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 Table of Contents  
Year : 2022  |  Volume : 10  |  Issue : 3  |  Page : 52-55

Nonsyndromic cases of multiple odontogenic keratocyst: Series of two cases

Department of Oral and Maxillofacial Surgery, Malla Reddy Institute of Dental Sciences, Hyderabad, Telangana, India

Date of Submission28-Mar-2022
Date of Decision02-Jul-2022
Date of Acceptance07-Jul-2022
Date of Web Publication28-Jul-2022

Correspondence Address:
Aishwarya Bhopathi
Malla Reddy Institute of Dental Sciences, Suraram, Quthubullapur, Hyderabad - 500 055, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjhs.mjhs_4_22

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Odontogenic keratocysts (OKCs) are developmental odontogenic cyst of epithelial origin. Multiple OKCs are often associated with a syndrome, especially nevoid basal cell carcinoma syndrome. The present article presents a series of two nonsyndromic cases of multiple OKCs. The presence of multiple OKCs and their recurrence can be attributed to factors such as the friable cystic lining, and daughter cysts. However, a comprehensive evaluation of any patients reporting multiple cysts/OKCs always has to be undertaken and the syndromic association should be ruled out.

Keywords: Multiple cysts, nevoid basal cell carcinoma syndrome, odontogenic keratocyst

How to cite this article:
Bhopathi A, Srinivas CN, Mohd YQ, Parushetti A. Nonsyndromic cases of multiple odontogenic keratocyst: Series of two cases. MRIMS J Health Sci 2022;10:52-5

How to cite this URL:
Bhopathi A, Srinivas CN, Mohd YQ, Parushetti A. Nonsyndromic cases of multiple odontogenic keratocyst: Series of two cases. MRIMS J Health Sci [serial online] 2022 [cited 2023 May 27];10:52-5. Available from: http://www.mrimsjournal.com/text.asp?2022/10/3/52/352627

  Introduction Top

Odontogenic keratocyst (OKC) is a cystic lesion of the jaw, arising from the odontogenic epithelium. It is considered a benign lesion, owing to its clinical behavior; the term OKC was first introduced by Philipsen in 1956. The pathology has undergone classification changes, in 2005, it was classified as a Keratocystic odontogenic tumor (KCOT) due to studies revealing genetic molecular alterations present in neoplasms. In 2017, it was reclassified as a cyst by the World Health Organization.[1] They account for 10%–20% of odontogenic cysts and are the third most common cyst of the jaws. About 10% of patients have multiple OKCs, and half of them have nevoid basal cell carcinoma syndrome (NBCCS). Other syndromes that have a known association include the orofacial digital syndrome, Ehlers–Danlos syndrome, Noonan syndrome, and Simpson–Golabi–Behmel syndrome.[2]

Diagnosis for the NBCCS can be made based on various major and minor clinical criteria and gene testing for the patient, which in fact confirms the diagnosis. The protein patched homolog 1 (PTCH1) or suppressor of fused protein (SUFU) is associated with the syndrome, mutations in PTCH1 are responsible for the multiple jaw cysts.[3]

The treatment for OKC includes marsupialization, enucleation with or without peripheral ostectomy, or marginal resection for large lesions followed by local application of Carnoy's solution/modified Carnoy's solution and 5-fluorouracil (5-FU), all of which primarily aim to reduce the recurrence of the lesion.[4],[5]

