Title: Classical Manifestations of Gorlin-Goltz Syndrome in an Operated Case of Medulloblastoma: A Case Report
Authors: Dr Dheeman Sarkar, Dr Sanjiv Patel, Dr Sapna Patel, Dr Akhilesh Patel
DOI: https://dx.doi.org/10.18535/jmscr/v8i10.22
Abstract
Gorlin-Goltz syndrome (also known as the basal cell nevus syndrome, nevoid basal cell carcinoma syndrome, or just Gorlin syndrome) is a rare phakomatosis characterized by multiple odontogenic keratocysts (OKC), multiple basal cell carcinomas (BCC) and other abnormalities. It is inherited in an autosomal dominant manner which shows a high level of penetrance and variable expressiblity. Early diagnosis is essential as it may progress to basal cell neoplasm or carcinomas. It has been rarely reported in India. We report here one such patient, diagnosed at a tertiary care hospital.
References
- Ana R Casaroto, Daniela CN Rocha Loures, Vanessa S Lara.Early diagnosis of gorlin- goltz syndrome: a case report. Head and Face Medicine 2009;
- Padma Pandeshwar,K. Jayanthi, and D. Mahesh. Gorlin-goltz syndrome. Dentistry 2012;
- Brelier F et al. Ultraviolet responses of Gorlin syndrome primary skin cells. Dermatology, 2008; 445-452
- Ian Bickle, Gagandeep Singh et al.Gorlin-goltz syndrome. Radiology. 2010; 5794
- Burgdorf W. Robert J. Gorlin, Löser C, Plewig G, Hrsg, Pantheon derdermatologie. 2008: 362–366
- Burgdorf WH, Padilla RS, Hordinsky M. In memoriam: Robert W. Goltz. Dermatology. 2014; 71:163–165
- Lo Muzio. Nevoid basal cell carcinoma syndrome (Gorlin syndrome). RareDiseases. 2008;
- E. Kimonis, A. M. Goldstein, B. Pastakia et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Medical Genetics. 1997;
- Santana, B. K. Yashodha Devi, and D. Jatti. Gorlin Goltz syndrome—a case report. Dental. 2011;
- Ljubenovi, D. Ljubenovi, I. Bini, D. Jovanovi, and M. Stanojevi. Gorlin-Goltz syndrome. Dermatology. 2007;