|Year : 2015 | Volume
| Issue : 2 | Page : 268-272
Non-syndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management
Sulabha A Narsapur, Sameer Choudhari, Neelkant M Warad, Shankar Manjunath
Department of Oral Medicine and Radiology, Al-Ameen Dental College and Hospital, Bijapur, Karnataka, India
|Date of Submission||06-Dec-2014|
|Date of Acceptance||13-Oct-2015|
|Date of Web Publication||21-Nov-2015|
Sulabha A Narsapur
Department of Oral Medicine and Radiology, Al-Ameen Dental College and Hospital, Athani Road, Bijapur - 586 108, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Odontogenic keratocyst (OKC), now known as keratocystic odontogenic tumor (KCOT), is the most common cystic lesion occurring in the maxillofacial region. Multiple occurrence of these cysts is less frequent and is usually associated with syndromes, the most common being the Gorlin Goltz syndrome or the nevoid basal cell carcinoma syndrome (NBCCS). We hereby report an unusual case of multiple OKCs in a healthy adult, associated with other dental anomalies. Management approach consisted of enucleation with curettage of the smaller lesions and decortication of buccal cortex in the large lesion. The condition in the present case may be because of the multifocal nature of OKC rather than its association with any syndrome. Concomitant occurrence of multiple OKCs with other dental anomalies may be coincidental. Impacted teeth or missing teeth must be radiographed to rule out the lesions associated with them.
Keywords: Multifocal nature, non-syndromic, odontogenic keratocyst
|How to cite this article:|
Narsapur SA, Choudhari S, Warad NM, Manjunath S. Non-syndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management. J Indian Acad Oral Med Radiol 2015;27:268-72
|How to cite this URL:|
Narsapur SA, Choudhari S, Warad NM, Manjunath S. Non-syndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2020 Jul 5];27:268-72. Available from: http://www.jiaomr.in/text.asp?2015/27/2/268/170155
| Introduction|| |
Odontogenic keratocyst (OKC), now designated by the World Health Organization as keratocystic odontogenic tumor (KCOT), is a benign uni- or multicystic intraosseous tumor of odontogenic origin. It has a characteristic lining of parakeratinized stratified squamous epithelium and has a potential for aggressive infiltrative behavior.  Multiple OKCs are unusual and their occurrence is often associated with nevoid basal cell carcinoma syndrome (NBCCS), Ehler-Danlos syndrome, Noonan syndrome, orofacial digital syndrome, or other syndromes. , Studies reveal that 5.8% of multiple OKCs presented without any features of a syndrome, 8.1% were associated with NBCCS, and 7.6% of them had recurrences.  We present a case of multiple OKC with dental anomalies, which was not associated with any syndrome. Follow-up of 3 years did not show any recurrence of lesion or any manifestation of the syndrome.
| Case Report|| |
A 32-year-old male patient reported to the Department of Oral Medicine and Radiology with a complaint of mild swelling in the left lower posterior region since 6 months, which was gradually progressive and not associated with pain [Figure 1]. His medical history was non-contributory. His dental history revealed extraction of 37, 31, and 32 a few years back. Extraoral examination revealed a non-tender diffuse swelling involving the left posterior mandibular region. Intraorally, a single diffuse swelling was seen extending from 36 to missing 38 region, which was non-tender, hard in consistency, with slight expansion of the buccal cortex and obliteration of the buccal vestibule. The overlying mucosa was normal; 63 was retained; 13, 25, and all the third molars were missing. Fixed partial denture was noted from 41 to 33. Extrusion along with mobility was noted with 14. Also, 23 was transposed between 24 and 26.
Panoramic radiograph revealed four radiolucent lesions in the maxillary and mandibular arches [Figure 2] and [Figure 3]. The first lesion being the largest was located in the left posterior mandible, which was well defined, unilocular with sclerotic border measuring approximately 5 × 7 cm. It extended from 36 to posterior border of the mandible antero-posteriorly and from the lower border of the mandible to more than half of the ramus of mandible superio-inferiorly. Also, 37 had been extracted a few years back and 38 was congenitally missing. Second lesion was noted in the right posterior mandible in missing 48 region. A well-defined unilocular radiolucency with sclerotic borders measuring approximately 2 cm in diameter was noted distal to 47 in congenitally missing 48 region. Third lesion was observed in the right maxillary anterior region. Well-defined unilocular pericoronal radiolucency with sclerotic border measuring approximately 2.5 cm in diameter was seen with impacted 13, enclosing its crown. Fourth lesion was seen in the left maxillary posterior region. Well-defined unilocular radiolucency with sclerotic borders measuring approximately 2 cm in diameter, enclosing completely the impacted 25 was noted. The second maxillary left premolar (25) was sandwiched between the roots of transposed 23 and 26. Short and dilacerated roots were observed in 14 and impacted 25.
Based on these findings, a provisional diagnosis of OKCs in the right and left posterior mandible and dentigerous cysts in the right anterior and left posterior maxilla was made. Ameloblastoma, residual cyst and central giant cell granuloma were considered in the differential diagnosis. The patient was referred to a physician and dermatologist for systemic evaluation of syndromes associated with multiple OKCs. The review of systems did not show any abnormalities. Hemogram was also within normal limits. Under general anesthesia, submandibular and Risdon's incision was made to approach the large cystic lesions. Decortication of buccal cortex was done along with application of Carnoy's solution [Figure 4]. Iliac graft was placed and stabilized by titanium mesh supported by titanium screws. The other three lesions were enucleated with curettage along with removal of the involved teeth. Specimens were subjected to histopathologic examination, which confirmed OKC with respect to all the four lesions [Figure 5]. Follow-up of 3 years did not show any recurrences with any of the lesions [Figure 6].
