The patient’s medical history and existing [(Fig._2)TD$FIG](‘‘osteoblastic rimming’’). A histological analysis of the tumor 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, Fig. 2 – Stroma rich in fibrous tissue from the trabecula cementifying fibroma, cementoblastoma, sclerosing cemen- bone formation and scattered calcification H&E, toma, fibrocementoma, periapical fibrous dysphasia or odon- magnification 100T togenic fibroma, depending on the morphology [4, 6, 9, 13].As 362 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 Conflict of interest/Konflikt interesu tumors are usually highly mineralized, and surgery may be required to remove a tumor even with the bone fragment. None declared. Two types of cementoma can be distinguished based on their histopathology: fibrocementoma and sclerosis cemen- references/pis´ miennictwo toma. An additional type known as a ‘‘limited growth tumor’’ is derived from cementoblasts of the periodontal membrane surrounding the root apex [9, 10]. [1] Hermes D, Krger S, Entenmann A. Odontogenic tumors in According to the WHO classification above, [3] two childhood – case report and review of literature. Klinische variantsofthistypeoftumorexist:thejuveniletrabecular Padiatrie 2007;219(4):234–239. ossifying fibroma and the juvenile psammomatoid ossifying [2] Eversole LR, Leider AS, Nelson K. Ossifying fibroma: fibroma. The diagnostic criteria of these two variants have a clinicopathologic study of sixty four cases. Oral Surgery much in common, therefore large disparities exist in Oral Medicine Oral Pathology 1985;60:505–511. the diagnoses of these tumors among pathologists [4, 6]. [3] World Health Organization Classification of Tumours, In the case of the tumor described in this study, subsequent International Agency for Research on Cancer (IARC). In: Barnes L, Eveson JW, Sidransky PD, editors. Pathology and microscopic images from the same sample fulfilled the genetics of head and neck tumours. Lyon: IARC Press; 2005. criteria for both variants of ossifying fibroma, according to [4] Juan Rosai Surgical Pathology, wyd. Mosby red. Rosai and the WHO classification [3]. Similar reports of the coexistence Ackerman’s, 2004;I:283–284. of both variants in a single tumor (juvenile trabecular and [5] Summerlin DJ, Tomich CE. Focal cemento-osseous psammomatoid variant of ossifying fibroma) can be found in dysplasia: a clinicopathologic study of 221 cases. Oral the literature [14, 15]. Such internal diversity of the Surgery Oral Medicine Oral Pathology 1994;78:611–620. [6] Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE. morphology of the tumor makes it difficult to determine Gigantiform cementoma: Clinicopathologic presentation of the correct diagnosis. 3 cases. Oral Surgery Oral Medicine Oral Pathology Oral According to Stachura and Domagala [10], bone lesions Radiology and Endodontics 2001;91:438–444. made of fibrous connective tissue and bone tissue can be [7] Burduk PK, Bilewicz R, Galke K, Marszałek A. placed on a continuum with fibrous dysplasia at one end and Fibrocementoma of the right maxilla – a case report. ossifying fibroma at the other. Since these entities have Medical and Biological Sciences 2010;24.3:51–54. ł similar microscopic images, a correct diagnosis therefore [8] Kaczmarzyk T, Stypu kowska J, Tomaszewska R, Czopek J, Nowotwory ze˛bopochodne i guzy nowotworopodobne kosći requires a joint analysis of histological, radiological and szcze˛kowych. Wyd. Kwintesencja, Warszawa 2009. clinical data (clinical and pathological diagnosis). [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 Conclusion Association 2008;107(4):288–294. [10] Stachura J, Domagała W, Patologia T. II wyd. Polska A query of the literature reveals a fundamental flaw in Akademia Umieje˛tnosći, Krako´ w 2005. [11] Su L, Weathers DR, Waldron CA. Distinguishing features of a unified classification of this type of tumor. There appears to focal cemento-osseous dysplasias and cemento-ossifying be no clear diagnostic criteria and, therefore, repeatable fibromas: I. A pathologic spectrum of 316 cases. Oral Surgery diagnoses are few and far between. Most commonly, diagno- Oral Medicine Oral Pathology Oral Radiology and ses established as part of everyday practice are based on the Endodontics 1997;84:301–309. classifications used by the individual physician as part of his [12] Voytek TM, Ro JY, Edeiken J, Ayala AG. Fibrous dysplasia job; however the WHO classification should be used. and cement-ossifying fibroma. American Journal of Surgical Pathology 1995;7:775–781. [13] Bielecki I, Mniszek J, Zielin´ ska-Pa jak E, Pajak J, Kluczewska E, Authors’ contributions/Wkład autoro´ w Zygan L. Otolaryngol Pol Cemento-ossifying fibroma – opis dwoćh przypadko´w i przegla˛d literatury 2005;59(1):109–114. [14] Emerson LP, Alexander M, Job A. Juvenile trabecular and W. Konopka, M. S´ miechura – study design, data collection, psamomatoid variant of ossifying of the maxilla with data interpretation, acceptance of final manuscript version, secondary aneurysmal bone cyst. The Internet Journal of literature search, funds collection; M. Struz˙ycka – study Otorhinolaryngology 2012;14:1. [15] Toyosawa S, Yuki M, Kishino M, Ogawa Y, Ueda T, design, data collection, data interpretation, literature search, Murakami S, et al. Ossifying fibroma vs fibrous dysplasia of funds collection; M. Kozakiewicz – study design, literature the jaw: molecular and immunological characterization. search; M. Dzieniecka – data interpretation. Modern Pathology 2007;20:389–396.