Gorlin-Goltz syndrome update, on the subject of a case  in the Morelia Childrens Hospital

Cristóbal Landa Román; Francisco Javier Gómez Pamatz

2017, Number 2

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Rev ADM 2017; 74 (2)

Gorlin-Goltz syndrome update, on the subject of a case in the Morelia Children's Hospital

Landa RC, Gómez PFJ

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Language: Spanish
References: 10
Page: 94-99
PDF size: 745.37 Kb.

ABSTRACT

Robert Gorlin a mouth researcher trained pathologist Minnesota and Robert Goltz a dermatologist described this syndrome in 1960. It is an autosomal dominant disorder, caused by the Patched 1 gene (PTCH1) located on chromosome 9q223 characterized by developmental defects and a high predisposition to cancer. The incidence is 1/56,000 and 1/221,000 patients. The condition is characterized by the development of basal cell carcinomas, odontogenic keratocystic and skeletal malformations. Due to its high predisposition to the development of aggressive basal cell carcinomas should be early and timely diagnosis for a favorable prognosis.

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