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ODONTOGENIC KERATOCYST - RECENT

UPDATES IN MANAGEMENT

Dr. Godwin Alex Kiruba J, Dr. Pradeep Devadoss, Dr. Nikita Ravi*

Department of Oral and Maxillofacial Surgery,

*Department of periodontology and Implantology,

Faculty of Dentistry, Meenakshi Ammal Dental College,

Meenakshi Academy of Higher Education and Research, Chennai, Tamilnadu, India.

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ABSTRACT

Odontogenic keratocyst is one of the most aggressive

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odontogenic cysts arising from odontogenic epithelium.

It can become quite large because of its ability

for significant expansion, extension into adjacent

tissues and rapid growth. Thus they are often locally

destructive and tend to recur. The goals of treatment

should eliminate the potential recurrence and minimize

the surgical morbidity.

KEY WORDS: Keratocystic odontogenic tumor,

Odontogenic keratocyst, Basal nevus syndrome,

Marsupialization, Vismodegib

Address for correspondence:

Dr. Pradeep Devadoss, MDS.,

Professor, Department of Oral and

Maxillofacial Surgery,

Faculty of Dentistry, Meenakshi Ammal

Dental College,

Meenakshi Academy of Higher Education

and Research, Chennai.

Received

: 04.11.2019

Accepted

: 17.12.2019

Published

: 27.12.2019

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JIDAM/Volume:6/Issue:4/Pages 154 - 157/October - December 2019

Godwin et al : Recent updates in management of odontogenic keratocyst

INTRODUCTION:

TREATMENT:

The term

“Odontogenic Keratocyst” was

The conventional treatment for Odontogenic

first suggested by Phillipsen in 1956¹. Historically,

keratocyst has been primarily, enucleation of the

the first reported case of a dental cyst was in 1774

lesion with peripheral ostectomy, followed by

by an anatomist, John Hunter. Since 1930s, many

chemical cauterization. Chemical cauterization was

researchers noted that dental cysts could be found

done using Carnoy`s solution which has chloroform

with keratinization in the lining of the cyst and

as a constituent. Due to the carcinogenic potential

keratin itself in the cyst contents. The general belief

of chloroform, its use has been discouraged and a

is that these cysts originate from remnants of the

Modified Carnoy`s solution was advocated. It lacked

dental lamina. But in many cases, a tooth is generally

the potency of Carnoy`s solution. Marsupialization,

present and, so, they are believed to originate from

Cryotherapy, Radical resection⁶ and Endoscopic

additional remnants of the lamina that are not

enucleation⁷ are also advocated based on clinical

involved in tooth formation. In a few cases, they may

criteria. With the advancements in research, many

arise from the oral mucosa, due to the presence of

newer trends have emerged in the management of

daughter cysts. The basal cell nevus syndrome or

this pathology. Ledderhof et al studied the usage

the Gorlin-Goltz Syndrome is a genetic condition

of topical 5- Fluorouracil, an anti-metabolite in the

that includes a triad of Odontogenic Keratocyst

place of modified Carnoy’s solution after enucleation

of the jaws, skeletal abnormalities like bifid ribs,

and peripheral ostectomy. There were no recurrences

frontal bossing, and calcification of the falx cerebri

or adverse effects in the follow up period and a

and cutaneous manifestations such as basal cell

significantly lower incidence of inferior alveolar

carcinomas and palmar pitting of the hands².

nerve paresthesia was observed. It was readily

available, had technical ease, had shorter operating

RADIOGRAPHIC FEATURES:

time, similar efficacy and had decreased morbidity

compared to modified Carnoy`s solution⁸. Cases of

Odontogenic

Keratocyst

present

Basal cell nevus syndrome are commonly associated

radiographically either as a unilocular or a

with mutations in the PTCH1 gene. The hedgehog

multilocular radiolucency, occurring mostly in the

pathway gets activated by binding of Sonic hedgehog

posterior mandible. Expansion of the buccal and

protein to its receptor, which diminishes the inhibitory

lingual plates occur late as it primarily tends to

effect of PTCH on Smoothened Protein (SMO), that

invade and spread along the marrow spaces³.

inturn activates an intracellular cascade which results

in the activation and nuclear translocation of Gli

PATHOLOGIC FEATURES:

family transcription factors. These receptors promote

cellular proliferation and survival.

The presence of keratin flakes or a protein

level of less than 4 g per 100 ml in the sample obtained

FUTURE PERSPECTIVE:

after aspiration biopsy are indicative of Odontogenic

Keratocyst⁴. Odontogenic Keratocyst has a higher

Vismodegib is a new drug, which is a selective

prevalence rate in the mandible than in the maxilla,

Hh pathway inhibitor blocks Hh signaling by binding

with a ratio of 3:1. In the mandible, it occurs more

to SMO and inhibiting the activation of downstream

often in the third molar area/ascending ramus when

Hh target genes. Vismodegib

(150mg) is orally

compared to the tooth-bearing area anterior to

administered once daily, is approved by the Food

the third molar with a ratio of 3:2⁵. There are two

and drug administration (FDA) in 2012. It should be

variants of this pathology based on their histological

administered for at least 11 months⁹. Ally et al found

appearance, one being Orthokeratinized and the

that Vismodegib can shrink some KCOTs in patients

other being Parakeratinized. Orthokeratinized OKC

with Basal Cell Naevus Syndrome, with a mean

have a lesser recurrence rate when compared with

size reduction of 50%. Two patients had complete

the Parakeratinized OKC.

resolution of 1 or more KCOTs, with no enlargement

of existing tumors, no recurrence in any case and no

development of new lesions¹⁰. It costs about $360 per

150mg tablet, so may work up to $131000 for 1 year

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JIDAM/Volume:6/Issue:4/Pages 154 - 157/October - December 2019

Godwin et al : Recent updates in management of odontogenic keratocyst

Fig 1- Site of action of Vismodegib

Schematic representation of hedgehog-signaling pathway: Hedgehog ligands bind to patched homolog

1, causing release of the suppression of smoothened by patched homolog 1. Smoothened interacts with

suppressor of fused, which promotes glioma-associated oncogene transcription factor. Constitutive

activation of smoothened protein plays a role in carcinogenesis. Vismodegib inhibits smoothened Protein

Jain et al reviewed Sonidegib, the second Hh

Basal Cell Nevus Syndrome may offer an alternative

signaling inhibitor approved by the FDA to treat Basal

to surgical patients. The obvious advantages of these

cell carcinoma following approval of the first SMO

treatment techniques are as follows:

antagonist Vismodegib in 2012. Sonidegib interacts

with SMO in the drug-binding pocket, where it acts as

• Eradication of the pathologic lesion

an antagonist, preventing downstream activation of

• Reduction of the potential recurrence

Hh pathway signaling¹¹(Fig 1). It costs upto $146000

for 1 year and can be given either orally or topically.

• Preservation of the continuity of the mandible,

Meticulous understanding of the pathogenesis of

thus maintaining jaw function and shape

odontogenic keratocyst, along with a proper follow-

up of the clinical trials of the recent advances, might

FINANCIAL SUPPORT AND

provide us a better prognosis in cases that can be

SPONSORSHIP:

treated without surgery and to improve the patient’s

overall quality of life.

Nil

CONCLUSION:

CONFLICTS OF INTEREST:

Odontogenic keratocyst is amongst the

There are no conflicts of interest.

numerous aggressive odontogenic cysts having

significant recurrence rate. Numerous surgical

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