Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105

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Case Report
Odontogenic keratocyst- Mimicking residual cyst in maxilla

Nalini Tomar1, *, Kuldeep Singh Bhadauria2

1 YMT Dental College & Research Institute, Navi Mumbai, Maharashtra, India
2 Thyrocare Technologies Limited, Navi Mumbai, Maharashtra, India

ARTICLE INFO ABSTRACT

Article history: OKC was classified as cystic lesion by WHO in 1971 & 1991, based on aggressive nature, growth pattern,
Received 04-05-2020 clinical, histological and immunohistochemical nature in 2005 they again classified it as benign lesion,
Accepted 12-05-2020 however in 2017 WHO head and neck pathology reclassified it as cystic lesion. It more commonly occurs
Available online 18-07-2020 in posterior mandible and rarely occurs in maxilla, in this case occurrence of OKC in maxillary posterior
region is very rare with distinctive expansion and lifting of maxillary sinus floor without perforating in
edentulous area makes it more difficult to detect and justify from residual cyst. Here a 65 years old patient
Keywords: came with chief complaint of pus discharge from upper left posterior region since 7 months, having a small
Carnoy’s solution
opening in edentulous ridge, which provisional diagnosis was given as residual cyst later after excision of
Maxillary sinus
lesion and histopathological analysis it was given as OKC.
Keratocystic odontogenic tumour
Odontogenic keratocyst © 2020 Published by Innovative Publication. This is an open access article under the CC BY-NC license
(https://creativecommons.org/licenses/by-nc/4.0/)

1. Introduction and pain can be seen likely due to secondary infections. 5

As they are often confused with other cysts and tumours
Philipsen in 1956, first described the term ‘odontogenic
because of vague appearances and similar features it is
keratocyst’ (OKC), as developmental cyst of jaw. 1
important to diagnosis. Similar in our case OKC occurred
Histologic criteria to detect OKC was given by Pindborg and
in edentulous area with history of long term presence but
Hansen. 2 Intially it was given as odontogenic keratocyst,
it cannot be proved clinically or radiological as OKC but
but later on WHO classified it as Keratocystic odontogenic
on histopathology it was proved. So complete diagnosis and
tumour because of its aggressive nature and growth pattern
proper treatment is necessary to reduce recurrences.
and mutational changes, 3 it is non inflammatory and arises
from cell rest of dental lamina. Now in 2017 again WHO
reclassified it as OKC. 4 They are usually seen in mandibular
posterior regions and rarely in maxilla, about 70-80% occur 2. Case Report
in the lower jaw, about 90% occur posterior to the canines
and 50% in the ascending ramus of the mandible, very rarely A 65 years old female patient reported to the department
in the maxilla. 5 with chief complaint of pus discharge from upper left
WHO gave new classification in which they changed posterior region of jaw since 7 months. Initially patient
the designation to OKC from keratocystic odontogenic was asymptomatic, later on sudden onset of swelling
tumour (KCOT) and described it as a benign uni or with pus discharge in that area. Swelling was smaller in
multilocular, intraosseous tumour of to odontogenic origin, size and gradually increased to present size. Patient gives
with aggressive and invading behaviour”. 3 Usually there are history of extraction 2 years back after which she had
no significant clinical symptoms, although mild swelling experienced mild pain during chewing for which she has
* Corresponding author. taken medication from local dentist, patient is diabetic and
E-mail address: [email protected] (N. Tomar). hypertensive since 2 years and under medication.

https://doi.org/10.18231/j.jooo.2020.023
2395-6186/© 2020 Innovative Publication, All rights reserved. 98
 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105 99

2.1. On examination
(Figure 1 a and b) Mild facial fullness and tenderness over
left side of cheek. Intraoral (Figure 2) single diffuse swelling
present in maxillary left posterior region which is of 3 x
3cms in size obliterating buccal vestibule, Anteroposteriorly
from 24 to 28 region, Supero-Inferior from alveolar ridge
to depth of vestibule, expanding the ridge overlying surface
appears normal and surrounding too. Intraoral examination
showed a tiny draining sinus is seen in crest of edentulous
ridge in 27 region.

