International Journal of Medical and Dental Case Reports (2016), Article ID 010416, 4 Pages

CASE REPORT

Keratocystic odontogenic tumor  - A case report and review

of literature
Akshay Shetty1, Tejavathi Nagaraj2, Keerthi Irugu2, Saurabh Kale1
1
Department of Oral Maxillofacial Surgery, Sri Rajiv Gandhi College of Dental Sciences and Hospital, Cholanagar, Bengaluru, Karnataka, India, 2Department of
Oral Medicine and Radiology, Sri Rajiv Gandhi College of Dental Sciences and Hospital, Cholanagar, Bengaluru, Karnataka, India

Correspondence Abstract
Dr. Keerthi Irugu, Department of Oral The odontogenic keratocyst (OKC) is categorized as one of the developmental
Medicine and Radiology, Sri Rajiv Gandhi
epithelial cyst which accounts for 11% of all cystic jaw lesions. The most peculiar clinical
Dental College and Hospital, Cholanagar,
feature of OKC tumor is its tendency to occur frequently. This could be the reason for
RT Nagar, Bengaluru - 560 032, Karnataka,
India. Tel.: +91-9916903205.
suggesting a variety of treatment modalities for this cyst. Many of the surgeons suggest
Email: keerthi.irugu@gmail.com more aggressive treatment modalities such as resection of the jaws. In this case, we have
done a surgical approach in managing a patient with a large OKC of the mandible.
Received 02 Feb 2016;
Keywords: Developmental, recurrence, resection
Accepted 15 Apr 2016

doi: 10.15713/ins.ijmdcr.48

How to cite the article:

Shetty A, Nagaraj T, Irugu K, Kale S.
Keratocystic odontogenic tumour - A case
report and review of literature. Int J Med Dent
Case Rep 2016;2:1-4.

Introduction Case Report

The identification and description of odontogenic
keratocyst (OKC) tumors were done in 1876 as odontogenic
developmental cysts of epithelial origin and later in 1956 it was
characterized by Phillipsen.[1,2] Pindborg and Hansen in 1962(2)
mentioned the histologic criteria required to diagnose OKC.
As the origin of this cyst was thought to be the primordium of
the tooth the initial terminology was given as “primordial cyst.”
Later, in 1992, the World Health Organization preferred the
term “odontogenic keratocyst” for such cysts with a keratinized
lining in the histologic grading of odontogenic tumors.[3] OKC
is best known for its repetitive occurring potential, invasive
nature, and its sporadic association with the nevoid basal cell
carcinoma syndrome (NBCCS). Three histologic forms were
known initially which includes an orthokeratinized variant, a
parakeratinized variant, and combination of these two variants.
The classification of the orthokeratinized variant as a discrete
clinical entity of “orthokeratinized odontogenic cyst” is due to its
less invasive clinical nature and repetitive pattern of this variant.
The percentage of OKCs versus other cysts of the jaws was
given by different authors are as follows;[4] Hjorting-Hansen
et al. (1969) and Toller (1972), - 11% Brannon (1976) and
Payne (1972) - 9%, Pindborg and Hansen (1963) - 7%.
A 62-year-old patient came to the Department of Oral Medicine
and Maxillofacial Radiology with a chief complaint of pain in
lower left back tooth region since 1 and ½ months. The pain was
persistent, throbbing, dull and continuous which aggravated
during lying down and relieved on taking medications. Medical
and allergy history were not significant. The patient had the
habit of bidi smoking since 20 years around 10-15 bidis/day.
Extraoral examination revealed no gross facial
asymmetry [Figure 1a]. On palpation, a bony hard swelling was
present extending 2 cm away from the distal aspect of 36 which
was not tender.
Intraoral examination of soft tissue showed a healing
extraction socket site present irt 38 with normal appearance of
the overlying alveolar mucosa. On palpation overlying mucosa
appeared smooth, firmly adhere to underlying periosteum with
no evidence of bony expansion on buccal and lingual side of 38.
The tooth 37 was tender on percussion [Figure 1b].
Examination of the hard tissue showed the presence of
stains and calculus and generalized attrition. With the above
findings of the site of lesion and history a provisional diagnosis
of keratocystic odontogenic tumor (KCOT) of the left mandible
was given.

