Cargando…

Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Saxena, Shikha, Sundaragiri, Krishna Sireesha, Bhargava, Akshay, Sankhla, Bharat
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802829/
https://www.ncbi.nlm.nih.gov/pubmed/33456241
http://dx.doi.org/10.4103/jomfp.JOMFP_358_19
Descripción
Sumario: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.