Open AccessStudying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series
Author(s) -
Shikha Saxena,
Krishna Sireesha Sundaragiri,
Akshay Bhargava,
Bharat Sankhla
Publication year - 2020
Publication title -
journal of oral and maxillofacial pathology/journal of oral and maxillofacial pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.455
H-Index - 25
eISSN - 1998-393X
pISSN - 0973-029X
DOI - 10.4103/jomfp.jomfp_358_19
Subject(s) - nevoid basal cell carcinoma syndrome , series (stratigraphy) , basal cell carcinoma , basal cell , biology , medicine , pathology , paleontology
Nevoid basal-cell carcinoma syndrome (NBCCS) or Gorlin-Goltz syndrome is an autosomal dominant-inherited condition that exhibits high penetrance and variable expressivity; however, this disorder can arise spontaneously. In 1960, Gorlin and Goltz described the syndrome as a condition, comprising the principle triad of multiple basal cell carcinoma, odontogenic keratocysts, and skeletal anomalies. The diagnostic findings of NBCCS in four patients were studied and compared with other reports in the Indian population. Early diagnosis of this syndrome is important for counseling of patients to prevent the life-long complications of this syndrome which includes the malignancy, oro-maxillofacial deformation, and destruction; this may be reduced if the diagnosis and treatment are made feasible at the earliest.