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Type of manuscript: Case Report
Title of manuscript: Odontogenic Keratocyst: An Unusual Clinical Presentation
Authors: Dr. Nitin Agarwal 1, Dr. Debanti Giri 2, Dr. Abhishek Sinha 3, Dr. Sunita Srivastava 4, Dr.
Ruchika K. Prasad 5, *Dr.Abhishek Singh Nayyar6
Affiliations:
Dr. Nitin Agarwal, Professor & Head, Dept of Oral Medicine and Radiology, Sardar Patel Post
Graduate Institute of Dental & Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, India.
Dr. Debanti Giri, Post graduate student, Dept of Oral Medicine and Radiology, Sardar Patel Post
Graduate Institute of Dental & Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, India.
Dr. Abhishek Sinha, Reader, Dept of Oral Medicine and Radiology, Sardar Patel Post Graduate
Institute of Dental & Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, India.
Dr. Sunita Srivastava, Senior Lecturer, Dept of Oral Medicine and Radiology, Sardar Patel Post
Graduate Institute of Dental & Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, India.
Dr. Ruchika K. Prasad, Senior Lecturer, Dept of Oral Medicine and Radiology, Sardar Patel Post
Graduate Institute of Dental & Medical Sciences, Raebareily Road, Lucknow, Uttar Pradesh, India.
Dr. Abhishek Singh Nayyar, Reader, Department of Oral Medicine and Radiology, SaraswatiDhanwantari Dental College and Hospital and Post-Graduate Research Institute, Parbhani,
Maharashtra, India. E-mail: [email protected]
*Corresponding Author:
Dr.Abhishek Singh Nayyar,
Reader cum Associate Professor, Department of Oral Medicine and Radiology,
Saraswati-Dhanwantari Dental College and Hospital and Post-Graduate Research Institute,
Parbhani, Maharashtra, India
E-mail id: [email protected]
Contact No.7350990780
Odontogenic Keratocyst: An Unusual Clinical Presentation
Abstract: The odontogenic keratocyst (OKC) is a developmental cyst of the jaws of epithelial origin.
This lesion is commonly found in the ascending ramus and posterior mandible, and can become
quite large due to its rapid growth and its extension into the adjacent structures. Herein, we are
reporting a case of odontogenic keratocyst involving the anterior mandible which was mimicking
other cysts and tumors which occur in that region more frequently. On closer view into the case, the
lesion was proved to be an OKC. The lesion was successfully treated by complete enucleation.
Because of the uniqueness of this case, the clinical, radiological, and histological features of this
cyst/tumor are discussed in this article.
Key words: Odontogenic Keratocyst, Odontogenic Cysts, Keratocystic Odontogenic Tumor, Mandible,
Bimodal, Carnoy’s solution
Introduction: Ever since Philipsen described the odontogenic keratocyst (OKC) in 1956, the lesion has
turned into a center of momentous clinical interest because of its unusual growth pattern and
tendency to recur even after surgical removal (Sulabha AN et al., 2013). This lesion was recently
renamed by Philipsen as keratocystic odontogenic tumour (KCOT) and reclassified as an odontogenic
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
multicystic intraosseous tumour of odontogenic origin, with a characteristic lining of parakeratinized
stratified squamous epithelium and potentially aggressive, infiltrative behavior (MacDonaldJankowski DS, 2011; Binali Çakur et al., 2008). Odontogenic Keratocyst (OKCs) of the jaw is also
recognized as a developmental cyst and there is general agreement that the odontogenic keratocyst
arises from the cell rests of the dental lamina. This cyst shows a different growth mechanism and
biologic behaviour unlike the other more common jaw cysts and tumors (Brad W Neville et al., 2002;
Tejasvi ML Avinash et al., 2010). Odontogenic keratocysts may be found in a wide range of patients
who range in age from infancy to old age. KCOTs are twice more frequent in the mandible than in
the maxilla (Guilherme Romano Scartezini et al., 2012) and mandible is occupied in 60% to 80% of
cases. Radiographically, OKCs appear as well-defined radiolucencies, which can be either unilocular
or multilocular and they may appear as small or large, round or ovoid radiolucent lesions, often with
scalloped, multilocular, distinct sclerotic margins. This type of cyst may occur in conjunction with an
impacted tooth as has been reported in 25 to 40% of cases; in such instances, the radiographic
features suggesting more towards a diagnosis of dentigerous cyst while smaller cysts makes the
diagnosis more dilemmatic (Brad W Neville et al., 2002; Guilherme Romano Scartezini et al., 2012;
Santhosh Kumar S Hiremath et al., 2011).
