# A calcified epithelial odontogenic tumor with extension to the maxillary, ethmoid and sphenoid sinuses

Ambroise Jagdev, Olivier Malard, Charles Lépine, Philippe Lesclous

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Ambroise Jagdev, Olivier Malard, Charles Lépine, Philippe Lesclous. A calcified epithelial odontogenic tumor with extension to the maxillary, ethmoid and sphenoid sinuses. Journal of Stomatology, Oral and Maxillofacial Surgery, 2024, 126 (1), pp.102020. 10.1016/j.jormas.2024.102020. hal-04991505

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Case Report

# A calcified epithelial odontogenic tumor with extension to the maxillary, ethmoid and sphenoid sinuses

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CrossMark

Ambroise Jagdevª, Olivier Malardᵇ, Charles Lépineᶜ, Philippe Lesclousª,\*

ARTICLE INFO

Article History:

Received 24 June 2024

Accepted 20 August 2024

Available online 23 August 2024

Keywords:

Calcified epithelial odontogenic tumor

Diagnosis

Intraosseous

Odontogenic tumor

Pindborg tumor

A B S T R A C T

Calcified epithelial odontogenic tumor (CEOT) with extension to the maxillary, ethmoid and sphenoid sinuses is currently unreported. This extension poses a challenge for effective treatment, as CEOT is a benign neoplasm with contrversial potential aggressiveness. We report a case of CEOT that was previously misdiagnosed as an inverted papilloma. A segmental resection of the part of the tumor responsible for the symptoms was performed. Histology revealed polyhedral epithelial cells within an amorphous eosinophilic hyaline material containing round calcified structures and confirmed the diagnosis of CEOT. Postoperative CT Scan showed the partial resection of the tumoral process. A mixed radiolucent/radiopaque aspect of the remaining CEOT was clearly discernible. The patient was warned that due to the incomplete excision of the tumoral process, the risk of recurrence was significant and that a close clinical and radiological follow-up every 6 months was mandatory for a long time.

© 2024 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY license

(http://creativecommons.org/licenses/by/4.0/)

1. Introduction

The calcifying epithelial odontogenic tumor (CEOT) is an epithelial odontogenic neoplasm first described by Thoma and Goldman [1], and further detailed by Pindborg in 1958 [2]. CEOT is defined as a benign epithelial odontogenic tumor that secretes an amyloid protein with a tendency to calcify [3]. Although CEOT is predominantly an intraosseous process (94 %), it also occurs as a rare peripheral extraosseous tumor [4]. It is most commonly found in the mandible of adults (maxilla/mandible ratio 1:2), in the premolar and molar regions, and in the fourth decade of life although it has been reported in a wide age range [5]. The relative frequency of CEOT extracted from several oral biopsy surveys ranged from 0.4 % to 3 % of all odontogenic tumors [5].

The clinical presentation is usually a painless swelling with slow growth [5]. A few cases of locally aggressive CEOTs invading adjacent structures and rare cases of malignant transformation of CEOT have been reported in the literature [6,7]. Radiographically, intraosseous CEOT present as unilocular or in 30 % of the lesions as multilocular radiolucencies with varying degrees of radiopacity [5]. These lesions often show cortical bone resorption and tooth displacement [6]. In approximatively 60 % of cases, the tumor is associated with an

unerupted tooth [8]. Histopathologically, CEOT is an unusual and variable combination of odontogenic epithelium and calcified structures [8].

Usually, the recommended treatment is conservative resection with a narrow margin of adjacent healthy bone. This treatment approach is associated with a reported recurrence rate of 10 to 15 % [6,9].

Maxillary cases of CEOT are scares in the literature and, to our knowledge, this is the first report of a very extensive maxillary CEOT involving the maxillary sinus with nasal, ethmoidal and sphenoidal extensions.

Case report

A 68-year-old patient with no significant past medical history presented with painless left nasal obstruction for the past 6 months associated with a single episode of epistaxis. He reported a history of an unerupted tooth extraction associated with a cyst in the left maxillary region >30 years ago. No histologic report was available but the patient reported no sequelae from the surgical procedure. A few weeks before consultation, he underwent a biopsy of a left sino-nasal tumor process which reported an inverted papilloma.

On physical and flexible endoscopic examination, he had a tumor mass completely obstructing the nasal cavity. There was no evidence of peripheral tissue invasion and no cervical lymph node involvement.

<footnote>ª Department of Oral Surgery, Nantes University Hospital, 1 place Alexis Ricordeau, 44000 Nantes, France</footnote>

<footnote>ᵇDepartment of Otorhinolaryngology, Head and Neck Surgery, Nantes University Hospital, 1 place Alexis Ricordeau, 44000 Nantes, France</footnote>

<footnote>Department of Pathology, Nantes University Hospital, 1 place Alexis Ricordeau, 44000 Nantes, France</footnote>

<footnote>* Corresponding author at: CHU de Nantes, 1 place Alexis Ricordeau, 44000 Nantes, France.</footnote>

<footnote>E-mail address: philippe.lesclous@univ-nantes.fr (P. Lesclous).</footnote>
<figure><img src="image_5.png" /><figcaption>**Fig. 1.** Small swelling of the left palate covered by a normal mucosa.</figcaption></figure>

Intraoral examination revealed a small swelling of the left palate. It appeared as a painless firm gingival mass covered by a normal mucosa (Fig. 1).

Because the suspicion of inverting papilloma, a magnetic resonance imaging was performed. It showed a large tumor mass invading the left maxillary sinus and extending into the ethmoid and

sphenoid sinuses, and the homolateral nasal cavity (Fig. 2). The tumor process was well circumscribed with no evidence of bone erosion.