  Case Reports Top

Case 1

A 25-year-old male patient reported swelling in the left side of the face for 2–3 months and moderate, throbbing, and continuous pain for 3 days. On extraoral examination, a diffuse, smooth glossy swelling with no change in color or sinus tract opening, in the left lower one-third of the face was observed [Figure 1] and [Figure 2]. The swelling was tender, firm in consistency with the local rise in temperature. On intraoral examination, mouth opening was 32 mm, full complement of teeth was present with mesial inclination of 33, and all third molars were missing. There was obliteration of the buccal vestibule extending anterioposteriorly from 37 to the ramus region on the left side [Figure 4]. The swelling was tender and hard-soft consistency with a local rise in temperature. There was expansion of buccal and lingual cortical plates, from 31 to the left anterior ramus region and 32–35 regions. Based on the clinical findings, we have given a provisional diagnosis of OKC which may be associated with impacted 38. However, a differential diagnosis of dentigerous cyst, ameloblastoma was considered. Aspiration revealed creamy white, caseous material with total protein content <5 g per 100 ml. Panoramic radiograph [Figure 5] and cone-beam computed tomography (CT) image [Figure 6] and [Figure 7] revealed unilocular radiolucent lesions with well-defined margins associated with impacted third molars in the bilateral maxilla and well-defined multilocular radiolucency associated with impacted third molars extending from mid ramus region on the left side to 45 and 43 to the right mandibular ramus region with expansion of cortical plates. Incisional biopsy [Figure 8] and [Figure 9] was done in the mandibular anterior region, which described a cystic cavity lined by corrugated parakeratinized stratified squamous epithelium without rete ridges, with inflammatory cells and blood vessels. Cystic lumen shows keratin all of which suggested a final diagnosis of multiple infected OKC. As multiple jaw cysts were present, we were of a suspicion whether this is associated with a syndrome; further investigations were carried out, which did not reveal any clear clinical/radiological features for the NBCCS. PTCH1 gene report also did not reveal any mutations. The enucleation of the jaw cysts in the maxillary and the mandibular region was done under general anesthesia and gauze impregnated with 5-FU was left within the enucleated cavities for 24 h. Histological examination of both the specimens from maxilla and mandible revealed a cystic cavity lined by parakeratinised stratified squamous epithelium with tombstone appearance of basal cell layers and multiple daughter cysts in the lumen and absence of rete pegs. All these findings confirmed the diagnosis of multiple infected OKC.

Case 2

A 23-year-old female reported with swelling in the right side of the face for 1 month and severe, throbbing, and continuous pain for 10 days. On extraoral examination, diffuse, smooth glossy swelling, with no change in color or sinus tract opening of the overlying skin was observed in the right lower one-third of the face. On palpation, the swelling was tender, firm in consistency, with no local rise in temperature. On intraoral examination, the mouth opening was 36 mm. All third molars and maxillary left second molar were missing. Over retained deciduous canine was present in the left maxilla. Erythematous gingiva was noted around 47 region, with tenderness and no local rise in temperature, expansion of lingual cortical plate in 47–48 region. A provisional diagnosis of dentigerous cyst was given and a differential diagnosis of OKC was considered. Panoramic radiograph and CT image of the maxilla and mandible show multiple impacted teeth. Well-defined unilocular radiolucency noted in bilateral maxilla associated with impacted teeth and well-defined multilocular radiolucency in mandible extending from 47 to mid-ramus region and 37 to ramus region with lingual cortical plate expansion of mandible extended from 37 to ascending ramus [Figure 12]. Incisional biopsy was done in the right mandibular posterior region, described a cystic cavity lined by corrugated parakeratinised stratified squamous epithelium with palisaded basal layer cells and absent rete ridges. The underlying stroma shows inflammatory cell infiltrate. Foci of satellite cysts were also reported, which confirmed the diagnosis of multiple infected OKC. The presence of multiple OKCs leads to suspicion for syndromic association. Palmar–plantar pitting was not present, the skull was normocephalic. No ocular anomalies were noted as well and the patient has not given a positive history for relative similar condition. Chest X-ray posterior-anterior view, skull X-ray lateral, and anteroposterior view also did not show features suggestive of NBCCS. Enucleation of the cysts in the maxilla and mandible was done and modified Carnoy's solution was placed in the cystic cavity for 3 min under general anesthesia. Histological examination of both the specimens from maxilla and mandible revealed a cystic cavity lined by parakeratinized stratified squamous epithelium with tombstone appearance of basal cell layers and multiple daughter cysts in the lumen and absence of rete pegs. All these findings confirmed the diagnosis of multiple infected OKC. A recurrence of similar cystic lesion was noted in the left mandibular ramus region for the first time after 17 months, and second time at 24 months after the treatment of initial lesion, and on the right side mandibular ramus region after 43 months of treatment of initial lesion, the histopathology report of all of which had also confirmed OKC. Multiple lesions and their recurrence draw attention to further confirm the absence of syndrome association. Gene mapping was advised, which reported no mutations with the gene.