Case Report: A 23 year old male patient reported the Department of Oral Medicine and Radiology
with a chief complaint of swelling in lower front tooth region and face since last 1 year when he
suddenly noticed a small swelling in the lower right front tooth region inside the mouth and he took
some medication. After taking those medications, the swelling completely subsided. After 6 months,
similar swelling appeared which was progressively increasing in size and was not subsided by taking
medication. The swelling continued increasing in size and affected the other teeth on opposite side
also. After 10 days, he noticed swelling on face in mirror. He immediately went to local dentist who
advised for a biopsy. Clinical examination revealed a single, ill defined swelling present in relation to
the lower 1/3 rd of the face near symphysis and parasymphysis regions which crossed midline and
was measuring approximately 5 X 3 cm in diameter extends mesiodistally from right parasymphyseal
region to left parasymphyseal region and superioinferiorly from the vermilion border of lip of right
side and corner of mouth of left side till 1 cm below the lower border of mandible. Swelling
appeared to be with smooth surface with shiny, slightly stretched skin and on palpation, swelling
appeared to be bony hard in consistency with well-defined margins and with a smooth lobular
surface. (Fig.1) Swelling was slightly tender on right side with no local rise in temperature. Superficial
skin was pinchable with no secondary changes. (Fig.2) Intra-orally, mandibular vestibule was
obliterated by swelling in relation to 35 to 43 region. On detailed intraoral examination, a single well
defined swelling was present in anterior part of mandible extending from 35 to 43 region with
buccolingual expansion. Buccally, swelling extended mesiodistally from mesial aspect of 43 to the
distal aspect of 35 crossing midline and buccolingually from the alveolar crest to the buccal and
labial vestibule obliterating the vestibular region. Superficial alveolar mucosa appeared to be
stretched with detachment of the marginal gingiva in relation to 33, 34, 35. Swelling was slightly
tender on right side. Swelling was bony hard in consistency with egg shell crackling which was
present distal to 33. Bucco-lingual expansion was seen evident throughout the swelling. (Fig.3, 4) On
pulp vitality test, 32 was found to be completely non-responding while 35 showed delayed response.
During aspiration, a dark blackish-red betadine coloured aspirate was taken out. (Fig.5) IAOPAR irt
31,32,33,41,42,43 were advised. IOPAR revealed multilocular well defined radiolucencies
overlapping each other at the apical regionof 31,32,33,41,42. Margin of the radiolucencies were
sclerotic. (Fig.6, 7) Orthopantomogram showed well defined multilocular radiolucency extending
from the periapical region of 35 till the peripical region of 43,44. Sclerotic margin with displacement
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of teeth could be appreciated. The lower border of the radiolucency overlapped and crossed the
inferior border of mandible. Apical 1/3 of roots in relation to 33,35,43,45 appeared knife edged
suggestive of root resorption. (Fig.8) Mandibular topographic occlusal radiograph was performed to
check the buccolingual expansion. In this radiograph, multilocular radiolucency was evidently seen in
the apex of 33,32,32,41,42 region. A thin sclerotic line was also appreciated on the buccal aspect of
32,33,34,35 suggestive of buccolingual expansion. Inferior border of mandible appeared intact.