Fig. 4: Showing after excision of lesion, opening in alveolar ridge

Fig. 1: (a),(b) front and lateral profile showing mild facial fullness
left cheek

Fig. 5: Iopa showing interrupted floor of maxillary sinus

Fig. 2: Showing diffuse swelling in left buccal vestibule

Fig. 6: a and b reveals large lesion involving left maxillary sinus

with anteroposterior and buccopalatal expansion

palpable which is soft, mobile, mildly tender. So provisional

diagnosis is given as residual cyst of left maxillary region.
Patient is advised for IOPA, OPG, CBCT, routine
Fig. 3: Aspiration showing cheesy fluid with blood. investigation, aspiration and biopsy. Aspiration- (Figure 3)
Milky white cheesy liquid with RBCs aspirate on
On palpation swelling is firm in consistency, mildly investigation revealed keratocyst. IOPA (Figure 5) and OPG
tender, compressible, egg shell crackling and expansion felt. reveals unilocular radiolucency surrounded by corticated
Clinically present 13, root pieces-14,15 and mesioproximal border which is interrupted at base and opening in soft tissue
caries with 17. Single left submandibular lymph node is which is hazy and appears like involving maxillary sinus.
100 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105

Fig. 7: CBCT sections showing unilocular hyperdense area in maxillary sinus encroaching and obliterating it except superior aspect.

CBCT(Figures 6 and 7) reveals well defined, corticated, Curettage & enucleation of the lesion followed by
unilocular hyperdense areas in left maxillary sinus application of Carnoy’s solution used for reducing the recur-
measuring around 26.7mm in vertical height and 33.3mm rence rate under general anaesthesia (Figure 4). Excised
in mediolateral dimensions. Antero-posterior extensions are tissue specimen was submitted for the histopathological
approximately 31.4mm in length. Missing 17, 16, 12, 11, examination. On histopathological examination it was
21, 22, 23, 24, 25, 26 and 27 is observed. Hyperdense confirmed as OKC. Patient was kept on follow up for 6
area obliterating left maxillary sinus from all aspects except months and no recurrent lesion seen.
the superior aspect of left maxillary sinus. Floor of left
maxillary sinus is displaced superiorly due to the extension 3. Discussion
of the lesion. Coronal Sections depicts the well defined
border of lesion involving and encroaching the lower part of In the earlier literature, the OKC was described as a
left maxillary sinus. Buccal and palatal bone in left posterior cholesteatoma (Hauer, 1926; Kostecka, 1929). Forssell
region in relation with edentulous area of 26, 27 and 28 is (1980) concluded that the first account of this lesion was
lost. The alveolar crest in the edentulous region is destructed given by Mikulicz in 1876, described it as a dermoid cyst. 1
in relation with 26 and 27, given as residual cyst in relation The epithelium of the keratocyst is strongly believed to
to left maxilla. arise from either the dental lamina or the residue of the
dental lamina (cell rests of Serre). 4
 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105 101

