Imaging Science in Dentistry

Korean Academy of Oral and Maxillofacial Radiology (대한영상치의학회)
권호 표지
DOI QR코드

DOI QR Code

Radiological features of familial Gorlin-Goltz syndrome

  • Hegde, Shruthi (Department of Oral Medicine and Radiology, AB Shetty Memorial Institute of Dental Sciences, Nitte University) ;
  • Shetty, Shishir Ram (Department of Oral Medicine and Radiology, AB Shetty Memorial Institute of Dental Sciences, Nitte University)
  • Received : 2011.10.19
  • Accepted : 2012.01.21
  • Published : 2012.03.31
Download PDF
⟨ Previous Next ⟩

Abstract

Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.

Keywords

References

  1. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 1960; 262 : 908-12. https://doi.org/10.1056/NEJM196005052621803
  2. Manfredi M, Vescovi P, Bonanini M, Porter S. Nevoid basal cell carcinoma syndrome: a review of the literature. Int J Oral Maxillofac Surg 2004; 33 : 117-24. https://doi.org/10.1054/ijom.2003.0435
  3. Casaroto AR, Loures DC, Moreschi E, Veltrini VC, Trento CL, Gottardo VD, et al . Early diagnosis of Gorlin-Goltz syndrome: case report. Head Face Med 2011; 7 : 2. https://doi.org/10.1186/1746-160X-7-2
  4. Markt JC. Implant prosthodontic rehabilitation of a patient with nevoid basal cell carcinoma syndrome: a clinical report. J Prosthet Dent 2003; 89 : 436-42. https://doi.org/10.1016/S0022-3913(03)00072-6
  5. Honavar SG, Shields JA, Shields CL, Eagle RC, Demirci H, Mahmood EZ. Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome. Ophthalmology 2001; 108 : 1115-23. https://doi.org/10.1016/S0161-6420(01)00560-7
  6. Doede T, Seidel J, Riede FT, Vogt L, Mohr FW, Schier F. Occult, life-threatening, cardial tumor in syndactylism in Gorlin Goltz syndrome. J Pediatr Surg 2004; 39 : e17-9.
  7. Deepa MS, Paul R, Balan A. Gorlin Goltz syndrome: a review. J Indian Acad Oral Med Radiol 2003; 15 : 203-9.
  8. Lo Muzio 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. https://doi.org/10.14219/jada.archive.1999.0276
  9. Melo ES, Kawamura JY, Alves CA, Nunes FD, Jorge WA, Cavalcanti MG. Imaging modality correlations of an odontogenic keratocyst in the nevoid basal cell carcinoma syndrome: a family case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004; 98 : 232-6. https://doi.org/10.1016/j.tripleo.2004.02.055
  10. Lee BD, Kim JH, Choi DH, Koh KS, Lee SR. Recurrent odontogenic keratocysts in basal cell nevus syndrome: report of a case. Korean J Oral Maxillofac Radiol 2004; 34: 203-7.
  11. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997; 69 : 299-308. https://doi.org/10.1002/(SICI)1096-8628(19970331)69:3<299::AID-AJMG16>3.0.CO;2-M
  12. Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990. p. 372-8.
  13. Jarish W. Zur lehre von den autgeschwulsten. Archiv Jur Dermatologic Syphilogic 1894; 28 : 163-222.
  14. Patil K, Mahima VG, Gupta B. Gorlin syndrome: a case report. J Indian Soc Pedod Prev Dent 2005; 23 : 198-203. https://doi.org/10.4103/0970-4388.19010
  15. Rai S, Gauba K. Jaw cyst-Basal cell nevus-Bifid rib syndrome: a case report. J Indian Soc Pedod Prev Dent 2007; 25: 137-9. https://doi.org/10.4103/0970-4388.36565
  16. Jones EA, Sajid MI, Shenton A, Evans DG. Basal cell carcinomas in gorlin syndrome: a review of 202 patients. J Skin Cancer 2011; 2011 : 217378.

Cited by

  1. Radiographic assessment of a keratocystic odontogenic tumour using cone-beam computed tomography vol.14, pp.3, 2012, https://doi.org/10.1007/s40368-013-0030-y
  2. Pediatric Panoramic Radiography: Techniques, Artifacts, and Interpretation vol.41, pp.2, 2012, https://doi.org/10.1148/rg.2021200112