Nevoid Basal Cell Carcinoma Syndrome
Overview
Affiliations
Binkley and Johnson first reported this syndrome in 1951. But it was in 1960, Gorlin-Goltz established the association of basal cell epithelioma, jaw cyst and bifid ribs, a combination which is now frequently known as Gorlin-Goltz syndrome as well as Nevoid Basal Cell Carcinoma Syndrome (NBCCS). NBCCS is inherited as an autosomal dominant trait with high penetrance and variable expressivity. NBCCS is characterized by variety of cutaneous, dental, osseous, opthalmic, neurologic and sexual abnormalities. One such case of Gorlin-Goltz syndrome is reported here with good illustrations.
Maxillary Odontogenic Keratocyst.
Walsh M, Hussein M, Carter M, Abdulrahman S J Surg Case Rep. 2022; 2022(4):rjac078.
PMID: 35422993 PMC: 9004408. DOI: 10.1093/jscr/rjac078.
Bifid rib with fused vertebrae - A rare abnormality of the skeletal system: A case report.
Mirzaei S, Moslemi S, Shafeghat F, Karimi S, Eftekharzadeh M Int J Surg Case Rep. 2021; 86:106281.
PMID: 34500251 PMC: 8430391. DOI: 10.1016/j.ijscr.2021.106281.
A Rare Case of Gorlin-Goltz Syndrome Presented to the Emergency Department as Facial Swelling.
Aloosi S, Mahmood K, Ali S, Mahmud P, Hasan S, Muhamed H Adv J Emerg Med. 2019; 2(4):e46.
PMID: 31172109 PMC: 6548149. DOI: 10.22114/AJEM.v0i0.83.
Gorlin-Goltz Syndrome: A Rare Case Report.
Kumar N, Padmashree S, Jyotsna T, Shastry S Contemp Clin Dent. 2018; 9(3):478-483.
PMID: 30166848 PMC: 6104368. DOI: 10.4103/ccd.ccd_96_18.
Gorlin-Goltz syndrome: An often missed diagnosis.
Thomas N, Vinod S, George A, Varghese A Ann Maxillofac Surg. 2016; 6(1):120-4.
PMID: 27563620 PMC: 4979327. DOI: 10.4103/2231-0746.186148.