Gorlin cyst associated with odontoma: Case report with surgical treatment
Quiste de Gorlin asociado a odontoma: reporte de un caso con su tratamiento quirúrgico

P. Villarroel Castro¹, R. Fariña Sirandoni², I. Espinoza Santander³

Recibido: 13.07.05

Aceptado: 15.11.05

1 Residente. Servicio de Cirugía Maxilofacial. Hospital Del Salvador.
2 Servicio de Cirugía Maxilofacial. Hospital Del Salvador; Unidad de Cirugía Maxilofacial Hospital Exequiel González Cortés; 
Profesor del Departamento de Cirugía Oral y Maxilofacial, Facultad de Odontología Universidad Mayor.
3 Profesor de Patología, Facultad de Odontología Universidad de Chile, Santiago de Chile, Chile.

Correspondencia:
Dr Rodrigo Fariña Sirandoni
Avda. Providencia 2330 of. 23, Providencia
Santiago, Chile
Email: rofari@gmail.com; paula78cl@yahoo.com

Introduction

Gorlin cyst is an unusual lesion that arises as a result of epithelial remains associated with odontogenesis. It generally presents as a cystic lesion that is well-defined and surrounded by epithelium. It has less invasive characteristics and for this reason it is considered a benign lesion. A non-cystic variety has also been found with behavior that is locally aggressive. It infiltrates tissues and in this case it is considered a neoplasm. ¹ This paper covers a case of Gorlin cyst located in the left infraorbital region, its surgical treatment, the results obtained, and the differential diagnoses that are possible are discussed.

Case Report

A young 18 year-old female presented with an increase in facial volume in the left infraorbital region that had been growing slowly for six months. Her medical history was insignificant, she was not taking any medication and she had no known allergies. The facial examination showed an increase in facial volume on the right side that had lead the disappearance of the naso-labial fold (Fig. 1), displacement of the left nasal wing and deviation of the columella and nasal tip towards the right side (Fig. 2).

The skin covering the lesion was of a normal appearance, and on consultation it measured 6 cm in diameter approximately. On palpation it was painless and it was firm in consistency.

The intraoral examination revealed an increase in volume at the back of the cavity in relation to the canine fossa. The area was covered with mucosa of normal characteristics and there were no signs of active infection (Fig. 3). The teeth by the lesion were vital and there was no mobility.

The imaging study by panoramic radiography showed a radiolucid, unilocular lesion that measured 4 x 5 cm, with well-defined borders. It extended from the mesial tooth 1.1 to the distal tooth 2.4, producing rhizolysis in the teeth. A small, isolated, radiopaque area could be seen in the lesion (Fig. 4). The computed axial tomography (CT) with coronal and axial views (Fig. 5) showed in the left anterior region of the maxilla, a rounded lesion that was well-defined, corticalized and with a seemingly liquid content.

A transoral approach was used for the surgical treatment by means of a crevicular flap with two vertical incisions. Once the area was exposed, bone tissue covering the lesion could be observed that was papery and crepitant. It had a cystic appearance and a soft consistency, and it was covered by a fibrous capsule.

The lesion was completely removed along the cleavage plane (Fig. 6). One layer was closed (Fig. 7), and iodoform gauze was left and gradually removed over the following two weeks (Fig. 8).

The macroscopic examination of the surgical specimen revealed a cystic lesion surrounded by a fibrous capsule containing a small calcification. There was a watery, coffeecolored substance inside it (Fig. 9).

The histopathologic examination revealed a well-defined cystic lesion that was surrounded by a fibrous capsule and epithelium that was made up of cuboidal basal cells, cells with a stellate reticulum-like appearance and others with the appearance of ghost cells. The calcified fragment that was attached to the cyst wall seemed, when examined microscopically, to be an odontoma with a central area of conjunctive tissue similar to dental pulp, dentine and immature enamel. It was fused with other mineralized tissue that had the appearance of basophilic granules and dentin tissue (Fig. 10). According to the histopathologic study the lesion corresponded to a Gorlin cyst associated with odontoma.

Two weeks after the surgical excision, the mucosa was completely closed and facial symmetry had been reestablished (Fig. 8). During the three year follow-up there was no relapse (Fig. 11).

