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

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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

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

tend to become extremely large with undue clinical signs or decompression. They are preferred to preserve bone, teeth, and
symptoms. preventing damage to other vital structures, and also for decreasing
Radiological examination of most of the cases reveals a the chances of pathologic fracture. They are the treatment
well-defined radiolucency with thin corticated margins with the modalities recommended for pediatric and poor surgical patients.
majority of these being unilocular, but larger lesions present as The principle behind these procedures is to decrease the cystic
multilocular lesions.[7,4] osmotic pressure by exposing it to the surrounding oral cavity. It
Various percentage of radiologic patterns of OKCs as shown is helpful in a bone deposition at the periphery of the lesion and
in Table 1. also a progressive reduction in the cyst size.[10] The most reliable
20-40% of OKCs are usually found with an unerupted tooth and single-step procedure which leads to ultimate complete
and bear a resemblance to a dentigerous cyst. Root resorption is resolution of the cystic lesion is marsupialization.
relatively rare with OKCs.[8] Two-step procedure in the management of OKC’s is
The OKC shows highly distinct histopathological features decompression which involves the placement of a surgical
with uniform cyst lining, wavy parakeratin formation, drainage tube, the following enucleation after the cyst has
hyperchromatic, and palisaded basal cells and a flat interface reduced to a manageable size.
between the epithelium and underlying connective tissue wall. Enucleation is the complete and intact removal of a cystic
When the cyst is inflamed, these classic microscopic features lesion by surgically husking it from the surrounding tissues.
are often completely lost, which can lead to an inappropriate 17-56% of the recurrence rate of enucleation alone has been
diagnosis. NBCCS (also known as Gorlin-Goltz syndrome or jaw reported.[11] Because of this, many surgeons prefer a combination
cyst-basal cell nevus-bifid rib syndrome) should be considered of enucleation and adjunctive therapies to eliminate any residual
when a patient presents with multiple OKCs. NBCCS is an cyst lining and islands within the cyst wall.
inherited genetic condition which occurs due to mutation of Adjunctive therapy includes the application of Carnoy’s
the PTCH1 gene. Palmar and plantar pits, bifid ribs, multiple solution which destroys cyst remnants by using chemical
basal cell cancers of the skin and calcified falx cerebri are the cautery. 18% reduction in the recurrence potential when
other findings of this syndrome. The lesions found with NBCCS both combination of enucleation with adjunctive treatment
are comparitively less aggressive than traditional basal cell performed.[11]
epithelioma, because of this the designation “nevoid,” or having The surgical approach for OKC’s is resection. It is defined
biologic behavior more similar to a nevus was mentioned.[6] as the surgical excision of a section of the involved maxillary
About 25-30% of OKC lesions tend to recur unlike most or mandibular jaws. If a rim of uninvolved bone is left behind
other cysts of odontogenic origin, and most recurrent cases it is known as marginal resection, whereas segmental resection
are seen during the first 5-7 years after treatment.[9] Up to involves the removal of a complete jaw without any continuity
date, there are no practical instruments or techniques available to the adjacent structures. This procedure is considered as an
to surgeons to help predict the recurrence of the lesions. aggressive treatment modality which most commonly helps
The probable etiology for the cause of recurrence includes in eliminating the chances of recurrence, but somehow it is
inappropriate expulsion of the original cyst lining, development regarded as a radical approach in the treatment of OKC which
of a new lesion from residual epithelial islands and or genotypic often leads to the morbidity of affected patients.
variations between lesions.[9] Because of these probable An ample amount of research is going on over the past few
reasons, the treatment protocol for OKCs is still controversial. decades regarding the genetic and molecular factors which are
The clinician should treat these lesions in such way to reduce involved in the pathogenesis of OKCs. The cyst was previously
both the risk of recurrence and morbidity of the patient. considered as developmental in origin, but now based on
available evidence suggests that it should be included as a benign
neoplasm.[12,13]
Treatment
Due to the presence of higher levels of the biologic
Treatment modalities for OKC commonly used with a proliferation markers Ki67, proliferating cell nuclear antigen
conservative approach for large lesions are marsupialization and in OKCs compared to other dentigerous cysts and radicular