1
Shetty, et al. Keratocystic odontogenic tumor - A case report and review of literature

An orthopantomogram (OPG) was taken which revealed Discussion

missing 38 and presence of well-defined multilocular
The OKC is a unique and prevalent clinical and histologic lesion
radiolucency on the left mandibular region measuring about
with aggressive nature. It usually arises in the dental lamina, but
8 cm × 4 cm extending from mesial aspect of 34 to sigmoid
some suggest a probable origin from basal cell component.[5]
region anteroposteriorly and superiorly from body of ramus to
About 70% or more cases involve the mandible, especially in the
lower border of the mandible inferiorly with loss of lamina dura
third molar, angle, and ramus areas. Next, most common site of
irt 36, 37 [Figure 2].
occurrence is the maxillary third molar followed by mandibular
Computed tomography scan was done to determine the
premolar and maxillary canine region.[2,6]
exact location of the borders of the tumor [Figure 3].
It accounts for 10% of all jaw cysts and shows the prevalence
Fine needle aspiration cytology was done, and sample
in wide age groups. Symptoms include pain, swelling and
showed clear scanty fluid which was watery in consistency.
drainage, especially with larger lesions and half of all these
Histopathological report of the H and E stained cytological
lesions are noticed as incidental radiographic findings.[7]
smear showed few cells with hyperchromatic nucleus in
Due to its potential to grow within the medullary bone, they
clusters, few keratin, and few inflammatory cells suggestive of
keratin-filled lesion.
Biopsy from multiple hard and soft tissue lining was taken
which showed parakeratinized stratified squamous cystic
lining with surface corrugation and flat interface of underlying
connective tissue wall with the separation of the epithelium from
the capsule showing tombstone appearance [Figure 3].
After relevant investigations, a final diagnosis of OKC was
given.
Treatment included surgical excision of the lesion with a wide
margin of 1 cm, and Carnoy’s solution applied after excision.
Reconstruction was done using Free Fibula osteocutaneous
flap [Figure 4]. An OPG was taken postoperatively [Figure 5].

Figure 3: Biopsy specimen and computed tomography image

showing extent of lesion

a b
Figure 1: (a) Extraoral examination revealed no gross facial
asymmetry. (b) Intraoral picture

Figure 4: Carnoy’s solution application and fibula reconstruction

Figure 2: Panoramic radiograph well defined multilocular

radiolucency on the left mandibular region Figure 5: Postoperative radiograph and extraoral picture