OKC are relatively common developmental odontogenic Noonans syndrome, Orofacial digital syndrome, Simpson
cysts and account for 10–12% of all jaw cysts. 6 The term golabi-behmel syndrome. 10,11
Odontogenic Keratocyst (OKC) was first coined by Philisen Radiographically most KCOTs are unilocular or mul-
in 1956 (Eryilmaz et al., 2009) and its characterstic features tilocular, presenting a well-defined peripheral rim (corti-
was first described by Pindborg and Hansen in 1963. 1 cated), with scalloped borders, root resorption, displacement
Odontogenic keratocyst (OKC) is so named because keratin of teeth with involvement of impacted tooth. Buccal bone
is produced by the cystic lining. It is a Parakeratin lined expansion more compared to lingual with or without
cyst-like lesion within bone. OKC is the one of the rare and perforation. Small lesion have minimal expansion due to
distinctive developmental odontogenic cyst which from the its growth pattern in antero posterior direction when it
dental lamina, containing clear fluid and a cheesy material increases it involved buccal and lingual bones too. 3,7,10
resembling keratin debris. 7 In the present case, the radiographic evaluation revealed
Its journey of nomenclature is as follows: unilocular radiolucency in left maxillary posterior region
which is aggressive osteolytic lesion, involving edentulous
1. Dental cyst (John Hunter 1774), area causing perforations in the buccal and palatal cortical
2. Dermoid Cyst (Mikulicz 1876) plates in the 24, 25, 26, 27 region, lesion encroaching
3. Primordial cyst (Robinson 1945) and lifting maxillary sinus floor without perforating it and
4. Keratocystoma (Shear) Odontogenic keratocyst maintaining its border.
(Philipsen 1956 & Pindborg and Hansen 1963) Radiological Types of keratocyst
5. Benign neoplasm (Toller 1967)
6. Odontogenic keratocyst (WHO 1971) 1. Replacement type: Cyst which forms in the place of
7. True benign cystic epithelial neoplasm (Ahlfors 1984) normal teeth.
8. Odontogenic keratocyst (WHO 1992) 2. Envelopmental type: Cyst which envelopes adjacent
9. Keratocystic odontogenic tumor (Benign neoplasm) impacted tooth.
(WHO 2005) 3. Extraneous type: Cyst which occur in ascending ramus
10. Odontogenic keratocyst (WHO 2017) 1,8 away from the teeth.
4. Collateral type: Cyst which occurs adjacent to the
The keratocystic odontogenic tumor is more common in root of teeth which are indistinguishable radiologically
males than females with ratio of 1.6:1 and occurs over from lateral periodontal cyst. 8
a wide age range. White more common, it is typically KCOT: In 1967, Toller suggested that the OKC should
diagnosed during the second to fourth decade. In our case, be considered as benign tumour because of its clinical
the patient was in his sixth decade. 9 Peripheral OKCs have nature. 1,4 In 1984, Ahlfors et. al 12 suggested that “if we
female predominance with male: female ratio of 2.2:1. 7 consider OKC as benign neoplasm, the question of modified
KCOTs (Figures 8 and 9) mostly occur in the body of the treatment would be raised.” Since, many published reports
mandible, most commonly in the molar region and vertical in these years have influenced WHO to again classify the
ramus. 9 However in present case, there was involvement of lesion as a tumour.
maxillary posterior region which is edentulous and lifting
of the maxillary sinus seen. In our case shows a rare site 1. Behaviour: KCOT is localised catastrophic and high
of occurrence in a female. Literature suggests that less than chances of recurrence.
1% of the KCOT cases occur in the maxilla with maxillary 2. Histopathology: Studies such as that by Ahlfors and
antrum involvement. In the maxilla, the canine region is the others 10 show the basal layer of the KCOT budding
most common location for KCOTs. 10 into connective tissue. In addition, WHO notes that
The clinical features include pain or without pain, soft- mitotic figures are frequently found in the suprabasal
tissue swelling, displacement of teeth, expansion of bone in layers. 2
anteroposterior in medullary space of bone, drainage, and 3. Genetics: PTCH (“patched”), a tumour suppres-
various neurological manifestations, such as, paresthesia sor gene involved in both NBCCS and sporadic
of the lip or teeth in case of lower jaw involvement. The KCOTs, occurs on chromosome 9q22.3-q31. 13–16
maxillary KCOT tends to be secondarily infected with Normally,PTCH forms a receptor complex with the
greater frequency than the mandibular ones, due to its oncogene SMO (“smoothened”) for the SHH (“sonic
vicinity to the maxillary sinus. 10 In this case too, the lesion hedgehog”) ligand. PTCH binding to SMO inhibits
was secondarily infected, with the presence of mild pain, growth-signal transduction. SHH binding to PTCH
a sinus opening and pus discharge. Expansion of buccal releases this inhibition. 14 If normal functioning of
cortex in 30% of maxillary and 50% of mandibular regions. 3 PTCH is lost, the proliferation-stimulating effects of
Syndromes associated with multiple OKC are Nevoid SMO are permitted to predominate.
Basal cell carcinoma syndrome (NBCCS), Gorlin goltz 4. Recent studies revealed that the etiology of NBCCS
syndrome, Marfans syndrome, Ehlers danlos syndrome, and KCOTs has a “2-hit mechanism,” with allelic
102 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105

Fig. 8: Showing WHO 2005 classification in which OKC is included in benign lesion as KCOT and 2017 reclassified as cystic lesion as
OKC.
 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105 103

