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 Table of Contents  
Year : 2015  |  Volume : 27  |  Issue : 2  |  Page : 264-267

Non-syndromic multiple keratocyst odontogenic tumor: A rare case report

1 Department of Oral Medicine and Radiology, Sarjug Dental College and Hospital, Darbhanga, Bihar, India
2 Department of Oral Medicine and Radiology, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India
3 Department of Psychiatry, Sri Krishna Medical College and Hospital, Muzaffarpur, Bihar, India
4 Department of Public Health Dentistry, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India

Date of Submission22-Oct-2014
Date of Acceptance13-Oct-2015
Date of Web Publication21-Nov-2015

Correspondence Address:
Abhijeet Alok
Department of Oral Medicine and Radiology, Sarjug Dental College and Hospital, Hospital Road, Laheriasarai, Darbhanga - 846 003, Bihar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-1363.170154

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Keratocystic odontogenic tumors (KCOTs) are one of the most frequent features of nevoid basal cell carcinoma syndrome (NBCCS). The condition is linked with mutation in the PTCH gene. Partial expression of the gene may result in occurrence of multiple recurring odontogenic keratocysts (OKCs). Although KCOTs are common in clinical practice, simultaneous occurrence of multiple cysts in both the maxilla and mandible of a patient is rare. These patients have early propensity to develop multiple neoplasms like basal cell carcinoma and medulloblastoma. Hence, early diagnosis and treatment is of utmost importance in reducing the severity of the long-term sequelae of NBCCS. We report a rare case of multiple KCOTs in a non-syndromic male patient, with emphasis on its diagnosis, radiographic features, and treatment.

Keywords: Basal cell nevus syndrome, keratocystic odontogenic tumor, mandible, multiple

How to cite this article:
Alok A, Panat SR, Singh ID, Singh S. Non-syndromic multiple keratocyst odontogenic tumor: A rare case report . J Indian Acad Oral Med Radiol 2015;27:264-7

How to cite this URL:
Alok A, Panat SR, Singh ID, Singh S. Non-syndromic multiple keratocyst odontogenic tumor: A rare case report . J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2020 Apr 8];27:264-7. Available from: http://www.jiaomr.in/text.asp?2015/27/2/264/170154

   Introduction Top

The maxillofacial region is more prone to cystic lesions than any other part of the body. Keratocystic odontogenic tumors (KCOTs) are the most common form of cystic lesions affecting the maxillofacial region. [1] They are clinically aggressive lesions which are thought to arise from the dental lamina or its remnants. [2] The condition is commonly associated with nevoid basal cell carcinoma syndrome (NBCCS). Nevoid basal cell carcinoma syndrome is associated with the triad of multiple basal nevi, multiple KCOTs, and skeletal abnormalities. [3] These triad of symptoms may be associated with other manifestations involving skeletal, craniofacial, neurological, skin, sexual, ophthalmic, and cardiac anomalies. [4] Multiple lesions have been known to occur in non-syndromic cases, though it is very rare. [5] These multiple lesions may be the first manifestation of NBCCS or they may occur because of the multifocal nature of KCOT. [6],[7] We present a case of recurrent multiple KCOTs which is not associated with any syndrome.

   Case Report Top

A 24-year-old male patient reported to the Department of Oral Medicine and Radiology, with a chief complaint of pain in the right lower back tooth region since 10 days. The history of present illness revealed that pain was present since 20 days. Pain was sudden and intermittent in nature. Pain radiated to the entire jaw region on the right side. There was a gradual decrease in the mouth opening over a period of time. Patient visited a local practitioner where he got pus from the right mandibular area discharged. But there was no relief in pain and mouth opening was still reduced.

