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Odontogenic Keratocyst: An Unusual Clinical Presentation
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Abstract: The odontogenic keratocyst (OKC) is a developmental cyst of the jaws of epithelial origin.
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This lesion is commonly found in the ascending ramus and posterior mandible, and can become quite
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large due to its rapid growth and its extension into the adjacent structures. Herein, we are reporting a
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case of odontogenic keratocyst involving the anterior mandible which was mimicking other cysts and
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tumors which occur in that region more frequently. On closer view into the case, the lesion was
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proved to be an OKC. The lesion was successfully treated by complete enucleation. Because of the
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uniqueness of this case, the clinical, radiological, and histological features of this cyst/tumor are
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discussed in this article.
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Keywords: Odontogenic Keratocyst, Odontogenic Cysts, Keratocystic Odontogenic Tumor,
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Mandible, Bimodal, Carnoy’s solution
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Introduction: Ever since Philipsen described the odontogenic keratocyst (OKC) in 1956, the lesion
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has turned into a center of momentous clinical interest because of its unusual growth pattern and
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tendency to recur even after surgical removal (Sulabha AN et al., 2013). This lesion was recently
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renamed by Philipsen as keratocystic odontogenic tumour (KCOT) and reclassified as an odontogenic
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neoplasm in the World Health Organization’s, 2005 edition of its histological classification of
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odontogenic tumours. According to this edition, the KCOT has been defined as a benign uni or
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multicystic intraosseous tumour of odontogenic origin, with a characteristic lining of parakeratinized
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stratified squamous epithelium and potentially aggressive, infiltrative behavior (MacDonald-
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Jankowski DS, 2011; Binali Çakur et al., 2008). Odontogenic Keratocyst (OKCs) of the jaw is also
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recognized as a developmental cyst and there is general agreement that the odontogenic keratocyst
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arises from the cell rests of the dental lamina. This cyst shows a different growth mechanism and
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biologic behaviour unlike the other more common jaw cysts and tumors (Brad W Neville et al., 2002;
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Tejasvi ML Avinash et al., 2010). Odontogenic keratocysts may be found in a wide range of patients
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who range in age from infancy to old age. KCOTs are twice more frequent in the mandible than in the
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maxilla (Guilherme Romano Scartezini et al., 2012) and mandible is occupied in 60% to 80% of
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cases. Radiographically, OKCs appear as well-defined radiolucencies, which can be either unilocular
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or multilocular and they may appear as small or large, round or ovoid radiolucent lesions, often with
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scalloped, multilocular, distinct sclerotic margins. This type of cyst may occur in conjunction with an
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impacted tooth as has been reported in 25 to 40% of cases; in such instances, the radiographic features
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suggesting more towards a diagnosis of dentigerous cyst while smaller cysts makes the diagnosis
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more dilemmatic (Brad W Neville et al., 2002; Guilherme Romano Scartezini et al., 2012; Santhosh
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Kumar S Hiremath et al., 2011).
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Case Report: A 23 year old male patient reported the Department of Oral Medicine and Radiology
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with a chief complaint of swelling in lower front tooth region and face since last 1 year when he
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suddenly noticed a small swelling in the lower right front tooth region inside the mouth and he took
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some medication. After taking those medications, the swelling completely subsided. After 6 months,
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similar swelling appeared which was progressively increasing in size and was not subsided by taking
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medication. The swelling continued increasing in size and affected the other teeth on opposite side
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also. After 10 days, he noticed swelling on face in mirror. He immediately went to local dentist who
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advised for a biopsy. Clinical examination revealed a single, ill defined swelling present in relation to
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the lower 1/3 rd of the face near symphysis and parasymphysis regions which crossed midline and
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was measuring approximately 5 X 3 cm in diameter extends mesiodistally from right parasymphyseal
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region to left parasymphyseal region and superioinferiorly from the vermilion border of lip of right
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side and corner of mouth of left side till 1 cm below the lower border of mandible. Swelling appeared
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to be with smooth surface with shiny, slightly stretched skin and on palpation, swelling appeared to be
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bony hard in consistency with well-defined margins and with a smooth lobular surface. (Fig.1)
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Swelling was slightly tender on right side with no local rise in temperature. Superficial skin was
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pinchable with no secondary changes. (Fig.2) Intra-orally, mandibular vestibule was obliterated by
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swelling in relation to 35 to 43 region. On detailed intraoral examination, a single well defined
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swelling was present in anterior part of mandible extending from 35 to 43 region with buccolingual
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expansion. Buccally, swelling extended mesiodistally from mesial aspect of 43 to the distal aspect of
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35 crossing midline and buccolingually from the alveolar crest to the buccal and labial vestibule
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obliterating the vestibular region. Superficial alveolar mucosa appeared to be stretched with
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detachment of the marginal gingiva in relation to 33, 34, 35. Swelling was slightly tender on right
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side. Swelling was bony hard in consistency with egg shell crackling which was present distal to 33.
