J Korean Assoc Oral Maxillofac Surg.  2022 Dec;48(6):386-389. 10.5125/jkaoms.2022.48.6.386.

Management of multiple odontogenic keratocysts associated with nevoid basal cell carcinoma syndrome: a case report of 11-year follow-up

• Kim HM
• Lim SJ
• Cho YC
• Sung IY
• Son JH

• KMID: 2537565
• DOI: http://doi.org/10.5125/jkaoms.2022.48.6.386

Abstract

Multiple odontogenic keratocysts (OKC) are a distinguishing feature of nevoid basal cell carcinoma syndrome (NBCCS). Owing to the high recurrence rate of syndromes associated OKCs, complete surgical resection is generally recommended as a definitive treatment. Herein, we report the management of multiple OKCs with marsupialization followed by excision with peripheral ostectomy in an NBCCS patient. We then discuss lesion progression over 11 years of annual follow-ups.

Keyword

Odontogenic cysts; Decompression; Recurrence

Figure

• 

  Fig. 1 Radiographs of multiple cystic lesions in the mandible (A) and falx cerebral calcification (B; arrows).

• 

  Fig. 2 Panoramic radiograph 6 months after definitive surgery.

• 

  Fig. 3 A recurrent lesion in the left posterior region of mandible 8 years after definitive surgery, which was removed by simple excision. The arrows indicate the lesion.

• 

  Fig. 4 Panoramic radiograph taken 11 years after initial presentation.

Reference

References

1. Jarisch . 1894; Zur lehre von den hautgeschwülsten. Arch Dermat Syph. 28:163–222. German. https://doi.org/10.1007/BF01843725. DOI: 10.1007/BF01843725.

2. Bakaeen G, Rajab LD, Sawair FA, Hamdan MA, Dallal ND. 2004; Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case. Int J Paediatr Dent. 14:279–87. https://doi.org/10.1111/j.1365-263X.2004.00560.x. DOI: 10.1111/j.1365-263X.2004.00560.x. PMID: 15242385.

3. Gorlin RJ. 1999; Nevoid basal cell carcinoma (Gorlin) syndrome: unanswered issues. J Lab Clin Med. 134:551–2. https://doi.org/10.1016/s0022-2143(99)90092-6. DOI: 10.1016/S0022-2143(99)90092-6. PMID: 10595780.

4. Kakarantza-Angelopoulou E, Nicolatou O. 1990; Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg. 48:593–9. discussion 599–600. https://doi.org/10.1016/s0278-2391(10)80472-0. DOI: 10.1016/S0278-2391(10)80472-0. PMID: 2187964.

5. Littler BO. 1979; Gorlin's syndrome and the heart. Br J Oral Surg. 17:135–46. https://doi.org/10.1016/s0007-117x(79)80041-4. DOI: 10.1016/S0007-117X(79)80041-4. PMID: 298837.

6. Vedtofte P, Praetorius F. 1979; Recurrence of the odontogenic keratocyst in relation to clinical and histological features. A 20-year follow-up study of 72 patients. Int J Oral Surg. 8:412–20. https://doi.org/10.1016/s0300-9785(79)80079-4. DOI: 10.1016/S0300-9785(79)80079-4. PMID: 120338.

7. Noy D, Rachmiel A, Zar K, Emodi O, Nagler RM. 2017; Sporadic versus syndromic keratocysts-can we predict treatment outcome? A review of 102 cysts. Oral Dis. 23:1058–65. https://doi.org/10.1111/odi.12687. DOI: 10.1111/odi.12687. PMID: 28449413.

8. Evans DG, Ladusans EJ, Rimmer S, Burnell LD, Thakker N, Farndon PA. 1993; Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. J Med Genet. 30:460–4. https://doi.org/10.1136/jmg.30.6.460. DOI: 10.1136/jmg.30.6.460. PMID: 8326488. PMCID: PMC1016416.

9. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, et al. 1997; Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet. 69:299–308. DOI: 10.1002/(SICI)1096-8628(19970331)69:3<299::AID-AJMG16>3.0.CO;2-M. PMID: 9096761.

10. Myoung H, Hong SP, Hong SD, Lee JI, Lim CY, Choung PH, et al. 2001; Odontogenic keratocyst: review of 256 cases for recurrence and clinicopathologic parameters. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 91:328–33. https://doi.org/10.1067/moe.2001.113109. DOI: 10.1067/moe.2001.113109. PMID: 11250631.

11. Manfredi M, Vescovi P, Bonanini M, Porter S. 2004; Nevoid basal cell carcinoma syndrome: a review of the literature. Int J Oral Maxillofac Surg. 33:117–24. https://doi.org/10.1054/ijom.2003.0435. DOI: 10.1054/ijom.2003.0435. PMID: 15050066.

12. Chapelle KA, Stoelinga PJ, de Wilde PC, Brouns JJ, Voorsmit RA. 2004; Rational approach to diagnosis and treatment of ameloblastomas and odontogenic keratocysts. Br J Oral Maxillofac Surg. 42:381–90. https://doi.org/10.1016/j.bjoms.2004.04.005. DOI: 10.1016/j.bjoms.2004.04.005. PMID: 15336762.

13. Stoelinga PJ. 2001; Long-term follow-up on keratocysts treated according to a defined protocol. Int J Oral Maxillofac Surg. 30:14–25. https://doi.org/10.1054/ijom.2000.0027. DOI: 10.1054/ijom.2000.0027. PMID: 11289615.

14. Chrcanovic BR, Gomez RS. 2017; Recurrence probability for keratocystic odontogenic tumors: an analysis of 6427 cases. J Craniomaxillofac Surg. 45:244–51. https://doi.org/10.1016/j.jcms.2016.11.010. DOI: 10.1016/j.jcms.2016.11.010. PMID: 28011178.