Heera R, Thatchani G V, Shinu Koshy


Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome or Basal cell nevus syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. Early diagnosis and treatment of this syndrome as well as family screening and genetic counselling are essential for the patients affected with this syndrome. This syndrome may be diagnosed early by a dentist by routine radiographic examinations in the first decade of life, since the odontogenic keratocysts(OKC) are usually one of the first manifestations of the syndrome. Here, we present two cases of Nevoid basal cell carcinoma syndrome reported to our college.


Nevoid basal cell carcinoma syndrome, Odontogenic keratocyst, Patched gene, Bid rib syndrome, Carnoy's solution.

Full Text:



Shafer WG, Hine MK, Levy BM, Rajendran R, Sivapathasundharam B. A textbook of oral pathology. Philadelphia: Saunders; 1983 Sep 20.

Shear M, Speight P. Cysts of the oral and maxillofacial regions. John Wiley & Sons; 2008 Apr 15.

Yamamoto K, Yoshihashi H, Furuya N, Adachi M, Ito S, Tanaka Y, Masuno M, Chiyo H, Kurosawa K: Further delineation of 9q22 deletion syndrome associated with basal cell nevus (Gorlin) syndrome: Report of two cases and review of the literature. Congenit Anom 2009, 49:8-14.

Cohen MM: Nevoid basal cell carcinoma syndrome: molecular biology and new hypotheses. Int J Oral Maxillofac Surg 1999, 28:216-23.

Satinoff MI, Wells C: Multiple basal cell naevus syndrome in ancient. Egypt Med Hist 1969, 13:294-7.

Farndon PA, Del Mastro RG, Evans DG, Kilpatrick MW: Location of gene for Gorlin syndrome. Lancet 1992, 339:581-2.

Shanley S, Ratcliffe J, Hockey A, Haan E, Oley C, Ravine D, Martin N, Wicking C, Chenevix-Trench G: Nevoid basal cell carcinoma syndrome: review of 118 affected individuals. Am J Med Genet 1994, 50:282-90.

Gorlin RJ: Nevoid basal cell carcinoma (Gorlin) syndrome: Unanswered issues. J Lab Clin Med 1999, 134:551-2.

Amezaga AOG, Arregui OG, Nuño SZ, Sagredo AA, Urizar JMA: Gorlin-Goltz syndrome: Clinicopathologic aspects. Med Oral Patol Oral Cir Bucal 2008, 13:338-43.

Muzio LL. Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Orphanet journal of rare diseases. 2008 Dec;3(1):32.

Joshi PS, Deshmukh V, Golgire S. Gorlin-goltz syndrome. Dental research journal. 2012 Jan;9(1):100.

Ali IK, Karjodkar FR, Sansare K, Salve P, Dora A, Goyal S. Nevoid Basal Cell Carcinoma Syndrome-Clinical and Radiological Findings of Three Cases. Cureus. 2016 Aug;8(8).

Gupta, Shalini R., et al. Nevoid basal cell carcinoma syndrome in Indian patients: a clinical and radiological study of 6 cases and review of literature. Oral surgery, oral medicine, oral pathology and oral radiology. 2012 Jan 1;113(1):99-110.

Lata J et.al. Gorlin–Goltz syndrome: A case series of 5 patients in North Indian population with comparative analysis of literature. Contemporary clinical dentistry. 2015 Sep;6(Suppl 1):S192.

Sudipto Sahu et al. An enigma of Gorlin–Goltz syndrome: Two cases reported in mother and daughter. Journal of Oral and Maxillofacial Pathology: JOMFP. 2019 Feb;23(Suppl 1):115.

Suchithra KS. Gorlin Goltz Syndrome–Case Series and Review of Literature. 2019;18(4):76–86.

Kalia V.et.al, The syndromic multiple odontogenic keratocyst in siblings: A familial study. Annals of maxillofacial surgery. 2011 Jan;1(1):77.

Baliga SD, Rao SS. Nevoid-basal cell carcinoma syndrome: a case report and overview on diagnosis and management. Journal of maxillofacial and oral surgery. 2010 Mar 1;9(1):82-6.

