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Gorlin Syndrome (NBCCS) - Updated Clinical Management Guidelines

Mitropoulos P, Norman R
Journal of the American Academy of Dermatology (JAAD) (2008)

JC: March 2026

This study provided updated clinical management guidelines for Gorlin syndrome (nevoid basal cell carcinoma syndrome), addressing surveillance, treatment of multiple BCCs, and genetic counseling. The guidelines incorporated recent evidence on hedgehog pathway inhibitors and emerging therapeutic approaches.

Take-Home Messages

  • Gorlin syndrome patients require lifelong skin surveillance and a multi-modal approach to managing their high BCC burden.
  • Hedgehog pathway inhibitors have emerged as important therapeutic options for Gorlin patients with numerous or inoperable BCCs.

Topic

BCC Management

Basal cell carcinoma treatment, hedgehog inhibitors, recurrence

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Abstract

An updated review of management of nevoid basal cell carcinoma syndrome (NBCCS) is presented. An ideal treatment of NBCCS does not exist, and surgical intervention has been the most commonly used treatment, as it provides excellent cure rates. However, patients with NBCCS typically present with a large number of basal cell carcinomas (BCCs) with repeated occurrence throughout life. Surgical intervention, although efficient, may be too painful and discomforting, especially if it has to be freq...

Literature review only. This summary is an editorial interpretation and may not reflect the complete findings of the original publication. Always refer to the full-text article for clinical decision-making.