On extraoral examination, a diffuse swelling was present on the right side of the face, extending antero-posteriorly from the right commissure of mouth to the angle of mandible. Supero-inferiorly, it extended from the tragus of ear to the lower border of mandible [Figure 1]. The overlying surface appeared smooth and normal in color. On palpation, it was non-tender, not associated with any discharge, and there was no local rise in temperature. Intraorally, on inspection, vestibular obliteration was seen with respect to 46, 47, and 48 region [Figure 2]. On palpation, pus discharge was present distal to 47. So, based on the clinical findings and history, a provisional diagnosis of chronic periodontal abscess with reference to 47 and 48 region was made with a differential diagnosis of infected lateral periodontal cyst with reference to 47, 48 region.
Figure 1: Extraoral fi gure of patient

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Figure 2: Intraoral fi gure of patient

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The investigations done included intraoral periapical radiograph (IOPAR) with reference to 47, 48 region, orthopantomograph (OPG), and computed tomography (CT) scan. Intra oral periapical radiograph revealed a well-defined radiolucency mesial to 48, which was roughly oval in shape and measuring about 1 × 1.5 cm in diameter. The internal structure appeared radiolucent with a radiopaque border. No root resorption was seen. The OPG revealed multiple radiolucencies in the right and left quadrants [Figure 3]. A well-defined radiolucency with sclerotic border, roughly oval in shape, approximately 1 × 1.5 cm in diameter, extending from distal root of 46 to distal root of 48 was seen. The internal structure appeared completely radiolucent. Two well-defined radiolucencies were present on the mesial and distal aspects of 33, which were roughly circular in shape, approximately 1 × 1.5 cm in diameter, with a radiopaque rim. A well-defined radiolucency was present mesial to 38, roughly pitcher in shape, approximately 1 × 2 cm in diameter, and extending from the distal root of 37 to 38. A radiopaque rim was also present. The internal structure appeared completely radiolucent. Mesioangular impaction with reference to 38, displacement of teeth with reference to 35, 36, and retained 74, 84 were seen. Multiple retained deciduous teeth were present in the maxillary arch.
Figure 3: OPG of patient

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The CT scan of the head and neck revealed multiple cystic lesions in the mandibular region [Figure 4]. The patient was further evaluated to rule out any syndrome due to the presence of multiple cystic lesions. Chest (PA) radiograph was insignificant [Figure 5]. Blood investigations were within normal limits. Dermatological examination did not reveal any cutaneous abnormalities like palmar and plantar defects. Under general anesthesia, based on the size of cyst, enucleation of the cysts in the mandibular region on the right and left sides was done. The histopathologic examination revealed multiple cystic spaces filled with keratin. These cystic spaces were lined by parakeratinized stratified squamous cystic epithelial lining of predominantly even thickness with palisaded basal cells and a corrugated surface suggestive of odontogenic keratocyst (OKC) [Figure 6]. So, a final diagnosis of non-syndromic multiple OKCs was made.
Figure 4: CT scan revealing multiple cystic lesions

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Figure 5: Chest PA radiograph

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Figure 6: Histopathologic picture of KCOT

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   Discussion Top

Odontogenic keratocyst was first identified by Philipsen in 1956 and was defined by the World Health Organization (WHO) as a cyst characterized by a thin fibrous capsule and a lining of keratinized stratified squamous epithelium of usually about five to eight cells in thickness and generally without rete pegs. [8] The OKC, now designated by WHO as a KCOT, is defined as "a benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potential for aggressive, infiltrative behavior." WHO recommends the term "keratocyst odontogenic tumor" as it reflects its neoplastic nature. [9] Multiple KCOTs commonly occur in NBCCS or Gorlin-Goltz syndrome, orofacial digital syndrome, Ehler-Danlos syndrome, Noonan syndrome, and Simpson-Golabi-Behmel syndrome. [10] The KCOT has a biological behavior similar to a benign neoplasm and it is still debated whether the origin of KCOT is developmental or neoplastic. If KCOT is associated with any syndrome, skeletal, cutaneous, neurologic, ophthalmic, and sexual abnormalities are also found in those patients. [11] These features were not present in our case.

