Journal of Otology & RhinologyISSN: 2324-8785

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Review Article, J Otol Rhinol Vol: 4 Issue: 5

Calcifying Epithelial Odontogenic Tumor (Pindborg): Review of the Literature and update

Beatriz Peral Cagigal1*, Luis Miguel Redondo González1,Beatriz Madrigal Rubiales2 and Alberto Verrier Hernández1
1Department of Oral and Maxillofacial Surgery, Río Hortega Hospital, Valladolid,Spain
2Department of Histopathology, Río Hortega Hospital, Valladolid, Spain
Corresponding author : Dr. B. Peral Cagigal
Servicio Regional de Cirugía Oral y Maxilofacial, Hospital Universitario del Río Hortega, CaleDulzaina, 2, 47012 Valladolid, Spain
Tel: +34686581844; Fax: 983331566
E-mail: [email protected]
Received: August 11, 2015 Accepted: September 05, 2015 Published: September 10, 2015
Citation: Peral-Cagigal B, Redondo-González LM, Madrigal-Rubiales B, Verrier-Hernández A (2015) Calcifying Epithelial Odontogenic Tumor (Pindborg): Review of the Literature and update. J Otol Rhinol 4:5. doi:10.4172/2324-8785.1000248

Abstract

The calcifying epithelial odontogenic tumor (CEOT) or Pindborg tumor is a rare benign odontogenic neoplasm of the jaws that accounts for 1% of all odontogenic tumors. The typical clinical presentation is a slow-growing intraosseous mass that causes expansion of the affected mandible. Most often, it is associated with an impacted tooth, is asymptomatic but locally aggressive and requires biopsy for diagnosis. Since the first description in 1955, only 200 cases approximately have been reported. The aim of this article is to make a systematic review of the literature and an update of Pindborg tumor, emphasizing the importance to realize a suitable histologic diagnosis and a treatment with safety margins to minimize the likelihood of recurrence.

Keywords: Calcifying epithelial odontogenic tumor; Pindborg tumor; Odontogenic tumors

Keywords

Calcifying epithelial odontogenic tumor; Pindborg tumor; Odontogenic tumors

Introduction

The calcifying epithelial odontogenic tumor (CEOT) is a rare benign epithelial odontogenic neoplasm of the jaws, with approximately 200 reported cases in the world literature and also named Pindborg tumor after the pathologist who described it in 1955. This tumor accounts for approximately 1% of all odontogenic tumors [1]. It is a benign but occasionally invasive, slow growing neoplasm with recurrence rates of 10-14%. It usually involves the premolar-molar area of the mandible. Malignant transformation is unusual but some cases have been reported in the literature. The radiographic appearance is characterized by uni- or multi-locular lesion that often shows a mixed radiolucent-radiopaque pattern. The definitive treatment includes en bloc resection with tumor-free surgical margins and long-term follow-up is recommended to detect recurrences.
The aim of this article is to make a systematic review of the literature and an update of Pindborg tumor and report a new case of this uncommon neoplasm.

Materials and Methods

A search in PubMed was performed, using the key words “calcifying epithelial odontogenic tumor” and “Pindborg tumor”. The search in the database was carried out since the year 2000 until April 2014. Using key words, 79 publications were initially screened. The abstracts of these 79 publications were scanned for relevance, and 22 full-text articles were selected and read in detail. We present an additional case of Pindborg tumor and review the previously reported cases in the medical literature, emphasizing in the clinical, histological and radiological features and the main aspects of its differential diagnosis, prognosis and treatment.