2
Keratocystic odontogenic tumor - A case report and review of literature
tend to become extremely large with undue clinical signs or
symptoms.
Radiological examination of most of the cases reveals a
well-defined radiolucency with thin corticated margins with the
majority of these being unilocular, but larger lesions present as
multilocular lesions.[7,4]
Various percentage of radiologic patterns of OKCs as shown
in Table 1.
20-40% of OKCs are usually found with an unerupted tooth
and bear a resemblance to a dentigerous cyst. Root resorption is
relatively rare with OKCs.[8]
The OKC shows highly distinct histopathological features
with uniform cyst lining, wavy parakeratin formation,
hyperchromatic, and palisaded basal cells and a flat interface
between the epithelium and underlying connective tissue wall.
When the cyst is inflamed, these classic microscopic features
are often completely lost, which can lead to an inappropriate
diagnosis. NBCCS (also known as Gorlin-Goltz syndrome or jaw
cyst-basal cell nevus-bifid rib syndrome) should be considered
when a patient presents with multiple OKCs. NBCCS is an
inherited genetic condition which occurs due to mutation of
the PTCH1 gene. Palmar and plantar pits, bifid ribs, multiple
basal cell cancers of the skin and calcified falx cerebri are the
other findings of this syndrome. The lesions found with NBCCS
are comparitively less aggressive than traditional basal cell
epithelioma, because of this the designation “nevoid,” or having
biologic behavior more similar to a nevus was mentioned.[6]
About 25-30% of OKC lesions tend to recur unlike most
other cysts of odontogenic origin, and most recurrent cases
are seen during the first 5-7 years after treatment.[9] Up to
date, there are no practical instruments or techniques available
to surgeons to help predict the recurrence of the lesions.
The probable etiology for the cause of recurrence includes
inappropriate expulsion of the original cyst lining, development
of a new lesion from residual epithelial islands and or genotypic
variations between lesions.[9] Because of these probable
reasons, the treatment protocol for OKCs is still controversial.
The clinician should treat these lesions in such way to reduce
both the risk of recurrence and morbidity of the patient.
Treatment
Treatment modalities for OKC commonly used with a
conservative approach for large lesions are marsupialization and
Table 1: Various percentage of radiologic patterns of odontogenic keratocysts
Reported radiologic patterns of odontogenic keratocysts
Author names Unilocular
Poorly defined  (%) Unilocular  (%)
Brannon (1977) 3 62
Browne (1970) - 57
Isberg-Holm (1977) - 57
Park and Kim (1985) - 73
Shetty, et al.
decompression. They are preferred to preserve bone, teeth, and
preventing damage to other vital structures, and also for decreasing
the chances of pathologic fracture. They are the treatment
modalities recommended for pediatric and poor surgical patients.
The principle behind these procedures is to decrease the cystic
osmotic pressure by exposing it to the surrounding oral cavity. It
is helpful in a bone deposition at the periphery of the lesion and
also a progressive reduction in the cyst size.[10] The most reliable
and single-step procedure which leads to ultimate complete
resolution of the cystic lesion is marsupialization.
Two-step procedure in the management of OKC’s is
decompression which involves the placement of a surgical
drainage tube, the following enucleation after the cyst has
reduced to a manageable size.
Enucleation is the complete and intact removal of a cystic
lesion by surgically husking it from the surrounding tissues.
17-56% of the recurrence rate of enucleation alone has been
reported.[11] Because of this, many surgeons prefer a combination
of enucleation and adjunctive therapies to eliminate any residual
cyst lining and islands within the cyst wall.
Adjunctive therapy includes the application of Carnoy’s
solution which destroys cyst remnants by using chemical
cautery. 18% reduction in the recurrence potential when
both combination of enucleation with adjunctive treatment
performed.[11]
The surgical approach for OKC’s is resection. It is defined
as the surgical excision of a section of the involved maxillary
or mandibular jaws. If a rim of uninvolved bone is left behind
it is known as marginal resection, whereas segmental resection
involves the removal of a complete jaw without any continuity
to the adjacent structures. This procedure is considered as an
aggressive treatment modality which most commonly helps
in eliminating the chances of recurrence, but somehow it is
regarded as a radical approach in the treatment of OKC which
often leads to the morbidity of affected patients.
An ample amount of research is going on over the past few