(Fig.9) On the basis of clinical examination and chair side investigations, a provisional diagnosis was
made for central giant cell granuloma with differential diagnosis of Glandular odontogenic cyst,
Aneurysmal bone cyst, Ameloblastoma, Odontogenic keratocyst, and an Arteriovenous
malformation in relation to 35 to 43 region. The lesion was examined at biopsy. Histopathologically,
H & E staining showed epithelia overlying the connective tissue stroma. The epithelium was
parakeratinized, stratified squamous, 8-10 layer thick. Basal cells were tall columnar in appearance
with hyperchromatic nuclei, exhibiting reverse polarity. Loss of cellular adhesion and subepithelial
split could also be seen. Connective tissue stroma showed juxtaepithelial hyalinization and was
sparsely cellular with loose collagen fibres, acute inflammatory cell infiltrate and areas of
haemorrhage. (Fig.10) Surgical enucleation was performed under all aseptic precautions and was
started antibiotic prophylaxis. Complete enucleation with aggressive curretage was performed.
Patient was kept under repeated follow-up for three months. Patient was re-called after 3 months
and after 9 months. (Fig.11)
Discussion: An insight into the times past of odontogenic cysts goes back to the 19th century, when
the odontogenic keratocyst (OKC) was first described in the year 1876 and was named by Phillipsen
in 1956 (Brad W Neville et al., 2002) who described it as a different entity characterized by a
keratinized lining, presence of satellite cysts and association with the nevoid basal cell carcinoma
syndrome (A Nagraja et al., 2012). Philipsen and Riechert have suggested that OKC should be
considered as a benign tumour and hence be called as KCOT or keratocystic odontogenic tumour.
Shear has countered this argument by saying that even if it is a neoplasm, it is suitable to be called as
OKC as many neoplasms do not essentially have a suffix ‘oma’. This debate was started by Shear
(2003) that OKC should be called as keratocystoma which led Philipsen and Riechert suggesting
keratinising cystic odontogenic tumour in 2004 and then Philipsen suggesting keratocystic
odontogenic tumor again in 2005 (Mahadesh Jyothi et al., 2010). This cyst has a propensity for
recurrence and the aggressive behaviour clinically and histologically has necessitated the
reclassification of the lesion by the World Health Organization (WHO, 2005) as a ‘keratocystic
odontogenic tumor’ (KCOT). The KCOT is defined as ‘a benign uni- or multicystic, intra-osseous tumor
of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium
and potential for aggressive, infiltrative behaviour (MacDonald-Jankowski DS, 2011; Binali Çakur et
al., 2008; Rajkumar GC et al., 2011). Odontogenic Keratocyst (OKCs) of the jaw is a type of
developmental cyst and there is general agreement that the odontogenic keratocyst arises from cell
rests of the dental lamina. This cyst shows a different growth mechanism and biologic behaviour
from the more common dentigerous cyst and radicular cyst (Brad W Neville et al., 2002; Tejasvi ML
Avinash et al., 2010). Around 60% of all cases are diagnosed odontogenic keratocysts in people
typically being found in adults in the second to fourth decades of life and with a slight male
predilection(M:F=1.6:1). The age distribution appears to be bimodal. There appears to be two peaks
of incidences between 25-34 years and 55-65 years of age.The mandible is occupied in 60% to 80%
of cases, with a marked tendency to involve the posterior body and ascending ramus where anterior
mandible is an uncommon site with the lesion crossing the midline (Sulabha AN et al., 2013; Brad W
Neville et al., 2002). In maxillary region, there are inconsistencies regarding the predominant
location of OKCs. One study shows that OKCs are distributed evenly between the anterior and
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posterior regions of maxilla; some show that there are more anterior lesions than posterior lesions