  Discussion Top

Multiple OKCs might be the first and the only manifestation of NBCCS without any other features associated with syndrome. However, other symptoms can occur in later decades of life. The appearance of recurrent OKCs in a young patient should lead to suspicion of NBCCS. The PTCH1 gene is associated with multiple jaw cysts in an NBCCS suspected individual, testing for heterozygosity of PTCH 1 gene was performed in both individuals to confirm the absence of the syndrome.[3] Both the cases in the present series were in the third decade of life which is the peak age of incidence of OKCs. The most common site of occurrence is the angle – ramus region of the mandible.[6] Considering the aggressive nature and recurrence rate, multiple cysts in both patients were treated by enucleation, and chemical cauterization. Carnoy's solution is a standard technique for OKC but it has its own banes due to the carcinogenic potential of the inhaled chloroform.[7] A modified Carnoy's solution (without chloroform) was used in the second case. 5-FU which is an antimetabolite, acts at the molecular level.[8] Immunohistochemical studies suggest that 5-FU may be more effective in preventing recurrence in cases of inflamed OKCs compared with noninflamed OKCs as was the first case where the patient had pain and inflammation.[9] There is no recurrence observed in the first case, recurrence at multiple sites was observed in the second case treated by enucleation and modified Carnoy's solution. However, there is a difference in follow-up of 1 year and 4 years for the first and second case, respectively. Concluding that 5-FU is the best treatment option for multiple, recurrent OKC, with limited cases is difficult, but the results noted were similar to the other studies.[8],[9]

  Conclusion Top

This paper aims to highlight that any patient reporting the multiple OKCs should be evaluated thoroughly for the possibility of NBCCS as multiple OKCs can occur a decade before other symptoms associated with NBBCS and clinical manifestations of NBCCS may remain hidden, there is a continued chance for the development of new and recurring cysts. Thus, as a general rule, a genetic test can be suggested whenever available, to rule out the syndrome and a strict follow-up is a must.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for 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.

  References Top

Polak K, Jędrusik-Pawłowska M, Drozdzowska B, Morawiec T. Odontogenic keratocyst of the mandible: A case report and literature review. Dent Med Probl 2019;56:433-6.  Back to cited text no. 1
Sundaragiri KS, Saxena S, Sankhla B, Bhargava A. Non syndromic synchronous multiple odontogenic keratocysts in a western Indian population: A series of four cases. J Clin Exp Dent 2018;10:e831-6.  Back to cited text no. 2
Mitropoulos P, Norman R. Nevoid basal cell carcinoma syndrome (Gorlin syndrome): Updated review of minimally invasive treatments. Cutis 2008;81:53-60.  Back to cited text no. 3
Titinchi F. Protocol for management of odontogenic keratocysts considering recurrence according to treatment methods. J Korean Assoc Oral Maxillofac Surg 2020;46:358-60.  Back to cited text no. 4
Ghali GE, Connor MS. Surgical management of the odontogenic keratocyst. Oral Maxillofac Surg Clin North Am 2003;15:383-92.  Back to cited text no. 5
Khan AA, Qahtani SA, Dawasaz AA, Saquib SA, Asif SM, Ishfaq M, et al. Management of an extensive odontogenic keratocyst: A rare case report with 10-year follow-up. Medicine (Baltimore) 2019;98:e17987.  Back to cited text no. 6
Alchalabi NJ, Merza AM, Issa SA. Using Carnoy's solution in treatment of keratocystic odontogenic tumor. Ann Maxillofac Surg 2017;7:51-6.  Back to cited text no. 7
[PUBMED]  [Full text]  
Akhter Lone P, Ahmed Wani N, Ahmed Janbaz Z, Bibi M, Kour A. Topical 5-fluorouracil application in management of odontogenic keratocysts. J Oral Biol Craniofac Res 2020;10:404-6.  Back to cited text no. 8
Caminiti MF, El-Rabbany M, Jeon J, Bradley G. 5-fluorouracil is associated with a decreased recurrence risk in odontogenic keratocyst management: A retrospective cohort study. J Oral Maxillofac Surg 2021;79:814-21.  Back to cited text no. 9


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