Stoelinga PJ, Peters JH, van de Staak WJ, Cohen MM Some new findings in the basal-cell nevus syndrome. Oral Surg Oral Med Oral Pathol. 1973; 36:686-692
Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med. 1960; 262:908-912
Jawa DS, Sircar K, Somani R, Grover N, Jaidka S, Singh S. Gorlin-Goltz syndrome. J Oral Maxillofac Pathol. 2009; 13:89-92
Kiwilsza M, Sporniak-Tutak K. Gorlin-Goltz syndrome – a medical condition requiring a multidisciplinary approach. Med Sci Mon Int Med J Exp Clin Res. 2012; 18:RA145-153
Maroto MR, Porras JL, Saez RS, de los Rios MH, Gonzalez LB. The role of the orthodontist in the diagnosis of Gorlin's syndrome. Am J Orthod Dentofacial Orthop. 1999; 115:89-98
Baliga SD, Rao SS. Nevoid-basal cell carcinoma syndrome: a case report and overview on diagnosis and management. J Maxillofac Oral Surg. 2010; 9:82-86
Lo Muzio L. Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Orphanet J Rare Dis. 2008; 3
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; 130:669-674
Lo Muzio L, Nocini PF, Savoia A, Consolo U, Procaccini M, Zelante L Nevoid basal cell carcinoma syndrome. Clinical findings in 37 Italian affected individuals. Clin Genet. 1999; 55:34-40
Yesudian D, Krishnan SG, Jayaraman M, Janaki VR, Yesudian P. Atypical gorlin's syndrome. Indian J Dermatol Venereol Leprol. 1995; 61:314-316
Dere KA Keratocystic odontogenic tumor: the correlation between recurrence rate and conservative treatment approach. nt J Oral Maxillofac Surg. 2015; 44
Madras J, Lapointe H. Keratocystic odontogenic tumour: reclassification of the odontogenic keratocyst from cyst to tumour. J Can Dent Assoc. 2008; 74
Kennedy RA. WHO is in and WHO is out of the mouth, salivary glands, and jaws sections of the 4th edition of the WHO classification of head and neck tumours. Br J Oral Maxillofac Surg. 2018; 56:90-95
Whaites E. Essentials of Dental Radiography and Radiology.Oxford: Elsevier; 2007
Yoshiura K, Weber AL, Runnels S, Scrivani SJ. Cystic lesions of the mandible and maxilla. Neuroimaging Clin N Am. 2003; 13:485-494
Scholl RJ, Kellett HM, Neumann DP, Lurie AG. Cysts and cystic lesions of the mandible: clinical and radiologic-histopathologic review. Radiographics. 1999; 19:1107-1124
Gohel A, Villa A, Sakai O. Benign jaw lesions. Dent Clin North Am. 2016; 60:125-141
Weber AL, Bui C, Kaneda T. Malignant tumors of the mandible and maxilla. Neuroimaging Clin N Am. 2003; 13:509-524
Gorlin-Goltz is a multisystemic inherited disorder characterized by multiple oral and systemic manifestations. Although rare, it is important for the general dentist to be aware of this condition due to the presentation of multiple odontogenic keratocysts, and to understand the differential diagnoses of jaw radiolucencies. This case describes a 53-year-old Afro-Caribbean patient who was referred by his dentist to the Maxillofacial team at Queen's Hospital regarding a ‘strange sensation’ in the mouth. Upon further investigation it was found that he had Gorlin-Goltz syndrome.
CPD/Clinical Relevance: This article highlights the importance of looking at ‘the bigger picture’ clinically and radiographically and provides a reminder of the oral manifestations of Gorlin-Goltz syndrome and differential diagnoses of jaw radiolucencies.
Article
Gorlin-Goltz is a rare condition characterized by various systemic manifestations, including multiple odontogenic keratocysts (OKCs). Due to the multitude of clinical and radiographic signs affecting the head and neck area, the dental practitioner has the opportunity to detect these signs and refer for further investigation and diagnosis. This emphasizes the importance of awareness of the condition's manifestations, in particular with regard to radiographic signs indicative of odontogenic keratocysts as they may present as incidental findings on routine dental radiographs.
In Gorlin-Goltz, multiple OKCs are found in the maxilla and the mandible, whereas in non-syndromic cases there is usually one sporadic OKC. It is important for the general dentist to be able to recognize anomalous radiographic appearances and be aware of the differential diagnoses of jaw radiolucencies, in particular the differences between benign and malignant conditions. This would enable the clinician to know when to refer for further management, including further imaging, for example dental panoramic tomography (DPT) and CT scans. The case report below highlights such a case where a patient was referred by his dentist regarding a radiolucency detected on an intra-oral radiograph.
Register now to continue reading
Thank you for visiting Dental Update and reading some of our resources. To read more, please register today. You’ll enjoy the following great benefits:
