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CASE REPORT PEER REVIEWED | OPEN ACCESS
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Non-syndromic multiple keratocystic odontogenic tumors: An
arduous challenge for oral and maxillofacial specialists
GV Reddy, M. Haranadha Reddy, Komali Garlapati,
Rembers Anusha, Pancha Venkat Bhagirath, Raj Kumar Badam,
K. Sravan Kumar Reddy
ABSTRACT
Introduction: Keratocystic odontogenic tumor (KOT) is a common developmental
odontogenic cyst affecting the maxillofacial region. Multiple odontogenic keratocysts
(OKCs) are usually seen in association with nevoid basal cell carcinoma syndrome but
approximately only 5% of patients with keratocystic odontogenic tumor have multiple cysts
without concomitant syndromic presentation. Only a few cases have been reported till date.
Case Report: This report emphasizes a unique case of a young 14-year-old female suffering
since an early age and the role of multidisciplinary approach in the diagnosis and management
of a case of multiple keratocystic odontogenic tumors in a non-syndromic patient.
Conclusion: The non-syndromic KCOTs are linked to the expression of a characteristic gene.
They are associated with severe morbidity in the younger age group due to their multiple
involvement of the jaws but their recurrence rate is less compared to that of syndromic
type. The diagnosis and management of these tumors mandates multidisciplinary approach
which can instill condence and improve quality of life of the patients.
(This page in not part of the published article.)
International Journal of Case Reports and Images, Vol. 7 No. 6, June 2016. ISSN – [0976-3198]
Int J Case Rep Images 2016;7(6):360–364.
www.ijcasereportsandimages.com
Reddy et al. 360
CASE REPORT OPEN ACCESS
Non-syndromic multiple keratocystic odontogenic tumors:
An arduous challenge for oral and maxillofacial specialists
GV Reddy, M. Haranadha Reddy, Komali Garlapati,
Rembers Anusha, Pancha Venkat Bhagirath, Raj Kumar Badam,
K. Sravan Kumar Reddy
ABSTRACT
Introduction: Keratocystic odontogenic tumor
(KOT) is a common developmental odontogenic
cyst affecting the maxillofacial region. Multiple
odontogenic keratocysts (OKCs) are usually
GV Reddy1, M. Haranadha Reddy2, Komali Garlapati3,
Rembers Anusha4, Pancha Venkat Bhagirath5, Raj Kumar
Badam6, K. Sravan Kumar Reddy7
Affiliations: 1MDS, Professor & Head of the Department,
Oral and maxillofacial Surgery, Panineeya Mahavidyalaya
Institute of Dental Sciences and Research Centre,
Hyderabad,Telangana, India; 2MDS, Professor, Oral and
Maxillofacial Surgery, Panineeya Mahavidyalaya Institute
of Dental Sciences and Research Centre, Hyderabad,
Telangana, India; 3MDS, Professor, Oral Medicine and
Radiology, Panineeya Mahavidyalaya Institute of Dental
Sciences and Research Centre, Hyderabad, Telangana, India;
4BDS, Post Graduate Student, Oral Medicine and Radiology,
Panineeya Mahavidyalaya Institute of Dental Sciences and
Research Centre, Hyderabad, Telangana, India; 5MDS,
Professor & Head of the Department, Oral and Maxillofacial
Pathology, Panineeya Mahavidyalaya Institute of Dental
Sciences and Research Centre, Hyderabad, Telangana,
India; 6MDS, Reader, Oral Medicine and Radiology,
Panineeya Mahavidyalaya Institute of Dental Sciences and
Research Centre, Hyderabad, Telangana, India; 7BDS, Post
Graduate Student, Oral and Maxillofacial Surgery, Panineeya
Mahavidyalaya Institute of Dental Sciences and Research
Centre, Hyderabad, Telangana, India.
Corresponding Author: Dr. Rembers Anusha, Department
of Oral Medicine and Radiology, Panineeya Mahavidyalaya
Institute of Dental Sciences and Research Centre, Road
No. 5, Kamala Nagar, Dilsukhnagar, Hyderabad - 500 060,
Telangana, India; Email: rembersanusha30@gmail.com
Received: 09 January 2016
Accepted: 02 March 2016
Published: 01 June 2016
seen in association with nevoid basal cell
carcinoma syndrome but approximately only
5% of patients with keratocystic odontogenic
tumor have multiple cysts without concomitant
syndromic presentation. Only a few cases have
been reported till date. Case Report: This report
emphasizes a unique case of a young 14-year-old
female suffering since an early age and the role of
multidisciplinary approach in the diagnosis and
management of a case of multiple keratocystic
odontogenic tumors in a non-syndromic patient.
Conclusion: The non-syndromic KCOTs are
linked to the expression of a characteristic gene.
They are associated with severe morbidity in
the younger age group due to their multiple
involvement of the jaws but their recurrence
rate is less compared to that of syndromic type.
The diagnosis and management of these tumors
mandates multidisciplinary approach which can
instill confidence and improve quality of life of
the patients.
Keywords: Keratocystic odontogenic tumor, Be-
nign neoplasm, Gorlin –Goltz syndrome, Tumor
suppressor gene
How to cite this article
Reddy GV, Reddy MH, Garlapati K, Anusha R,
Bhagirath PV, RK Badam, Reddy KSK. Non-syndromic
multiple keratocystic odontogenic tumors: An arduous
challenge for oral and maxillofacial specialists. Int J
Case Rep Images 2016;7(6):360–364.
Article ID: Z01201606CR10652GR
*********
doi:10.5348/ijcri-201664-CR-10652
CASE REPORT PEER REviEwEd | OPEN ACCESS
International Journal of Case Reports and Images, Vol. 7 No. 6, June 2016. ISSN – [0976-3198]
Int J Case Rep Images 2016;7(6):360–364.
www.ijcasereportsandimages.com
Reddy et al. 361
INTRODUCTION
Keratocystic odontogenic tumor (KCOT/KOT) is a
developmental cyst derived from the enamel organ or
dental lamina. The definition “odontogenic keratocyst”
was first proposed by Philipsen in 1956 [1]. The KCOTs
are the most common form of cystic lesions affecting
the maxillofacial region, with an incidence rate of about
12–14% of all odontogenic cysts, more frequent in
males (M/F 2:1) [2]. Multiple KCOTs are associated
with syndromes such as Nevoid basal cell carcinoma
syndrome (NBCCS) /Gorlin–Goltz syndrome/ Bifid rib
syndrome) usually in younger patients [3, 4]. Occurrence
of multiple KCOT is rare and to date only a few cases have
been reported in the literature. We report a rare case of
multiple KOTs in a non-syndromic patient associated
with impacted/partially erupted teeth.
CASE REPORT
A 14-year-old female patient reported with a chief
complaint of swelling in the right side of face (Figure 1A)
since four months with a history of slowly progressing
swelling.
Her past history revealed that she was a diagnosed and
surgically operated case of dentigerous cyst in relation to
left deciduous maxillary teeth (C, D) at 6 years of age. At
the age of 10 years, she was suspected for re-infection of
the cyst with intra oral sinus opening and was advised
an orthopantomogram (OPG) which revealed multiple
radiolucencies involving maxilla and mandible (Figure
2A). Her personal history revealed mixed diet with no
deleterious habits and family history was not contributory
and there was no history of consanguineous marriage of
her parents.
On extraoral examination, her face was asymmetrical
due to diffuse swelling measuring 4x4 cm approximately
in the right middle third of face with involvement of right
ala of nose and upper lip associated with obliteration of
nasolabial fold (Figure 1A) and it was non-tender, firm
to hard in consistency with no local rise of temperature
on palpation. Temporomandibular joint examination
revealed tenderness on palpation on right side while
opening with adequate mouth opening of 35 mm.
Intraoral examination revealed missing 13, 25, 37 and an
oval shaped swelling measuring 4x2 cm approximately
in relation to right maxillary teeth (Figure 1B). It was
soft in consistency, fluctuant, compressible, and tender.
A panoramic radiograph was taken which revealed well
defined unilocular multiple radiolucencies bilaterally in
maxilla and mandible (Figure 2) and the radiolucencies
are described in (Table 1).
The patient was further evaluated to rule out any
syndrome due to the presence of multiple cystic lesions.
The patient’s chest (Figure 3) and skull radiographs
were unremarkable. Dermatological examination did
not reveal any cutaneous abnormalities like palmar
and plantar defects. Hematologic investigations were
within normal limits. Computed tomography scan of
maxillofacial region with axial (Figure 4A) and coronal
(Figure 4B–C) sections revealed multiple hypodense
lesions in the maxilla and mandible.
Aspiration of the cystic lesions showed white cheesy
keratin like material. Incisional biopsy was done in
relation to right and left maxilla and mandible and was
subjected to histopathological examination which was
suggestive of multiple KOTs. Under general anesthesia,
four lesions were enucleated (Figure 5A–B) followed by
chemical cauterization (Carnoy’s solution) and tissue
specimens were sent for histopathologic examination
(Figure 6) which revealed “Parakeratinized Odontogenic
Keratocyst” from all four quadrants which were of uniform
epithelial lining 6–8 cells thick lacking rete ridges. The
lumen was filled with keratin, cholesterol clefts and
hyaline bodies and the connective tissue wall show 3–4
micro cysts, cholesterol clefts and inflammatory cells at
few areas.
Follow-up was done on 1st, 2nd and 3rd months
postoperatively. The postoperative period was uneventful
without any complications. Patient is asymptomatic since
then without any complaints.
DISCUSSION
The KOT is a common developmental odontogenic
cyst and its biologic behavior is similar to a benign
neoplasm [5]. It occurs at any age with peak incidence
during the second and third decades, with a slight male
predominance [6]. In 25–40% cases, involvement of
unerupted tooth has been reported [5]. Brahnon in his
analysis of clinical features of 312 cases of OKC found that,
5.8% of patients with multiple OKC had no other features
of syndrome [7].The KOT is locally destructive and
recurrence rate is very high where published recurrence
rates for keratocystic odontogenic tumors range from 5%
to about 70%. The recurrence rate of KOT associated with
NBCC is about 82% whereas for solitary KOT is ranging
between 2.5% and 62.5% [6].
PTCH (patched), a tumor suppressor gene involved
in both syndrome associated and sporadic KOTs, occurs
on chromosome 9q22.3 – q31. Syndromes associated
with multiple KOTs are Gorlin–Goltz syndrome/NBCCS,
oro-facial-digital syndrome, Noonan syndrome, Ehler
danlos syndrome [5]. There is no specific laboratory
test to diagnose NBCCS, although the diagnosis is made
clinically using the criteria suggested by Evans et al. [6].
Evans et al. first published major and minor criteria
for diagnosis of Gorlin–Goltz syndrome, later modified
by Kimonis et al. and according to them the positive
diagnosis of Gorlin–Goltz syndrome is when two major
or one major and two minor criteria are satisfied [6,7].
International Journal of Case Reports and Images, Vol. 7 No. 6, June 2016. ISSN – [0976-3198]
Int J Case Rep Images 2016;7(6):360–364.
www.ijcasereportsandimages.com
Reddy et al. 362
The major criteria are:
• Multiple Basal cell carcinomas (BCCs) or one
occurring under the age of 20 years
• Histologically proven KOTs of the jaws
• Palmar or plantar pits (three or more)
• Bilamellar calcification of the falx cerebri
• Bifid, fused or markedly splayed ribs
• First degree relative with NBCCS
Figure 1: A 14-year-old female girl presenting with asymmetrical
face: (A) Clinical photograph of extraoral frontal view showing
swelling on right of the face, (B) Clinical Intraoral photograph
showing an oval shaped swelling measuring approximately 4x2
cm in size.
Figure 2: (A, B) Orthopantomograph showing large multiple
radiolucent lesions in maxilla and mandible (Figure A 10 years
of age).
Figure 3: Chest posteroanterior view reveals normal anatomic
findings.
Figure 4: Computed tomography (CT) scans showing lesions
in four quadrants (A) Axial section of CT scan showing well
defined osteolytic lesion, predominantly hypodense measuring
approximately 1x2 cm involving alveolar process of left maxilla
in relation to 27.
(B) Coronal section of CT scan showing well defined osteolytic
lesion, predominantly hypodense measuring approximately
4x4 cm involving alveolar and palatine process of right maxilla
with impacted13 displaced superiorly, (C) Coronal section of
CT scan showing well-defined osteolytic lesion, predominantly
hypodense measuring approximately 2x2 cm involving left
sided body and ramus of mandible in relation to impacted 37.
Figure 5: Excision of lesions (A) Bony cavity after cyst
enucleation in the right ramus region, (B) Bony cavity after cyst
enucleation in the right anterior maxilla.
Table 1: Showing region, extent and appearance of large
multiple radiolucent lesions in maxilla and mandible of
orthopantomograph
Region Radiographic
appearance
Approximate
Size
Mesial of 11 to
distal of 15
Well-defined large
unilocular radiolucency
with corticated border
in relation to vertically
impacted impacted 13
4x5 cm
Mesial of 25 to
distal 27
Non-homogenous density
of bone in relation to
root apices of 23, 24, 26,
Missing 25 and partially
erupted 27 (surgical scar)
1.5x2 cm
Mesial of 36 to
ramus region of
mandible
Well-defined unilocular
radiolucency with
impacted 37
5x3 cm
Distal of 47 to
ramus region of
mandible
Well-defined unilocular
radiolucency with 47
5x4 cm
International Journal of Case Reports and Images, Vol. 7 No. 6, June 2016. ISSN – [0976-3198]
Int J Case Rep Images 2016;7(6):360–364.
www.ijcasereportsandimages.com
Reddy et al. 363
The minor criteria are:
• Macrocephaly (adjusted for height)
• Congenital malformation: Cleft lip or palate,
frontal bossing, coarse face, moderate or severe
hypertelorism
• Other skeletal abnormalities: Sprengel deformity,
marked pectus deformity, marked syndactyly of the
digits
• Radiological abnormalities: Bridging of the sella
turcica, vertebral anomalies such as hemivertebrae,
fusion or elongation of the vertebral bodies,
modeling defects of the hands and feet or flame
shaped hands or feet
• Ovarian fibroma
• Medulloblastoma
However, there may be variations in the major
diagnostic criteria for NBCCS in some populations due
to genetic and geographic differences [8]. Our patient
was apparently healthy and did not meet any of these
diagnostic criteria for NBCCS, such as pits on the palms
of the hands or soles of the feet, multiple basal cell skin
cancers, skeletal (bone) changes, calcium deposits in the
brain and developmental disability.
Histopathological examination in our case revealed
parakeratinized stratified squamous epithelium with
absence of rete pegs and palisaded basal cell layer,
giving an appearance of tombstone or picket fence. The
connective tissue revealed multiple daughter cysts and
cystic lumen revealed keratin, giving a picture of KOT.
Treatments are normally classified as conservative or
aggressive. Conservative treatment modalities include
simple enucleation, with or without curettage, or
marsupialization [8]. Aggressive treatment modalities
includes peripheral ostectomy, chemical curettage with
carnoy’s solution, cryotherapy, or electrocautery and
resection [9]. The goal is to choose the treatment modality
that Carries the lowest risk of recurrence and the least
morbidity. Voorsmit et al. [9] (1981) have observed a
reduction in recurrence rate if enucleation followed by
application of Carnoy’s solution (2.5%) when compared
with enucleation alone (13.5%). Therefore, enucleation
followed by application of Carnoy’s solution can result in
a reasonably low recurrence rate with less morbidity when
compared to other treatment modalities. Kuroyanagi et
al. suggested the presence of Ki-67 expression in OKC,
which might be helpful for considering the alternative
surgical procedure to avoid recurrence and might be
used as a prognostic indicator. In recent studies, the
hypothesis that suppression of sonic hedgehog (SHH)
signaling pathway might be effective for the treatment of
OKC [10].
CONCLUSION
“These lesions take a giant leap in its stride”. It is the
responsibility of the oral and maxillofacial specialists to
do a comprehensive clinical examination and necessary
investigations to not only diagnose but also rule out any
associated syndromes and provide apt treatment. Since
partial expression of the gene can result in non-syndromic
multiple KOTs, the patient must be referred to a clinical
geneticist for counseling. Long-term follow-up after
treatment must be performed to detect any other features
associated with NBCCS, a tendency for multiplicity and
recurrence. Hence, apart from surgery even gene therapy
can play a significant role in such patients.
*********
Author Contributions
GV Reddy – Substantial contributions to conception and
design, Acquisition of data, Analysis and interpretation
of data, Drafting the article, Revising it critically for
important intellectual content, Final approval of the
version to be published
M. Haranadha Reddy– Analysis and interpretation
of data, Drafting the article, Revising it critically for
important intellectual content, Final approval of the
version to be published
Komali Garlapati – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Anusha Rembers – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Venkat Bhagirath Pancha – Substantial contributions
to conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Figure 6: Excisional biopsy specimens of four quadrants
showing cystic lining with para keratinized stratified squamous
epithelium of uniform 6–8-cell thickness (H&E stain, x400).
International Journal of Case Reports and Images, Vol. 7 No. 6, June 2016. ISSN – [0976-3198]
Int J Case Rep Images 2016;7(6):360–364.
www.ijcasereportsandimages.com
Reddy et al. 364
Raj Kumar Badam – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
K. Sravan Kumar Reddy – Substantial contributions to
conception and design, Acquisition of data, Analysis
and interpretation of data, Drafting the article, Revising
it critically for important intellectual content, Final
approval of the version to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© 2016 GV Reddy et al. This article is distributed under
the terms of Creative Commons Attribution License which
permits unrestricted use, distribution and reproduction in
any medium provided the original author(s) and original
publisher are properly credited. Please see the copyright
policy on the journal website for more information.
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9. Voorsmit RA, Stoelinga PJ, van Haelst UJ. The
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