Table 1: Various percentage of radiologic patterns of odontogenic keratocysts

Reported radiologic patterns of odontogenic keratocysts
Author names Unilocular Multilocular
Poorly defined  (%) Unilocular  (%) With scalloped border Bilocular Multilocular  (%) Total  (Cases)
Brannon (1977) 3 62 - 10 23 52
Browne (1970) - 57 21 - 23 90
Isberg-Holm (1977) - 57 33 - 10 -
Park and Kim (1985) - 73 27 - - 22

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

cysts, indicating the proliferation of the lining epithelium in the 4. Langland OE, Langlais RP. Diagnostic Imaging of Jaws. 2nd ed.
pathogenesis of OKC.[14,15] Baltimore: Williams and Wilkens; 1995.
The WHO in 2005 in view of its evidence of neoplastic 5. Neville BW. Oral and Maxillofacial Pathology. 3rd ed. St. Louis,
origin officially assorted the term OKC as KCOT.[16] This MO: Elsevier/Saunders; 2009.
term KCOT is the most current and precise nomenclature, but 6. Rajendran R, Sivapathasundaram B. Shafer’s Textbook of Oral
Pathology. 5th ed. USA: Elsevier; 2006.
practically these both terms OKC and KCOT are synonyms and
7. Boffano  P, Ruga  E, Gallesio  C. Keratocystic odontogenic
indistinguishable.
tumor  (odontogenic keratocyst): Preliminary retrospective
Most of the oral pathologists, including those at the review of epidemiologic, clinical, and radiologic features of
University of Tennessee still using the nomenclature OKC in 261 lesions from University of Turin. J  Oral Maxillofac Surg
biopsy reports to decrease dilemma among the professionals 2010;68:2994-9.
who may not be used to the term KCOT. Currently, there 8. White  C, Pharoah  MJ. Oral Radiology - Principles and
are no supplementary advanced laboratory techniques Interpretation. 7th ed. St Louis (MO): Mosby/Elsevier; 2014.
available associated in the diagnosis of OKC except the 9. Mendes RA, Carvalho JF, van der Waal I. Characterization and
traditional routinely used hematoxylin and eosin stained management of the keratocystic odontogenic tumor in relation
microscopic slides. Perhaps continued research in this regard to its histopathological and biological features. Oral Oncol
will successfully help in identifying an accurate diagnostic 2010;46:219-25.
10. Almeida P Jr, Cardoso Lde  C, Garcia IR Jr, Magro-Filho  O,
tool which aids pathologists and surgeons in sorting out the
Luvizuto  ER, Felipini  RC. Conservative approach to the
aggressive and recurrent lesions and thereby guiding the
treatment of keratocystic odontogenic tumor. J  Dent
treatment outcome and prognosis.[17] 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
Summary
experimental evidence of aggressive behaviour. Oral Oncol
The present case of OKC was noticed in a 62- year-old male 2002;38:219-26.
patient on the left mandible showing the clinical and radiographic 12. Henley J, Summerlin DJ, Tomich C, Zhang S, Cheng L. Molecular
presentation, diagnosis, treatment, and follow-up. Research evidence supporting the neoplastic nature of odontogenic
is still going on appropriate treatment modalities for OKCs keratocyst: A laser capture microdissection study of 15  cases.
Histopathology 2005;47:582-6.
because of it is genetic and molecular basis of pathogenesis.
13. Agaram  NP, Collins  BM, Barnes  L, Lomago  D, Aldeeb  D,
Surgeons should thoroughly examine each case individually and Swalsky  P, et  al. Molecular analysis to demonstrate that
provide with different treatment options to the patients. 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
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