Pereira T, Tamgadge A, Sapdhare S, Pujar A. Multiple keratocystic odontogenic tumors in nevoid basal cell carcinoma syndrome. Annals of maxillofacial surgery. 2015 Jul;5(2):277.

Kiran NK, Raj TT, Mukunda KS, Reddy VR. Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome). Contemporary clinical dentistry. 2012 Oct;3(4):514.

Subramanyam SB, Sujata DN, Sridhar K, Pushpanjali M. Nevoid Basal cell carcinoma syndrome: a case report and review. Journal of maxillofacial and oral surgery. 2015 Mar 1;14(1):11-5.

Anchlia S, Vyas S, Bahl S, Nagavadiya V. Gorlin-Goltz syndrome in twin brothers: an unusual occurrence with review of the literature. Bmj Case Reports. 2015;2015.

Mehta DN, Raval N, Patadiya H, Tarsariya V. Gorlin Goltz Syndrome. Annals of medical and health sciences research. 2014;4(2):279-82.

Kulkarni GH, Khaji SI, Metkari S, Kulkarni HS, Kulkarni R. Multiple keratocysts of the mandible in association with Gorlin-Goltz syndrome: a rare case report. Contemporary clinical dentistry. 2014 Jul;5(3):419.

Mohan RP, Verma S, Agarwal N, Singh U. Gorlin-Goltz syndrome: a rare case report. Case Reports. 2013 Jun 25;2013.

Tandon S, Chauhan Y, Sharma M, Jain M. Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child. International journal of clinical pediatric dentistry. 2016 Jul;9(3):264.

Agrawal A, Murari A, Vutukuri S, Singh A. Gorlin-goltz syndrome: case report of a rare hereditary disorder. Case reports in dentistry. 2012;2012.

Pandeshwar, Padma, K. Jayanthi, and D. Mahesh. "Gorlin-goltz syndrome." Case reports in dentistry 2012 (2012).

Patankar AP, Kshirsagar RA, Dugal A, Mishra A, Ram H. Gorlin-Goltz syndrome: A series of three cases. National journal of maxillofacial surgery. 2014 Jul;5(2):209.

Ramesh M, Krishnan R, Chalakkal P, Paul G. Gorlin-Goltz syndrome: case report and literature review. Journal of oral and maxillofacial pathology: JOMFP. 2015 May;19(2):267.

Thomas N, Vinod SV, George A, Varghese A. Gorlin–Goltz syndrome: An often missed diagnosis. Annals of maxillofacial surgery. 2016 Jan;6(1):120.

Hegde S, Shetty SR. Radiological features of familial Gorlin-Goltz syndrome. Imaging science in dentistry. 2012 Mar 1;42(1):55-60.

Pol CA, Ghige SK, Kalaskar RR, Gosavi SR. Gorlin-Goltz syndrome: A rare case report. Contemporary clinical dentistry. 2013 Oct;4(4):547.

Chandran S, Marudhamuthu K, Riaz R, Balasubramaniam S. Odontogenic Keratocysts in Gorlin–Goltz Syndrome: A Case Report. Journal of international oral health: JIOH. 2015;7(Suppl 1):76.

Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin syndrome with bilateral polydactyly: A rare case report. International journal of clinical pediatric dentistry. 2013 Sep;6(3):208.

Khaliq MI, Shah AA, Ahmad I, Hasan S, Jangam SS. Keratocystic odontogenic tumors related to Gorlin–Goltz syndrome: A clinicopathological study. Journal of oral biology and craniofacial research. 2016 May 1;6(2):93-100.

Bahadure RN, Jain ES, Badole GP. Gorlin and Goltz syndrome: A case report with surgical review. International journal of clinical pediatric dentistry. 2013 May;6(2):104.

Kumar NN, Padmashree S, Jyotsna TR, Shastry SP. Gorlin-Goltz syndrome: A rare case report. Contemporary clinical dentistry. 2018 Jul;9(3):478.


  • There are currently no refbacks.