Case data

Head and Neck

Oncologia

CEMENTO-OSSIFYING FIBROMA

148
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Tipo Caso 1
  • André Mannato - IRMANDADE SANTA CASA DE MISERICÓRDIA DE SÃO PAULO
  • Diego Cardoso Fragoso - IRMANDADE SANTA CASA DE MISERICÓRDIA DE SÃO PAULO
  • Henrique Bortot Zuppani - IRMANDADE SANTA CASA DE MISERICÓRDIA DE SÃO PAULO
HENRIQUE AUGUSTO LINO - IRMANDADE SANTA CASA DE MISERICÓRDIA DE SÃO PAULO
Email: henriquelino@live.com
5/15/2020
6/19/2020
Male, 12 anos
Cementoma, Tumores Odontogênicos, Fibroma, Fibroma Ossificante

Abstract

A 12-year-old male presented proptosis of the right eye, nasal obstruction, and headaches for a month. The computed tomography (CT) showed an expanding and insufflating lesion with heterogeneous attenuation coefficients, mostly hyperattenuating, located at the right nasal cavity. The histopathological examination concluded the tumor as a cemento-ossifying fibroma. Afterward, the lesion was enucleated.

Clinical History

A 12-year-old male presented proptosis of the right eye, followed by nasal obstruction and headache for about a month. The rhinoscopy showed a bony-consistency lesion posteriorly to the right middle turbinate. It was bulging the local structures and decreasing the caliber of the air column. The patient underwent enucleation.

Radiological findings

The CT scout reveals an expanding and insufflating heterogeneous lesion with circumscribed and hyperdense borders projecting over the orbital and nasal topography (Figure 1). The CT showed a predominant hyperdense oval-shaped expanding bone lesion with some insufflating characteristics and circumscribed borders, measuring about 5,3 x 4,1 x 3,6 cm. It was centralized at the right lamina papyracea with an extension to the homolateral sphenoid sinus (Figures 2 and 3). The lesion determined bone remodeling and expansive effect over the adjacent structures, which was mainly characterized by the extrinsic compression of the orbital components (Figures 4 and 5). The most compromised eye structures were the ones near the orbital apex of the right side with lateral displacement of the extraconal fat, inferior and medial rectus muscles of the eye and the optical nerve.

Discussion

The cemento-ossifying fibroma (COF) is a benign mesenchymal odontogenic tumor, according to the 2017 World Health Organisation’s classification [1]. The COF is rare neoplasia, often occurring on female patients between the third and fourth decade of life. The most commonplace of involvement is the mandible in about 70% of the cases [2]. The COF’s etiology is unknown, but it has been supposed that local trauma may trigger its development. The lesion usually arises from the adjacent dental elements of the mandible and the maxilla. The lesion is composed of pluripotent cells from the mesenchymal tissue of the periodontal ligament with the capability to differentiate into fibrous tissue, cemento, bones, and mixed-composition tissues [3,4]. The radiological aspect of the COF is represented by a mixed radiolucent and radiopaque lesion, with multiple compartments, a definite capsule, circumscribed borders, and sclerotic margins. As the tumor maturates, it becomes radiopaque [5]. The lesion has two types: adult and juvenile [1]. The first one affects women between the third and fourth decade of life, usually appearing as an incidental finding on an asymptomatic patient [6]; the latter ones manifest on young male patients, generally younger than 15 years old, with more aggressive and expansive behavior. In some cases, it may also behave as a destructive lesion, and it may compress adjacent structures. The juvenile type has the highest recurrence rate (roughly 30-58%) [2], as the presented case. COF’s management depends upon some factors, like location and dimensions. The small tumors might be enucleated or curetted. The larger and aggressive lesions, on the other hand, must be resected with wide resection margins. Eventually, this latter group of patients may also benefit from chemotherapy with alfa-interferon or subcutaneous calcitonin. Radiotherapy is avoided because it increases the malignancy rates of the lesion. Regardless of the treatment, the COF should be monitored in order to detect recurrences [2].

List of Advantages

  • Fibrous dysplasia
  • Ossifying fibroma
  • Aneurysmal bone cyst
  • Calcifying odontogenic cysts (Gorlin’s) and other odontogenic cysts
  • Keratocystic odontogenic tumour
  • Calcifying epithelial odontogenic tumour

Diagnosis

  • Cemento-Ossifying Fibroma

Learning

We report a case of a patient with a juvenile type of cemento-ossifying fibroma with fast progression and expansive behavior determining symptoms. The juvenile COF (the aggressive variant) has the highest rates of recurrence. Thus, its recognition is relevant in order to plan the proper management.

References

  • [1] Bala TK, Soni S, Dayal P, Ghosh I. Cemento-ossifying fibroma of the mandible: A clinicopathological report. Saudi Med J. 2017;38(5):541–5.
  • [2] Aburas S, Bandura P, Al- Ibraheem A, Berger S, Meier M, Turhani D. A large maxillary cemento-ossifying fibroma superimposed with solitary bone cyst documented over 18 years: A case report. Int J Surg Case Rep [Internet]. 2020;68:257–62. Available from: https://doi.org/10.1016/j.ijscr.2020.03.011
  • [3] Singhal A, Singhal P, Ram R. Cemento-ossifying fibroma. Contemp Clin Dent. 2012;3(1):83.
  • [4] Chang HJ, Donahue JE, Sciandra KT, Evangelista PT. Best cases from the AFIP juvenile ossifying fibroma of the calvaria. Radiographics. 2009;29(4):1195–9.
  • [5] Dunfee BL, Sakai O, Pistey R, Gohel A. Radiologic and Pathologic Characteristics of Benign and Malignant Lesions of the Mandible. RadioGraphics [Internet]. 2006 Nov 1;26(6):1751–68. Available from: https://doi.org/10.1148/rg.266055189
  • [6] Toledano-Serrabona J, Núñez-Urrutia S, Vegas-Bustamante E, Sánchez-Torres A, Gay-Escoda C. Florid cemento-osseous dysplasia: Report of 2 cases. J Clin Exp Dent. 2018;10(11):e1145–8.

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