Fig. 9: Showing WHO 2005 classification in which OKC is included in benign lesion as KCOT and 2017 reclassified as cystic lesion as
OKC.
104 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105

loss at 9q22.42,43. 13 Tumour suppressor gene is 7. Others findings are satellite cysts, daughter cysts (7-
inactivated in “2 hit mechanism”. The first hit is a 30%), solid epithelial proliferation, odontogenic rests
mutation in one allele, which, although it can be basal layer budding may be seen. Fibrous connective
dominantly inherited, has no phenotypic effect. The tissue wall may get mineralised and may include
second hit refers to loss of the other allele and is cholesterol crystals and Rushton bodies.
known as “loss of heterozygosity” (LOH). In KCOTs,
this leads to the dysregulation of the oncoproteins The mutliple factors could be responsible for reoccurrence
cyclin D1 and p53. Lench and others 15 indicate that of okc are :
LOH in the 9q22.3-q31 region has been reported
for many epithelial tumours, including basal cell 1. After incomplete removal of cystic lining
carcinomas, squamous cell carcinomas and transitional 2. Very thin and friable nature of epithelial lining,
cell carcinomas; they note that LOH is, “by definition 3. Higher level of cell proliferative activity in the
a feature of tumorigenic tissue”. 17 epithelium
4. Budding in the basal layer of the epithelium
Numerous surgical modalities have been suggested for the 5. Bony perforation
treatment of KCOTs, including, enucleation with primary 6. Adherence to adjacent soft tissue
closure, enucleation with open packing, marsupilization, 7. Supra-epithelial and Sub-epithelial split of the
enucleation with use of Carnoy’s solution or cryotherapy, epithelial lining
with a marginal or radical section. Although KCOTs are 8. Parakeratinization of the surface layer
stated to be the most aggressive and recurrent form of 9. Remnants of dental lamina epithelium not associated
tumors, there are cases where KCOTs have been treated by with original OKC and development of new OKC in
enucleation. In the present case also it has been noted that the adjacent area.
there has been complete healing following the enucleation 10. Growth of new OKC from satellite cyst /daughter
with carnoys’s solution procedure. Nevertheless, a long- cyst/remnants/cell rest. 4,8
term follow-up is required to check recurrence.
Recurrence of OKC/KCOT: Recurrence rate was found to
1. Enucleation - 30%. vary from 0% to about 62%, depending on the kind of
2. Enucleation + carnoy’s solution - 9%.. treatment management and follow-up period. 8
3. Enucleation + peripheral ostectomy - 18%
4. Enucleation + carnoy’ solution + peripheral ostectomy 4. Conclusion
- 8%.
5. Enucleation + cryotherapy - 38%. Although we have many diagnostic tools to research articles
6. Marsupialization - 33%. still OKCs are routinely seen but their diagnosis is as
7. Marsupialization + cystectomy - 13%. difficult as previously, because of its appearances in both
8. Resection - 0%. multi and unilocular fashion with occurrence in wide age
group to any site and due to varying pattern its difficult
Recurrence rates with different treatment modalities to precise it. Its difficult to give exact diagnosis and treat
(Madras and Lapointe, 2008). 4,11 it, as high chances of occurrences is there. So it should
Histological features - Pindborg, phillipsen and Henrik- be considerate in differential diagnosis of cystic lesion and
sen (Pindborg et al., 1962) 18 suggested series of histological tumours.
features for the diagnosis of OKC which includes:
1. Stratified squamous epithelium lining which is thin 5. Source of Funding
and having wavy appearance – 8-10 layers thick. None.
2. Lacks of rete ridges/pegs / epithelial extensions.
3. Well defined basal cell layer having cuboidal or 6. Conflict of Interest
columnar cells arranged in palisaded fashion described
as “picket fence or tombstone appearance.” There is no conflict of interest.
4. A thin spinous cell layer which often shows direct
transition from basal cell layer (artifactual separation References
of epithelium from basement membrane) and spinous 1. Shear M, Speight P. Odontogenic keratocyst. In: Shear M, Speight
cell layer intracellular oedema. P, editors. Cysts of the Oral and Maxillofacial Regions. Oxford:
5. Surface keratinisation which is corrugated and rippled Blackwell Munksgaard; 2007. p. 6–58.
2. El-Hajj G, Anneroth G. Odontogenic keratocysts—a retrospective
and mostly parakeratosis (keratinized cells with nuclei. clinical and histologic study. Int J Oral Maxillofac Surg.
6. Cystic wall composed of fibrous connective tissue 1996;25(2):124–9.
which is thin and usually uninflamed.
 Tomar and Bhadauria / Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology 2020;6(2):98–105 105