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Bucco-lingual expansion was seen evident throughout the swelling. (Fig.3, 4) On pulp vitality test, 32
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was found to be completely non-responding while 35 showed delayed response. During aspiration, a
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dark blackish-red betadine coloured aspirate was taken out. (Fig.5) IAOPAR irt 31,32,33,41,42,43
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were advised. IOPAR revealed multilocular well defined radiolucencies overlapping each other at the
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apical regionof 31,32,33,41,42. Margin of the radiolucencies were sclerotic. (Fig.6, 7)
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Orthopantomogram showed well defined multilocular radiolucency extending from the periapical
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region of 35 till the peripical region of 43,44. Sclerotic margin with displacement of teeth could be
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appreciated. The lower border of the radiolucency overlapped and crossed the inferior border of
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mandible. Apical 1/3 of roots in relation to 33,35,43,45 appeared knife edged suggestive of root
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resorption. (Fig.8) Mandibular topographic occlusal radiograph was performed to check the
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buccolingual expansion. In this radiograph, multilocular radiolucency was evidently seen in the apex
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of 33,32,32,41,42 region. A thin sclerotic line was also appreciated on the buccal aspect of
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32,33,34,35 suggestive of buccolingual expansion. Inferior border of mandible appeared intact.
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(Fig.9) On the basis of clinical examination and chair side investigations, a provisional diagnosis was
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made for central giant cell granuloma with differential diagnosis of Glandular odontogenic cyst,
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Aneurysmal bone cyst, Ameloblastoma, Odontogenic keratocyst, and an Arteriovenous malformation
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in relation to 35 to 43 region. The lesion was examined at biopsy. Histopathologically, H & E staining
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showed epithelia overlying the connective tissue stroma. The epithelium was parakeratinized,
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stratified squamous, 8-10 layer thick. Basal cells were tall columnar in appearance with
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hyperchromatic nuclei, exhibiting reverse polarity. Loss of cellular adhesion and subepithelial split
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could also be seen. Connective tissue stroma showed juxtaepithelial hyalinization and was sparsely
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cellular with loose collagen fibres, acute inflammatory cell infiltrate and areas of haemorrhage.
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(Fig.10) Surgical enucleation was performed under all aseptic precautions and was started antibiotic
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prophylaxis. Complete enucleation with aggressive curretage was performed. Patient was kept under
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repeated follow-up for three months. Patient was re-called after 3 months and after 9 months. (Fig.11)
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Discussion: An insight into the times past of odontogenic cysts goes back to the 19th century, when
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the odontogenic keratocyst (OKC) was first described in the year 1876 and was named by Phillipsen
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in 1956 (Brad W Neville et al., 2002) who described it as a different entity characterized by a
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keratinized lining, presence of satellite cysts and association with the nevoid basal cell carcinoma
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syndrome (A Nagraja et al., 2012). Philipsen and Riechert have suggested that OKC should be
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considered as a benign tumour and hence be called as KCOT or keratocystic odontogenic tumour.