and others concluded that the posterior region is more predominant site (Santhosh Kumar S
Hiremath et al., 2011). Patients with keratocysts may complain of pain, mobility of teeth in the
affected area, swelling, or discharge. Nasal obstruction, paresthesia, and root erosion are more rare
symptoms. Occasionally diseased person may experience paresthesia of the lower lip or teeth. In
many instances, patients were amazingly free of symptoms until the cysts reached a large size and
involved the maxillary sinus and the entire ascending ramus, including the condylar and coronoid
processes. These patients may be unacquainted of the lesions until they build up pathologic
fractures or may be incidental finding during examination (Binali Çakur et al., 2008; Tejasvi ML
Avinash et al., 2010). In our case, the patient did not spontaneously complain of pain, and was
initially referred for opinion and biopsy as swelling didn’t subside for long. This type of cyst tends to
expand in an antero-posterior direction within the medullary cavity of the bone without causing
obvious bone expansion and this unique feature often becomes useful in its clinical and radiographic
diagnosis because dentigerous and radicular cysts of comparable size are usually associated with
bony expansion (Brad W Neville et al., 2002; Santhosh Kumar S Hiremath et al., 2011) which was not
similar to our clinical finding in this case. Radiographically, OKCs appear as well-defined
radiolucencies, which can be either unilocular or multilocular. They typically extend into the marrow
cavity with either a smooth border contributing to mild bulging of the cortex but without significant
cortical expansion. Keratocystic odontogenic tumors can show a more aggressive growth pattern
including multilocularity, cortical expansion, perforation of the cortical bone, tooth and mandibular
canal displacement, root resorption, and extrusion of erupted teeth (Brooke Devenney-Cakir et al.,
2011). Unilocular OKCs can be located periapically, simulating periapical cysts; between the roots of
teeth, simulating lateral periodontal cysts or lateral radicular cysts; surrounding the crown of
unerupted teeth, simulating dentigerous cysts; or in the maxillary midline, simulating nasopalatine
duct cysts. Large unilocular OKCs can be impossible to tell apart from cystic ameloblastomas. OKCs
have a tendency for intra-osseous growth, more often in a longitudinal than in a transverse direction
(minimal expansion), as seen in this case, thereby replacing the bone marrow, rather than giving rise
to periosteal bone formation, which would result in a bony swelling. Rapid growth does not allow
enough time for the periosteum to lay down new bone. These different types of appearances of OKC
make diagnosis more dilemmatic as in our present case. The luminal content can have different
consistencies described as a “straw-colored fluid,” “thick pus-like” material or a caseous, thick,
cheesy, milk white mass (Rajkumar GC et al., 2011). But in our case aspiration finding was exclusively
different which were more suggestive of an aspirate from arterio-venous malformations or
Aneurysmal Bone Cyst. Histologically, KCOTs have been classified by some authors into parakeratotic
and orthokeratotic subtypes. Classification is based on the lining and the type of keratin produced.
Compared with the parakeratotic subtype, the orthokeratotic subtype produces keratin more closely
resembling the normal keratin produced by the skin, with a keratohyaline granular layer immediately
adjacent to the layers of keratin, which do not contain nuclei. The parakeratotic subtype has more
disordered production of keratin; no keratohyaline granules are present, and cells slough into the
keratin layer. However, in case of OKCs the lining epithelium is highly characteristic and consists of
keratinized surface (parakeratinized-83% and orthokeratinized-10%) which is typically corrugated.