3. Philipsen HP. Keratocystic odontogenic tumour. In: Barnes L, Eveson 14. Cohen MM. Nevoid basal cell carcinoma syndrome: molecular
JW, Reichart P, Sidransky D, editors. Pathology and genetics of head biology and new hypotheses. Int J Oral Maxillofac Surg.
and neck tumours. Lyon: IARC Press; 2005. p. 306–7. 1999;28(3):216–23.
4. Madras J, Lapointe H. Keratocystic odontogenic tumor: Reclassifica- 15. Lench NJ, High AS, Markham AF, Hume WJ, Robinson PA.
tion of the odontogenic keratocyst from cyst to tumour. J Can Dent Investigation of chromosome 9q22.3-q31 DNA marker loss in
Assoc. 2008;74:165. odontogenic keratocysts. Eur J Cancer Part B: Oral Oncol.
5. White SC, Pharoah MJ. Cysts of the jaws. In: White SC, editor. Oral 1996;32(3):202–6.
Radiology: Principles and Interpretation. St. Louis: Mosby Elsevier; 16. Barreto DC, Gomez RS, Bale AE, Boson WL, Marco LD. PTCH Gene
2009. p. 395–402. Mutations in Odontogenic Keratocysts. J Dent Res. 2000;79(6):1418–
6. Forssell K, Forssell H, Kahnberg KE. Recurrence of keratocysts: 22.
a long-term follow-up study. Int J Oral Maxillofac Surg. 17. Muzio LL, Staibano S, Pannone G, Bucci P, Nocini PF, Bucci E, et al.
1988;17(1):25–8. Expression of Cell Cycle and Apoptosis-related Proteins in Sporadic
7. Chirapathomsakul D, Sastravaha P, Jansisyanont P. A review of Odontogenic Keratocysts and Odontogenic Keratocysts Associated
odontogenic keratocysts and the behavior of recurrences. Oral Surg, with the Nevoid Basal Cell Carcinoma Syndrome. J Dent Res.
Oral Med, Oral Pathol, Oral Radiol, Endodontol. 2006;101:5–9. 1999;78(7):1345–53.
8. Passi D, Singhal D, Singh M, Mishra V, Panwar Y, Sahni A. 18. Pindborg JJ, Philipsen HP, Henriksen J. Studies on odontogenic cyst
Odontogenic Keratocyst (OKC) or KeratocysticOdontogenic Tumor epithelium. In: Sognnaes RF, editor. Fundamentals of Keratinization.
(KCOT)-Journey of OKC from cyst to tumor to cyst again: a American Association of the Advancement of Science; 1962. p. 151–
comprehensive review with recent updates on who classification. Int J 60.
Curr Res. 2017;9:54080–6.
9. Madras J, Lapointe H. Keratocystic odontogenic tumor: Reclassifi
cation of the odontogenic keratocyst from cyst to tumor. JCan Dent Author biography
Assoc Nation. 2008;74:165.
10. Rajendran R, Sivapathasundaram S. Shafer’s Textbook of Oral Nalini Tomar Senior Lecturer
Pathology. 6th ed. New Delhi: Elsevier Publication; 2009.
11. González-Alva P, Tanaka A, Oku Y, Yoshizawa D, Itoh S, Sakashita Kuldeep Singh Bhadauria Consultant Pathologist
H, et al. Keratocystic odontogenic tumor: a retrospective study of 183
cases. J Oral Sci. 2008;50(2):205–12.
12. Ahlfors E, Larsson Å, Sjögren S. The odontogenic keratocyst: A
benign cystic tumor? J Oral Maxillofac Surg. 1984;42(1):10–9. Cite this article: Tomar N, Bhadauria KS. Odontogenic keratocyst-
13. Knudson AG. Mutation and Cancer: Statistical Study of Mimicking residual cyst in maxilla. J Oral Med, Oral Surg, Oral Pathol,
Retinoblastoma. Proc Natl Acad Sci. 1971;68(4):820–3. Oral Radiol 2020;6(2):98-105.