A conservative surgical approach was proposed to the patient to avoid a mutilating procedure including a complete hemimaxillectomy. Only a segmental resection of the part of the tumor responsible for the symptoms was performed. This approach began by the resection of the medial concha immediately followed by the excision of part of the tumor invading the nasal cavity. Then a septoplasty and an unciformectomy were performed to access to the medial meatus allowing partial ethmoidectomy from the cranial base to the frontal sinus and medial hemimaxillectomy. Then, partial sphenoidectomy was performed. This surgical management ended by revision of the sphenoethmoidal and ethmoidomaxillary cavities.

Histology revealed well-defined sheets of polyhedral epithelial cells within an abundant amorphous eosinophilic hyaline material. Within this material, round calcified structures, known as Lieserang rings, were well visualized (Fig. 3). The epithelial cells had eosinophilic or clear cytoplasm and nuclear pleomorphism but no mitotic fates were noted. There was no evidence of malignancy. The entire excised tissue, from the sphenoid sinus to the ethmoid and maxillary sinuses and the maxillary bone were involved by the tumor process. The margins of excision were infiltrated by the tumor process. All these features supported the diagnosis of CEOT.

Immediate postoperative follow-up was uneventful. Postoperative CT Scan showed the partial resection of the tumoral process. In the antero-lateral part of the left maxilla, a mixed radiolucent/

<figure><img src="image_6.png" /><figcaption>**Fig. 2.** Magnetic Resonance Imaging: coronal and axial views showing an important tumor mass invading the left maxillary sinus and extending in the ethmoid sinus and in the nasal cavity until the skull base (a, b), and coronal view showing the extension of the tumor process in the sphenoid sinus (red arrow) (b).</figcaption></figure>

<figure><img src="image_7.png" /><figcaption>**Fig. 3.** Histopathological findings: H&E staining, x 40 (a) and x 200 (b). A: Amorphous eosinophilic hyaline material, C: Calcified structures, E: Polyhedral epithelial cells.</figcaption></figure>
<figure><img src="image_8.png" /><figcaption>**Fig. 4.** Immediate postoperative tomodensitometry showing the partial resection of the tumor process (a), and its remaining part in the antero-lateral part of the left maxilla.</figcaption></figure>

radiopaque aspect of the remaining lesion was clearly visible (Fig 4). The patient was warned that due to the incomplete excision of the tumor process, the risk of recurrence was significant and that a close clinical and radiological follow-up every 6 months for a long time was mandatory.

Discussion

CEOT with extension into the maxillary sinus and/or into the nasal cavity is extremely rare [9,10], but to our knowledge, this is the first report of a CEOT with extension into the maxillary sinus combined with ethmoidal and sphenoidal invasion.

CEOT displays a variety of architectural patterns and three variants have been described in the literature, the clear cell CEOT, the Langerhans cell CEOT and the microcyst CEOT, which were first reported in 1967 by Abrams and Howel [11], in 1990 by Asano et al. [12], and in 2017 by Sanchez-Romero et al. [13], respectively. Obviously, the present case is representative of the first type.

When located to the maxilla, patients may sometimes complain of nasal congestion, epistaxis and headache as in the present case.

The most common clinical presentation of intraosseous CEOTs is a cystic lesion associated with an unerupted tooth including but not limited to dentigerous/follicular cyst, periapical/inflammatory cyst, and odontogenic keratocyst [6]. Also suspected are giant cell granuloma, ameloblastoma, cemento-osseous lesion, dental follicle and odontoma [6]. In the present case, it cannot be excluded that the cystic aspect associated with an unerupted tooth was the initial aspect of this CEOT >30 years ago. More puzzling is the report of an inverted papilloma from a biopsy performed few weeks before consultation. In general, CEOT is easily diagnosed by histology alone, particularly the clear cell type as in the present case.

The most common radiographic presentation is a unilocular mixed radiolucent/radiopaque lesion. A multilocular presentation with such an aspect is much rarer [6], and deep extension into the maxillary, ethmoidal and sphenoidal sinuses is currently unreported.

Segmental resection is a treatment modality associated with a recurrence rate of approximately 7.5 % [6]. However, given the lack of standardization of treatment and follow-up, there is no consensus regarding the true recurrence rate of CEOT [6]. As the segmental resection was partial and as some extremely rare cases of malignant

CEOT have been described [6,12], a close and regular follow-up including radiologic control every 6 months was suggested to the patient.

Regarding the pathogenesis, it was initially thought that CEOT arises from the reduced enamel epithelium of an associated unerupted tooth. However, due to the existence of cases not associated with an unerupted tooth and because of a peripheral variant of CEOT, other etiopathogenesis has been considered. The current opinion is that CEOT arises from the disintegration of the complex system of dental lamina giving rise to countless numbers of epithelial remnants that persist in the jawbone and gingiva after the completion of odontogenesis [3,5,6,8,14]. Mutations in some tumor suppressor genes and some oncogenes have been investigated without reliable evidence today [15].

This indicates that further research is needed to elucidate the pathogenesis of CEOT.

Declaration of competing interest

The authors declare that they have no competing interest.

CRediT authorship contribution statement

**Ambroise Jagdev:** Writing – original draft, Investigation, Funding acquisition. **Olivier Malard:** Visualization, Investigation, Data curation. **Charles Lépine:** Resources, Formal analysis. **Philippe Lesclous:** Writing – review & editing, Visualization, Supervision, Investigation, Formal analysis, Conceptualization.

References

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