It comprises approximately 11% of all jaw cysts. It occurs most commonly in the mandible, especially in the posterior body and ramus region. [12] The KCOTs have a high recurrence rate, reportedly between 25% and 60% (when associated with NBCCS, the recurrence rate is about 82%). [13] The most frequent clinical manifestations reported at first admission were swelling, pain, or both. [14] Our patient reported both swelling and pain. Histologically, KCOTs show the presence of a thin, band-like parakeratinized or orthokeratinized stratified squamous epithelium, with a prominent basal layer of columnar or cuboidal cells, and an inflammation-free connective tissue wall. Radiographically, keratocysts present well-defined radiolucent lesions with smooth, usually corticated margin and may be either multilocular or unilocular. In our case, well-defined radiolucency with corticated margins was present. Other investigations which should be done to rule out any syndromic association are chest PA and CT scan, which was done in our case. The occurrence of multiple KCOTs may be the first and only manifestation of NBCCS, as seen in our patient. Treatment modalities include marsupialization and enucleation, combined with adjuvant cryotherapy or chemical cautery or Carnoy's solution, and marginal or radical resection. [15]

   Conclusion Top

Due to the possibility that multiple KCOTs might be the first and only manifestation of NBCCS, the higher rate of recurrence of KCOT in NBCCS, and the probable development of other associated problems in future, it is the responsibility of the dentist to rule out the presence of this syndrome and start appropriate treatment as soon as the diagnosis is made and conduct a careful follow-up.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Koseoglu BG, Atalay B, Erdem MA. Odontogenic cysts: A clinical study of 90 cases. J Oral Sci 2004;46:253-7.  Back to cited text no. 1
Kramer IR, Pindborg JJ, Shear M. Histological Typing of Odontogenic Tumours. 2 nd ed. Berlin: Springer Verlag; 1992. p. 37.  Back to cited text no. 2
Gorlin RJ, Goltz RW. Multiple nevoid basal cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 1960;262:908-12.  Back to cited text no. 3
Manfredi M, Vescovi P, Bonanini M, Porter S. Nevoid basal cell carcinoma syndrome: A review of literature. Int J Oral Maxillofac Surg 2004;33:117-24.  Back to cited text no. 4
Auluck A, Suhas S, Pai KM. Multiple odontogenic keratocysts: Report of a case. J Can Dent Assoc 2006;72:651-6.  Back to cited text no. 5
Lo Muziol L, Nocini P, Bucci P, Pannone G, Consolo U, Procaccini M. Early diagnosis of nevoid basal cell carcinoma syndrome. J Am Dent Assoc 1999;130:669-74.  Back to cited text no. 6
Boyne PJ, Hou D, Moretta C, Pritchard T. The multifocal nature of odontogenic Keratocysts. J Calif Dent Assoc 2005;33:961-5.  Back to cited text no. 7
Guruprasad Y, Chauhan DS. Multiple Odontogenic keratocysts in a nonsyndromic patient. J Cranio Max Dis 2012;1:36-40.  Back to cited text no. 8
  Medknow Journal  
Barnes L, Eveson JW, Reichart P, Sidransky D. Pathology and Genetics of Head and Neck Tumours. WHO classification of Tumors Series. Lyon: IARC Press; 2005. p. 306-7.  Back to cited text no. 9
Lindeboom JA, Kroon FH, de Vires J, van den Akker HP. Multiple recurrent and de novo odontogenic keratocysts associated with oral-facial-digital syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:458-62.  Back to cited text no. 10
Mosqueda-Taylor A, Irigoyen-Camacho ME, Diaz-Franco MA, Torres-Tejero MA. Odontogenic cyst. Analysis of 856 cases. Med Oral 2002;7:89-96.  Back to cited text no. 11
Dayan D, Buchner A, Gorsky M, Harel-Raviv M. The peripheral odontogenic keratocyst. Int J Oral Maxillofac Surg 1988;17:81-3.  Back to cited text no. 12
Sapp JP, Eversole LR, Wysocki GP. Contemporary Oral and Maxillofacial Pathology. 2 nd ed. St. Louis: Mosby; 2004. p. 54.  Back to cited text no. 13
Dominguez FV, Keszler A. Comparative study of keratocysts, associated and non-associated with nevoid basal cell carcinoma syndrome. J Oral Pathol 1988;17:39-42.  Back to cited text no. 14
Stoelinga PJ. Long term follow-up on keratocysts treated according to a defined protocol. Int J Oral Maxillofac Surg 2001;30:14-25.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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