Results and Discussion

CEOT is a rare and benign odontogenic neoplasm. Although benign and usually painless and slow growing, CEOT is locally aggressive with a tendency to invade adjacent bone, displace teeth and cause root resorption. One of their characteristics is the cortical expansion and the relationship with a non erupted tooth (especially mandibular molars) or odontoma in more than 50% of cases. It usually involves the premolar-molar area of the mandible (with a mandibular to maxillary ratio of 2 to 1), with a peak prevalence in the fourth and fifth decades and equal sex distribution [2].
The histogenesis of the CEOT remains controversial. Although it is established that CEOT arises from the epithelial components of the enamel organ, but whether the stratum intermedium, the dental lamina remnants or the reduced enamel epithelium are involved in the etiology still remains mysterious [3].
Two varieties have been described according to their location: intraosseous or central (94% of cases) and extraosseous or peripheral (6% of cases). The intraosseous tumors are most common in the molar and premolar region of the mandible. The extraosseous tumors are often located in the anterior part of the jaw and generally occur as single, painless, nonbleeding, gingival masses that commonly resemble oral hyperplastic or reactive lesions [2]. Around 20 cases of peripheral CEOT have been described in the literature, typically smaller than 2 cm and even one of them with multifocal involvement [1].
The most common clinical manifestation of CEOT, when is detected, is a solid and slow-growing intraosseous mass that causes expansion of the cortical plates, without pain or altered sensation. These tumors are highly infiltrative and capable of aggressive behavior. When located in the maxilla, patients may sometimes complain of nasal congestion, epistaxis, pain, proptosis and headache. Extension of the tumor into the maxillary sinus is a rarity with only 7 cases reported [4].
Most CEOT are solid in nature, histopathologically, and might have few cyst-like spaces within them. However, a true cystic CEOT is a rare possibility described in 3 cases up to now [5].
The radiographic appearance is characterized by uni- or multilocular radiolucent areas (“soap bubble” or “honeycomb”) with radiopaque masses of varying sizes inside the lesions (Figure 1A). According to a study by Kaplan et al., 58% are unilocular, 27% multilocular, and 15% nonloculated [6]. In many cases, especially in tumors of relatively short duration, the calcified areas are tiny and may be undetectable in radiographs. Later, as the lesion ages, calcium salts are deposited and it becomes increasingly radio-opaque. Classically “driven snow” appearance has been described. When there is an unerupted tooth, the radiopacities tend to be located close to the tooth crown [7].
Figure 1: (A) Panoramic radiograph of a case treated in our department, with multilocular radiolucent lesion from the first premolar to subcondylar region of the left side; (B) The CT scan shows an osteolytic intraosseous lession of left hemimandible, causing expansion and focal erosion of both cortical plates.
CT scan shows a well defined mass of soft tissue attenuation, that contains thick bone trabeculae and scattered high density foci. CT scan images (Figure 1B) and 3D reconstructions may be helpful in delineating the extent of tumor, the involvement of cortical plates, basal bone, and extraosseous extension, wich is essential for surgical planning [8].
Magnetic resonance imaging (MRI) reveals a lesion that is predominantly hyperintense on T2-weighted and hypointense on T1- weighted images. There are areas of signal void within the lesion that correspond to the bony septa. These features distinguish the lesions from more aggressive neoplasms [9].
Ameloblastoma, dentigerous cyst, calcifying odontogenic cyst, ossifying fibroma, adenomatoid odontogenic tumor and malignant CEOT should be considered in the differential diagnosis of Pindborg tumor [10]. Definitive diagnosis is based on histologic examination.
CEOT is distinguished from other odontogenic tumors because of its unique histopathologic features. Histologically, CEOT is unencapsulated and composed of polyhedral odontogenic epithelial cell cords, interspersed with extracellular amyloid (pale eosinophilic masses) and concentric calcifications known as Liesegang rings (Figure 2), which are pathognomonic of this tumor [11]. The origin of amyloid is unclear. The epithelial cells have abundant eosinophilic cytoplasm, sharply defined cell borders, and well-developed intercellular bridges. Mitotic figures are rarely encountered. The eosinophilic material has an apple-green birefringence under polarized light after staining with Congo red. Calcification is characteristic, but noncalcifying variants also occur.
Figure 2: The histology section shows concentric calcifications or Liesegang rings (H&E stain, 40×).
In addition to the classic form, 3 additional histologic variants of CEOT have been described that may have a bearing on their prognosis and treatment. Non-calcifying CEOT with Langerhans cells and CEOT with cementum-like and bone-like material are known to be less aggressive and are amenable to more conservative surgical treatment. The third variant, clear cell CEOT is more aggressive and has a higher rate of recurrence than conventional CEOT [12] and some authors would consider this form to be a low-grade odontogenic carcinoma [13]. To date, have been published around 13 cases of clear cell variant [14] and 7 cases of Langerhans cell variant which has only been described in Asian population (mainly in the anterior portion of the maxilla) [15].
CEOT is managed surgically. The tumor is not encapsulated, and most authors agree that successful treatment requires surgical excision of the lesion with a safety margin of 1 cm to avoid recurrence (9 y 10). Peripheral tumors may be treated with a smaller margin of 0.5 cm, because of its lesser aggressiveness. Methods of treatment vary from simple enucleation or curettage to radical and extensive resections. Surgical decision making often depends on case parameters, such us the anatomic location of the tumor, the size and duration, histopathologic findings, patient age, health status, and consideration of reconstruction methods following surgery (Figure 3). Thus, maxillary tumors would be treated more aggressively because of their close proximity to vital and important structures, whereas mandibular lesions could be approached more conservatively [13].
Figure 3: (A) The specimen shows the resected portion of the mandible with safety margins; (B) Immediate reconstruction of the mandible defect in patient with fibula free flap and reconstruction plate.
CEOT have a well-recognized propensy to recur (10-14%) after surgical excision and exhibit an even greater recurrence rate if they are treated by curettage or if resection is incomplete. Moreover, a relatively higher recurrence rate of 22% has been reported for the clear cell variant [14]. Such cases need to have a long-term follow-up to rule out recurrence (minimum of 5 years).
Malignant transformation of a CEOT is extremely rare but possible, with only 8 cases reported in the English literature. Malignant transformation occurred in the primary tumor in 5 cases and in recurrent tumors in the remaining 3 cases [12].

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