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Shear has countered this argument by saying that even if it is a neoplasm, it is suitable to be called as
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OKC as many neoplasms do not essentially have a suffix ‘oma’. This debate was started by Shear
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(2003) that OKC should be called as keratocystoma which led Philipsen and Riechert suggesting
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keratinising cystic odontogenic tumour in 2004 and then Philipsen suggesting keratocystic
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odontogenic tumor again in 2005 (Mahadesh Jyothi et al., 2010). This cyst has a propensity for
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recurrence and the aggressive behaviour clinically and histologically has necessitated the
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reclassification of the lesion by the World Health Organization (WHO, 2005) as a ‘keratocystic
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odontogenic tumor’ (KCOT). The KCOT is defined as ‘a benign uni- or multicystic, intra-osseous
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tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous
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epithelium and potential for aggressive, infiltrative behaviour (MacDonald-Jankowski DS, 2011;
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Binali Çakur et al., 2008; Rajkumar GC et al., 2011). Odontogenic Keratocyst (OKCs) of the jaw is a
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type of developmental cyst and there is general agreement that the odontogenic keratocyst arises from
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cell rests of the dental lamina. This cyst shows a different growth mechanism and biologic behaviour
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from the more common dentigerous cyst and radicular cyst (Brad W Neville et al., 2002; Tejasvi ML
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Avinash et al., 2010). Around 60% of all cases are diagnosed odontogenic keratocysts in people
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typically being found in adults in the second to fourth decades of life and with a slight male
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predilection(M:F=1.6:1). The age distribution appears to be bimodal. There appears to be two peaks
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of incidences between 25-34 years and 55-65 years of age.The mandible is occupied in 60% to 80%
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of cases, with a marked tendency to involve the posterior body and ascending ramus where anterior
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mandible is an uncommon site with the lesion crossing the midline (Sulabha AN et al., 2013; Brad W
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Neville et al., 2002). In maxillary region, there are inconsistencies regarding the predominant location
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of OKCs. One study shows that OKCs are distributed evenly between the anterior and posterior
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regions of maxilla; some show that there are more anterior lesions than posterior lesions and others
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concluded that the posterior region is more predominant site (Santhosh Kumar S Hiremath et al.,
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2011). Patients with keratocysts may complain of pain, mobility of teeth in the affected area, swelling,
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or discharge. Nasal obstruction, paresthesia, and root erosion are more rare symptoms. Occasionally
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diseased person may experience paresthesia of the lower lip or teeth. In many instances, patients were
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amazingly free of symptoms until the cysts reached a large size and involved the maxillary sinus and
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the entire ascending ramus, including the condylar and coronoid processes. These patients may be
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unacquainted of the lesions until they build up pathologic fractures or may be incidental finding
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during examination (Binali Çakur et al., 2008; Tejasvi ML Avinash et al., 2010). In our case, the
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patient did not spontaneously complain of pain, and was initially referred for opinion and biopsy as
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swelling didn’t subside for long. This type of cyst tends to expand in an antero-posterior direction
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within the medullary cavity of the bone without causing obvious bone expansion and this unique
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feature often becomes useful in its clinical and radiographic diagnosis because dentigerous and
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radicular cysts of comparable size are usually associated with bony expansion (Brad W Neville et al.,
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2002; Santhosh Kumar S Hiremath et al., 2011) which was not similar to our clinical finding in this
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case. Radiographically, OKCs appear as well-defined radiolucencies, which can be either unilocular
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or multilocular. They typically extend into the marrow cavity with either a smooth border contributing
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to mild bulging of the cortex but without significant cortical expansion. Keratocystic odontogenic
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tumors can show a more aggressive growth pattern including multilocularity, cortical expansion,
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perforation of the cortical bone, tooth and mandibular canal displacement, root resorption, and
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extrusion of erupted teeth (Brooke Devenney-Cakir et al., 2011). Unilocular OKCs can be located
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periapically, simulating periapical cysts; between the roots of teeth, simulating lateral periodontal
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cysts or lateral radicular cysts; surrounding the crown of unerupted teeth, simulating dentigerous
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cysts; or in the maxillary midline, simulating nasopalatine duct cysts. Large unilocular OKCs can be
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impossible to tell apart from cystic ameloblastomas. OKCs have a tendency for intra-osseous growth,
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more often in a longitudinal than in a transverse direction (minimal expansion), as seen in this case,
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thereby replacing the bone marrow, rather than giving rise to periosteal bone formation, which would
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result in a bony swelling. Rapid growth does not allow enough time for the periosteum to lay down
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new bone. These different types of appearances of OKC make diagnosis more dilemmatic as in our
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present case. The luminal content can have different consistencies described as a “straw-colored
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fluid,” “thick pus-like” material or a caseous, thick, cheesy, milk white mass (Rajkumar GC et al.,
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2011). But in our case aspiration finding was exclusively different which were more suggestive of an
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aspirate from arterio-venous malformations or Aneurysmal Bone Cyst. Histologically, KCOTs have
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been classified by some authors into parakeratotic and orthokeratotic subtypes. Classification is based
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on the lining and the type of keratin produced. Compared with the parakeratotic subtype, the
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orthokeratotic subtype produces keratin more closely resembling the normal keratin produced by the