Thickness of the epithelium is found uniformly arranged with 6 to 10 layers without rete-ridges
(Binali Çakur et al., 2008).The keratin contains nuclei and is referred to as parakeratin. The
parakeratotic type is the most frequent (80%) and has a more aggressive clinical presentation than
the orthokeratotic variant. Histopathological picture shows presence of a well defined, often
palisaded, basal layer consisting of columnar or cuboidal cells; intensely basophilic nuclei of
columnar basal cells oriented away from the basement membrane; parakeratotic layers, often with a
corrugated surface; and mitotic figures frequently present in suprabasal layers (Binali Çakur et al.,
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2008; Guilherme Romano Scartezini et al., 2012). This palisade like arrangement of basal layer is
often described as “picket fence” or “tombstone” appearance. Upper portion of the epithelium is
composed of stratified squamous epithelium with high mitotic index without any clear cell
formation. Epithelial plaque formation is absent in OKCs but the connective tissue wall often shows
small islands of epithelium (Santhosh Kumar S Hiremath et al., 2011). OKCs have a high recurrence
rate ranging from 2.5 to 62 % (Sulabha AN et al., 2013; Santhosh Kumar S Hiremath et al., 2011) and
after they occur due to incomplete removal of the original cyst’s lining, thin friable cystic lining,
growth of the new OKC from small satellite cyst of odontogenic epithelial cell rests left behind after
surgical treatment, or by development of an unrelated OKC in an adjacent region of jaw which is
interpreted as a recurrence (Sulabha AN et al., 2013; Rajkumar GC et al., 2011). The recurrence of
OKC is thought to be based on great mitotic activity and growth potential found in epithelium,
further than other sources of recurrences such as remnants of dental lamina and epithelial islands
(Guilherme Costa Carvalho Silvaa et al., 2006). There is no doubt that recurrences may arise if any
part of the lining is left behind. All efforts should, therefore, be made at proper enucleation and
elimination of possible remnants of the cyst wall in case the cyst ruptures and has to be removed
piecemeal. There is also a possibility that microcysts are present in the connective tissue of the cyst
wall and that these are left behind after enucleation. Some keratocysts show active budding of the
basal layer of the epithelial lining that may reach to the periphery of the connective tissue wall and,
therefore, may also be the source of a true recurrent cyst. The third reason for a recurrent OKC is
the development of a new keratocyst from an epithelial island or microcyst left behind in the
mucosa (Paul JW Stoelinga, 2005). A recent study suggests that PTCH1 mutations, particularly those
causing protein truncations, are associated with OKCs showing increased proliferative activity and
thus related to a phenotype of higher recurrent tendency. When taking into consideration removal
of a keratocyst, however, it is important to keep in mind the 3 possible reasons why an OKC could
recur as stated above. Therefore, treatment should aim at complete elimination of possible vital
cells left behind in the defect. In Browne’s series, three different treatment methods were
evaluated, which were marsupialization, enucleation and primary closure, and enucleation and open
dressing (Rajkumar GC et al., 2011). There was no correlation between treatment method and the
rate of recurrence. Morgan and colleagues categorize surgical treatment methods for KCOT as
conservative or aggressive. Conservative treatment of OKC is “cyst-oriented” and, thus, includes
enucleation, with or without curettage, or marsupialization. Aggressive treatment addresses the
“neoplastic nature” of the KCOT and includes peripheral ostectomy, chemical curettage with
Carnoy’s solution or en bloc resection (Tejasvi ML Avinash et al., 2010; Rajkumar GC et al., 2011).
Enucleation has a benefit over marsupialization as complete specimen can be sent for
histopathologic examination (Rajkumar GC et al., 2011). The purpose of using Carnoy’s solution is to
provide a total elimination of epithelial remnants from the cyst walls, which may cause recurrences
(Guilherme Romano Scartezini et al., 2012). Researchers have suggested that the recurrence rate is
relatively low with aggressive treatment, whereas more conservative methods tend to result in more
recurrences and after the combined therapy of enucleation and Carnoy’s solution, the recurrence
rate was found to be 9% (Aydin Ozkan et al., 2012). Recurrence is documented in many cases even
after 10 years of follow up and treatment.
Conclusion: In conclusion, benign uni- or multicystic intraosseous tumors of odontogenic origin
should be considered in the differential diagnosis of jaw lesions. Due to variation of its clinical and
radiological appearances diagnosis, becomes confusing and tricky. In spite of, even in the incidence
of clinical and radiological features suggestive of KCOT, a definitive diagnosis cannot be made
without microscopic investigation. Only thorough investigation will allow to arrive at the most
effective treatment and thus to pass up the recurrences.
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