Dental and Medical Problems

Dent Med Probl
Index Copernicus (ICV 2021) – 132.50
MEiN – 70 pts
CiteScore (2021) – 2.0
JCI (2021) – 0.5
Average rejection rate (2022) – 79.69%
ISSN 1644-387X (print)
ISSN 2300-9020 (online)
Periodicity – quarterly

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Dental and Medical Problems

2018, vol. 55, nr 4, October-December, p. 447–451

doi: 10.17219/dmp/96272

Publication type: clinical case

Language: English

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Creative Commons BY-NC-ND 3.0 Open Access

Tetrad presentation of non-syndromic odontogenic keratocyst: An uphill diagnostic and therapeutic challenge

Opis przypadku grupy czterech bezobjawowych zębopochodnych torbieli rogowaciejących – złożoność diagnostyczna i lecznicza

Varshini Marimuthu1,A,B,C,D, Urvashi Ashwin Shetty1,A,B,C,D,E, Pushparaja Shetty1,A,E,F

1 Department of Oral Pathology and Microbiology, the A.B. Shetty Memorial Institute of Dental Sciences, NITTE (Deemed to be University), Deralakatte, India


Odontogenic keratocyst (OKC), in the last decade sceptically referred to as keratocystic odontogenic tumor (KCOT), is known for its subclinical extensive growth potential and significant rate of recurrences. Odontogenic keratocyst, being the third most common cystic lesion (10–20%) of the maxillofacial region, is often recognized as a sporadic lesion and is well-documented in the literature. Multiple presentation of these cysts over a lifetime is relatively uncommon and is usually seen in conjunction with nevoid basal cell carcinoma syndrome (NBCC), orofacial digital syndrome, Noonan syndrome, Ehlers–Danlos syndrome, Simpson–Golabi–Behmel syndrome, or other syndromes. The ‘two-hit’ hypothesis postulated by Knudson best explains this anomaly, wherein multiple OKCs associated with the syndromes arise as a consequence of the allelic loss in the patched (PTCH) gene, mapped to the long arm of chromosome 9q22.3-q31. A partial expression of the gene may result in multiple OKCs (5%) without any related syndromes. Though concurrent occurrence of non-syndromic multiple OKCs is a rare phenomenon, a handful of cases have been documented over the past few years. Adding to this, we report a case of multiple OKCs occurring synchronously and bilaterally in all 4 quadrants in non-syndromic, otherwise healthy persons, which could indicate a shift in trend.

Key words

multiple, odontogenic cysts, keratocyst, non-syndromic

Słowa kluczowe

mnogie, zębopochodne torbiele rogowaciejące, rogowaciejący guz nowotworowy, bezobjawowy

References (18)

  1. Makarla S, Bavle RM, Muniswamappa S, Narasimhamurthy S. A large extragnathic keratocystic odontogenic tumour. Case Rep Pathol. 2015;2015:723010.
  2. Sansare K. Response to the critique of „Keratocystic odontogenic tumor: Systematic review with analysis of 72 additional cases from Mumbai, India”. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;115:841–842.
  3. Kurdekar RS, Prakash J, Rana AS, Kalra P. Non-syndromic odontogenic keratocysts: A rare case report. Natl J Maxillofac Surg. 2013;4:90–93.
  4. Shear M, Speight PM. Odontogenic keratocyst. In: Shear M, Speight PM. Cysts of the Oral and Maxillofacial Regions. Oxford, UK: Blackwell Munksgaard; 2007:6–26.
  5. El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. Odontogenic and maxillofacial bone tumours. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, eds. WHO Classification of Tumours of Head and Neck. 4th ed. Lyon, France: IARC Press; 2017:204–236.
  6. Nayak MT, Singh A, Singhvi A, Sharma R. Odontogenic keratocyst: What is in the name? J Nat Sci Biol Med. 2013;4:282–285.
  7. Lucas RB. Pathology of Tumours of the Oral Tissues. 2nd ed. Edinburgh and London, UK: Churchill Livingstone; 1972:361–362.
  8. Mehta D, Raval N, Patadiya H, Tarsariya V. Gorlin–Goltz syndrome. Ann Med Health Sci Res. 2014;4:279–282.
  9. MacDonald-Jankowski DS. Orthokeratinized odontogenic cyst: A systematic review. Dentomaxillofac Radiol. 2010;39:455–467.
  10. Bartake A, Shreekanth N, Prabhu S, Gopalkrishnan K. Non-syndromic recurrent multiple odontogenic keratocysts: A case report. J Dent (Tehran). 2011;8:96–100.
  11. Kargahi N, Kalantari M. Non-syndromic multiple odontogenic keratocyst: A case report. J Dent Shiraz Univ Med Sci. 2013;14:151–154.
  12. Maheshwari V, Patel N, Jadhav R, Engineer P. Non syndromic odontogenic keratocyst: A case report. J Dent Med Sci. 2015;14:64–67.
  13. Reddy GV, Reddy MH, Garlapati K, et al. Non-syndromic multiple keratocystic odontogenic tumors: An arduous challenge for oral and maxillofacial specialists. Int J Case Rep Images. 2016;7:360–364.
  14. Gomes CC, Diniz MG, Gomez RS. Review of the molecular pathogenesis of the odontogenic keratocyst. Oral Oncol. 2009;45:1011–1014.
  15. Tarakji B, Baroudi K, Hanouneh S, Azzeghaiby SN, Nassani MZ. Possible recurrence of keratocyst in nevoid basal cell carcinoma syndrome: A review of literature. Eur J Dent. 2013;7:126–134.
  16. Stoelinga PJW. The management of aggressive cysts of the jaws. J Maxillofac Oral Surg. 2012;11:2–12.
  17. Abdullah WA. Surgical treatment of keratocystic odontogenic tumour: A review article. Saudi Dent J. 2011;23:61–65.
  18. Tandon P, Saluja H, Shah S, Dadhich A, Sachdeva S. Efficacy of Carnoy’s solution as an adjunct to conventional treatment modalities in aggressive bony tumors. Pravara Med Rev. 2017;9:26–28.