| Discussion|| |
Ever since Philipsen described the OKC in 1956, the lesion has continued to raise considerable clinical interest because of its unusual growth pattern and tendency to recur after surgical removal.  Odontogenic keratocyst constitutes 3-21.5% of odontogenic cysts.  Multiple OKCs in non-syndromic patients are rare, though few cases have been reported in the literature. ,,,, Some suggested their case being partial expression of NBCCS.  In contrast to all the previous reports, the present case involves concomitant occurrence of multiple OKCs, transposition of maxillary canine, along with its associated dental anomalies like retained deciduous tooth, short and dilacerated roots, and missing teeth in a healthy individual, which is a very rare phenomenon. Non-syndromic multiple OKCs may occur due to the multifocal nature of OKC rather than a genetic defect.  Often multiple OKCs form one of the components of NBCCS, along with nevoid basal cell carcinoma of skin, bifid ribs, calcification of fax cerebri, frontal and parietal bossing, mandibular prognathism, and cutaneous, ophthalmic, neurological, and sexual abnormalities. , None of the syndromic features were present except for the presence of multiple OKCs and other dental anomalies.
The etiopathogenesis of sporadic intraosseous OKC appears to be linked to defects in activation of Hedgehog (HH) signaling pathway, caused by Patched-1 (PTCH-1) inactivation. PTCH-1 protein is the receptor for Sonic Hedgehog (Shh) protein. PTCH-1 inhibits the signaling by repressing smoothened (Smo) activity that leads to nuclear Gli activation. This mechanism is the key regulator of embryonic development, controlling cell proliferation and differentiation, particularly in the orofacial region. 
Odontogenic ketatocysts are more frequently seen in second to third decade of life with male predilection. Patients with multiple OKCs with or without NBCCS are usually younger than those with single OKC.  The present case was a male patient in his fourth decade of life. Majority of the lesions occur in the posterior mandible, and the mandible is affected twice that of maxilla.  In the present case, two lesions affected posterior mandible, one affected posterior maxilla, and one was seen in the anterior maxilla.
Odontogenic ketatocyst is a developmental cyst with distinct histopathologic and clinical behavior. Previously, OKC was termed as primordial cyst. It occurs in place of a tooth where cystic degeneration of the enamel organ epithelium may occur before the developmental of the dental hard tissue.  Some still believe primordial cyst and OKC are same, though it is not universally accepted. Most primordial cysts are actually OKCs.  Both the mandibular lesions in the present case were associated with missing third molars. Twenty-five to forty percent of OKCs are associated with unerupted teeth.  Both the maxillary OKCs were associated with impacted teeth.
Transposition is a rare developmental dental anomaly causing interchange in position of two permanent teeth within the same quadrant of the jaws. It is often associated with other dental anomalies like peg-shaped lateral incisors, rotation of teeth, missing teeth, dilacerations and malformation of other teeth, retention of deciduous teeth, etc. It is more frequent in maxillary arch with the maxillary canine.  Interestingly, in the present case, transposition of the left maxillary canine was noted along with its other accompanied dental anomalies like retention of left deciduous canine, all third molars missing, and short and dilacerated roots of right maxillary first premolar and left maxillary second premolar. The impaction of maxillary second premolar may be due to transposition of canine and prolonged impacted tooth are often known be associated with many lesions.
Clinically, OKC manifests with discharge, swelling, pain, or sometimes may be asymptomatic.  In our case, the large OKC was associated with mild swelling, while the other three lesions were accidently discovered on radiographs. Extrusion and mobility of the first premolar in right maxilla was due to the underlying lesion. Radiographically these lesions present as well-defined unilocular or multilocular lesions often not distinguishable from ameloblastoma. When associated with impacted teeth, they are often confused with dentigerous cyst. In the present case, a similar situation was observed.
Radiological variants include follicular type which surrounds the crown of unerupted tooth and is attached to neck of the tooth resembling the dentigerous cyst radiographically, envelopmental type which embraces an adjacent tooth, replacemental type which is formed in place of normal tooth, extraneous type which is seen in ascending ramus away from the teeth, and collateral type which is adjacent to the roots of teeth and are indistinguishable from lateral periodontal cyst radiographically. , Follicular OKCs are relatively uncommon.  In the present case, both the maxillary OKCs were of follicular type.
Histopathologically, OKC with NBCCS shows parakeratinized, intramural epithelial remnants, satellite cyst, lower thickness of epithelium, and fewer nuclei than the solitary cyst. , In the present case, cystic cavity with parakeratinized epithelium was seen, with the palisaded basal layer having epithelium of greater height with more basal nuclei which are characteristic of solitary cyst.
Treatment modalities may be conservative which includes enucleation with or without curettage, along with cryotherapy or marsupialization which preserves the anatomical structures including teeth. Aggressive treatment includes peripheral ostectomy, chemical curettage with Carnoy's solution, or en bloc resection, which are recommended for NBCCS cases, large KCOT, and recurrent lesions.  In the present case, aggressive treatment for the large lesion and conservative treatment for the smaller lesion were done. Odontogenic ketatocysts associated with NBCCS have higher recurrence when compared to solitary OKC, which may be due to the higher rate of proliferation of the epithelial lining.  In the present case, follow-up of 3 years did not show recurrence with any of the lesions.
| Conclusion|| |
With the above discussion, we conclude that the present case of multiple OKCs is non-syndromic and its association with a dental anomaly like transposition along with its associated dental anomalies may be coincidental, and such a presentation is a rare phenomenon. It is a research question if such concomitant findings are coincidental or may be considered a syndrome in itself. Impacted teeth or missing teeth must be radiographed to rule out the lesions associated them. However, we still recommend geneticist counseling and long-term follow-up of such cases, as these are the first and only initial manifestation of NBCCS.
Declaration of Patient Consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]