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skin, with a keratohyaline granular layer immediately adjacent to the layers of keratin, which do not
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contain nuclei. The parakeratotic subtype has more disordered production of keratin; no keratohyaline
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granules are present, and cells slough into the keratin layer. However, in case of OKCs the lining
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epithelium is highly characteristic and consists of keratinized surface (parakeratinized-83% and
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orthokeratinized-10%) which is typically corrugated. Thickness of the epithelium is found uniformly
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arranged with 6 to 10 layers without rete-ridges (Binali Çakur et al., 2008).The keratin contains nuclei
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and is referred to as parakeratin. The parakeratotic type is the most frequent (80%) and has a more
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aggressive clinical presentation than the orthokeratotic variant. Histopathological picture shows
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presence of a well defined, often palisaded, basal layer consisting of columnar or cuboidal cells;
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intensely basophilic nuclei of columnar basal cells oriented away from the basement membrane;
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parakeratotic layers, often with a corrugated surface; and mitotic figures frequently present in
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suprabasal layers (Binali Çakur et al., 2008; Guilherme Romano Scartezini et al., 2012). This palisade
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like arrangement of basal layer is often described as “picket fence” or “tombstone” appearance. Upper
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portion of the epithelium is composed of stratified squamous epithelium with high mitotic index
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without any clear cell formation. Epithelial plaque formation is absent in OKCs but the connective
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tissue wall often shows small islands of epithelium (Santhosh Kumar S Hiremath et al., 2011). OKCs
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have a high recurrence rate ranging from 2.5 to 62 % (Sulabha AN et al., 2013; Santhosh Kumar S
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Hiremath et al., 2011) and after they occur due to incomplete removal of the original cyst’s lining,
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thin friable cystic lining, growth of the new OKC from small satellite cyst of odontogenic epithelial
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cell rests left behind after surgical treatment, or by development of an unrelated OKC in an adjacent
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region of jaw which is interpreted as a recurrence (Sulabha AN et al., 2013; Rajkumar GC et al.,
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2011). The recurrence of OKC is thought to be based on great mitotic activity and growth potential
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found in epithelium, further than other sources of recurrences such as remnants of dental lamina and
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epithelial islands (Guilherme Costa Carvalho Silvaa et al., 2006). There is no doubt that recurrences
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may arise if any part of the lining is left behind. All efforts should, therefore, be made at proper
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enucleation and elimination of possible remnants of the cyst wall in case the cyst ruptures and has to
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be removed piecemeal. There is also a possibility that microcysts are present in the connective tissue
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of the cyst wall and that these are left behind after enucleation. Some keratocysts show active budding
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of the basal layer of the epithelial lining that may reach to the periphery of the connective tissue wall
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and, therefore, may also be the source of a true recurrent cyst. The third reason for a recurrent OKC is
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the development of a new keratocyst from an epithelial island or microcyst left behind in the mucosa
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(Paul JW Stoelinga, 2005). A recent study suggests that PTCH1 mutations, particularly those causing
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protein truncations, are associated with OKCs showing increased proliferative activity and thus
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related to a phenotype of higher recurrent tendency. When taking into consideration removal of a
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keratocyst, however, it is important to keep in mind the 3 possible reasons why an OKC could recur
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as stated above. Therefore, treatment should aim at complete elimination of possible vital cells left
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behind in the defect. In Browne’s series, three different treatment methods were evaluated, which
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were marsupialization, enucleation and primary closure, and enucleation and open dressing (Rajkumar
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GC et al., 2011). There was no correlation between treatment method and the rate of recurrence.
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Morgan and colleagues categorize surgical treatment methods for KCOT as conservative or
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aggressive. Conservative treatment of OKC is “cyst-oriented” and, thus, includes enucleation, with or
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without curettage, or marsupialization. Aggressive treatment addresses the “neoplastic nature” of the
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KCOT and includes peripheral ostectomy, chemical curettage with Carnoy’s solution or en bloc
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resection (Tejasvi ML Avinash et al., 2010; Rajkumar GC et al., 2011). Enucleation has a benefit over
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marsupialization as complete specimen can be sent for histopathologic examination (Rajkumar GC et
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al., 2011). The purpose of using Carnoy’s solution is to provide a total elimination of epithelial
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remnants from the cyst walls, which may cause recurrences (Guilherme Romano Scartezini et al.,
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2012). Researchers have suggested that the recurrence rate is relatively low with aggressive treatment,
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whereas more conservative methods tend to result in more recurrences and after the combined therapy
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of enucleation and Carnoy’s solution, the recurrence rate was found to be 9% (Aydin Ozkan et al.,
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2012). Recurrence is documented in many cases even after 10 years of follow up and treatment.
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Conclusion: In conclusion, benign uni- or multicystic intraosseous tumors of odontogenic origin
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should be considered in the differential diagnosis of jaw lesions. Due to variation of its clinical and
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radiological appearances diagnosis, becomes confusing and tricky. In spite of, even in the incidence of
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clinical and radiological features suggestive of KCOT, a definitive diagnosis cannot be made without
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microscopic investigation. Only thorough investigation will allow to arrive at the most effective
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treatment and thus to pass up the recurrences.
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