This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Gorlin syndrome

Authoring team

Gorlin's Syndrome is a rare autosomal dominant condition characterised by a marked predisposition to developing multiple basal cell carcinomata. The description of basal cell carcinoma associated with congenital rib anomalies and acquired jaw cysts dates back to 1894 as quoted by Zvulunov et al (1). This association, along with calcification of falx cerebri, was established as a unique syndrome by Gorlin and Goltz in 1960. Therefore, it is also known as Gorlin-Goltz syndrome and Gorlin syndrome as well as nevoid basal cell carcinoma syndrome and basal cell nevus syndrome.

In recent years, the pathogenesis has become clearer with a clear link to chromosome 9. There are clear criteria for diagnosis.

Classical clinical features include:

  • multiple basal cell carcinoma lesions on the skin
  • palmar pits
  • cysts within the mandible
  • fused or bifid ribs
  • calcification of the falx cerebri
  • cataracts

Ref: A. Zvulunov, D. Strother, G. Zirbel et al., Nevoid basal cell carcinoma syndrome. Report of a case with associated Hodgkin?s disease. J Pediatr Hematol Oncol 17 (1995), pp. 66-70.


Create an account to add page annotations

Add information to this page that would be handy to have on hand during a consultation, such as a web address or phone number. This information will always be displayed when you visit this page

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

Connect

Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.