decades regarding the genetic and molecular factors which are
involved in the pathogenesis of OKCs. The cyst was previously
considered as developmental in origin, but now based on
available evidence suggests that it should be included as a benign
neoplasm.[12,13]
Due to the presence of higher levels of the biologic
proliferation markers Ki67, proliferating cell nuclear antigen
in OKCs compared to other dentigerous cysts and radicular
Multilocular
With scalloped border Bilocular Multilocular  (%) Total  (Cases)
- 10 23 52
21 - 23 90
33 - 10 -
27 - - 22
3
Shetty, et al.
cysts, indicating the proliferation of the lining epithelium in the
pathogenesis of OKC.[14,15]
The WHO in 2005 in view of its evidence of neoplastic
origin officially assorted the term OKC as KCOT.[16] This
term KCOT is the most current and precise nomenclature, but
practically these both terms OKC and KCOT are synonyms and
indistinguishable.
Most of the oral pathologists, including those at the
University of Tennessee still using the nomenclature OKC in
biopsy reports to decrease dilemma among the professionals
who may not be used to the term KCOT. Currently, there
are no supplementary advanced laboratory techniques
available associated in the diagnosis of OKC except the
traditional routinely used hematoxylin and eosin stained
microscopic slides. Perhaps continued research in this regard
will successfully help in identifying an accurate diagnostic
tool which aids pathologists and surgeons in sorting out the
aggressive and recurrent lesions and thereby guiding the
treatment outcome and prognosis.[17]
Summary
The present case of OKC was noticed in a 62- year-old male
patient on the left mandible showing the clinical and radiographic
presentation, diagnosis, treatment, and follow-up. Research
is still going on appropriate treatment modalities for OKCs
because of it is genetic and molecular basis of pathogenesis.
Surgeons should thoroughly examine each case individually and
provide with different treatment options to the patients.
References
1. Greenberg  MS, Glick  M, Ship  JA. Burket’s Oral Medicine.
11th ed. Hamilton (Canada): BC Decker; 2008.
2. Khalam AS, Zacariah RK. Under diagnosis of an odontogenic
keratocyst -  A controversial lesion. IOSR J Dent Med Sci
2012;2 (5):37-40.
3. Nayak UA, Rajeshkumar BP, Ahmed J, Baliga MJ. A review of
odontogenic keratocyst with a report of unusual occurrence in
the maxilla. Indian J Dent Res 2002;13:183-6.
Keratocystic odontogenic tumor - A case report and review of literature
4. Langland OE, Langlais RP. Diagnostic Imaging of Jaws. 2nd ed.
Baltimore: Williams and Wilkens; 1995.
5. Neville BW. Oral and Maxillofacial Pathology. 3rd ed. St. Louis,
MO: Elsevier/Saunders; 2009.
6. Rajendran R, Sivapathasundaram B. Shafer’s Textbook of Oral
Pathology. 5th ed. USA: Elsevier; 2006.
7. Boffano  P, Ruga  E, Gallesio  C. Keratocystic odontogenic
tumor  (odontogenic keratocyst): Preliminary retrospective
review of epidemiologic, clinical, and radiologic features of
261 lesions from University of Turin. J  Oral Maxillofac Surg
2010;68:2994-9.
8. White  C, Pharoah  MJ. Oral Radiology - Principles and
Interpretation. 7th ed. St Louis (MO): Mosby/Elsevier; 2014.
9. Mendes RA, Carvalho JF, van der Waal I. Characterization and
management of the keratocystic odontogenic tumor in relation
to its histopathological and biological features. Oral Oncol
2010;46:219-25.
10. Almeida P Jr, Cardoso Lde  C, Garcia IR Jr, Magro-Filho  O,
Luvizuto  ER, Felipini  RC. Conservative approach to the
treatment of keratocystic odontogenic tumor. J  Dent
Child (Chic) 2010;77:135-9.
11. Shear M. The aggressive nature of the odontogenic keratocyst:
Is it a benign cystic neoplasm? Part  1. Clinical and early
experimental evidence of aggressive behaviour. Oral Oncol
2002;38:219-26.
12. Henley J, Summerlin DJ, Tomich C, Zhang S, Cheng L. Molecular
evidence supporting the neoplastic nature of odontogenic
keratocyst: A laser capture microdissection study of 15  cases.
Histopathology 2005;47:582-6.
13. Agaram  NP, Collins  BM, Barnes  L, Lomago  D, Aldeeb  D,
Swalsky  P, et  al. Molecular analysis to demonstrate that
odontogenic keratocysts are neoplastic. Arch Pathol Lab Med
2004;128:313-7.
14. Mendes RA, Carvalho JF, van der Waal I. Biological pathways
involved in the aggressive behavior of the keratocystic
odontogenic tumor and possible implications for molecular
oriented treatment - An overview. Oral Oncol 2010;46:19-24.
15. Li TJ. The odontogenic keratocyst: A cyst, or a cystic neoplasm?
J Dent Res 2011;90:133-42.
16. Sabin S, Anoop K, Anoop M, Meera M. Keratocystic odontogenic
tumor of the mandible - A case report. Austin J Dent 2015;2:1-2.
17. Gomez  RS, De Marco  L. Possible molecular approach to the
treatment of odontogenic keratocyst. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod 2005;99:527-8.