Discussion

According to Curran, an odontogenic cyst or tumor can be suspected in a follicular space that has expanded to 5 mm.^2,3 In a retrospective study of 2.645 cases of embedded teeth other types of pathologic lesions were found in 32.9% of cases.² Based on the available findings, a differential diagnosis can be made between the following pathologic entities:

• Dentigerous cyst (DC). This is the most common odontogenic cyst after the radicular cyst, accounting for 20% of the epithelial cysts of the maxilla. It is a benign lesion associated with the permanent dental crown⁴ or with non-erupted supernumeraries. It can be associated with odontomas and on rare occasions with temporary teeth.^1,4,5 The smaller ones are asymptomatic but the larger ones can produce facial asymmetries. They appear most commonly by the third molars of the mandible and secondly by the canine region of the maxilla. ^1,2,5 They generally appear in the second decade of life^5,6 and they have a predilection for the male sex (1,6:1).^1,5,6 The radiographic image shows a radiolucid unilocular image of a varied size, with well-defined borders and corticalized, associated with the crown of a non-erupted tooth.^4,5,7

• Adenomatoid odontogenic tumor, follicular variety (AOT). The follicular AOT is an intraosseous lesion associated with a non-erupted tooth, accounting for 75% of all AOTs. It affects young individuals, around the 2nd decade in life, with a predilection for females (2:1).^1,8,9 It mainly affects the anterior area and it is commonly to be found in the maxilla.^1,9 It is asymptomatic and it generally does not measure more than 3 cm in diameter. It can be distinguished from the GC in that in addition to covering the coronal portion, it covers the radicular portion of the embedded tooth.^1,9

• Calcifying odontogenic cyst (Gorlin cyst) (GC). The calcifying epithelial odontogenic cyst or Gorlin cyst (GC) is a lesion that arises from the remains of odontogenic epithelium, and it is found exclusively in the maxilla. It was first described as a pathologic entity in 1962 by Gorlin el al.¹³

It is a very unusual lesion accounting for 1% of all maxillary cysts^6,10 and its histology and clinical manifestations are varied.¹ The world health organization (WHO) has described it as a non-neoplastic cyst but classifies it as a benign tumor.¹⁴ Other authors consider it as two entities, one cystic and the other neoplastic.^15,17

The cystic variation is surrounded by epithelium and 80 to 90% of cases appear as such. However, between 2 and 16% present as the noncystic variety, infiltrating tissues, being locally aggressive and with malignant characteristics. For these reasons it is considered a neoplasm.¹ In this clinical case we observed the cystic variety in the form of an intraosseous lesion which, according to the literature, is most common type. Between 12 and 21% of cases present as an extraosseous lesion (peripheral), ^1,6,15,18,19 which manifest as a sessile or pedunculated gingival mass, with no clinical characteristics that distinguish it from a gingival fibroma, peripheral cyst or granuloma of giant cells.¹ Both are as common in the mandible as they are in the maxilla.

In this patient it was to be found in the incisor-canine area of the maxilla with this area of the maxilla being the most affected with a rate of 65% according to reports in the literature.^1,17,18

The age of the patient coincides with the prevalence reported by other authors, with the most prevalent age being between the 2^nd and 3^rd decade, and a mean age of 33 years.^1,17-19

It has been associated with ameloblastoma, ameloblastic fibroma, and complex odontoma; the latter is to be found in younger patients in particular, such as in this case, representing 20 to 50% of the cases reported in the literature.^1,11,18-21 The neoplastic variety is generally to be found in elderly patients although it is less common.¹

The radiography does not show a typical image, as the image may be uni- or multilocular. The presence of calcifications of various sizes can be observed.

With regard to the therapeutic alternatives, all these are aimed at eliminating the etiological factor, that is, the epithelial remains and the cystic process. In larger sized lesions decompressive treatment can be carried out so that complete exeresis of the cyst can later be carried out.²⁴

Of special interest are the possibilities of repairing the bone defect. First intention healing in a cavity of the size in this case increases the risk of clot infection. For this reason second intention healing was carried out by means of an iodoform gauze that was removed progressively, allowing the cavity to be always filled.

We believe that when located in maxillary bone tissue, primary bone grafts are not necessary for lesions of this size, in young patients as the considerable repair potential and the undamaged periosteum will allow proper bone regeneration.

The histology of the GC generally consists is a cystic lesion covered by a fibrous capsule and lined on the inside by a basal layer of cylindrical cells and then by various layers of cells that are similar to the stellate reticulum-type. The underlying epithelium is able to induce the formation of mature dental tissue in the wall of the connective tissue.^16,18 The presence of masses with ghost cells situated in the epithelium or in the capsule that surrounds it, is a characteristic of this lesion.

Conclusions

Gorlin Cyst is an uncommon lesion in the cystic pathology of the maxilla. The treatment of choice is always surgical, and it consists in the total elimination of the lesion. If the surgical technique is correct, the success rates are high and relapse rates are minimal.

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