Clinical practice guidelines for the management of basal cell carcinoma in Gorlin syndrome.
Recommended Citation
Cahn BA, Zhang R, Sidwell AM, Adam CM, Tiv J, Murrar R, Koh J, Griffin T, Nadir U, Yi MD, Ahmed A, Ma M, Koza E, Haq M, Shi V, Bale AE, Lewin JM, Christensen SR, Waldman A, Harmon CB, Alt-Holland A, Arkin LM, Morrison JC, Bogart K, Gregory MD, Lohman ME, Rossi A, Marghoob AA, Goldberg L, Jiang SB, Giordano CN, Choi JN, Teng JMC, Mittal BB, Vidimos AT, Leffell DJ, Xu YG, Haber R, Storrs PA, Tsoukas M, Samie FH, Bickers DR, Nehal KS, Mosterd K, Billingsley EM, Epstein EH, Jr., Elston D, and Alam M. Clinical practice guidelines for the management of basal cell carcinoma in Gorlin syndrome. J Am Acad Dermatol 2025;94(2):485-494.
Document Type
Article
Publication Date
2-1-2026
Publication Title
Journal of the American Academy of Dermatology
Keywords
Humans, Basal Cell Nevus Syndrome, Skin Neoplasms, Basal Cell Carcinoma, Quality of Life, Evidence-Based Medicine, Combined Modality Therapy, Delphi Technique, Decision Making, Shared
Abstract
BACKGROUND: Gorlin syndrome (GS) is a rare genetic disorder characterized by a predisposition to developing numerous basal cell carcinomas (BCCs) throughout life. The absence of specific clinical guidelines for managing BCCs in GS has resulted in fragmented care and inconsistent treatment approaches.
OBJECTIVE: To develop evidence-based guidelines for managing BCCs in GS, addressing both clinical and psychosocial challenges.
METHODS: A multidisciplinary panel employed a modified Grading of Recommendations Assessment, Development and Evaluation approach, integrating systematic reviews, expert surveys, patient interviews, and Delphi consensus rounds to formulate recommendations.
RESULTS: The final guidelines include 47 recommendations spanning topical therapies, systemic treatments, surgical interventions, and multimodal strategies. Additional recommendations emphasize shared decision-making, comprehensive monitoring, and psychosocial support to address the chronic nature of BCCs in GS. Specific therapies, including hedgehog inhibitors and field treatments, are recommended to reduce surgical fatigue and enhance quality of life.
LIMITATIONS: Given the scarcity of GS-specific data, expert consensus informed several recommendations, highlighting the need for ongoing research to strengthen the evidence base.
CONCLUSION: These guidelines provide a structured framework for improving BCC management in GS, thereby enhancing clinical outcomes and patient quality of life. This process serves as a model for creating patient-centered guidelines in rare conditions with limited evidence.
Medical Subject Headings
Humans; Basal Cell Nevus Syndrome; Skin Neoplasms; Basal Cell Carcinoma; Quality of Life; Evidence-Based Medicine; Combined Modality Therapy; Delphi Technique; Decision Making, Shared
PubMed ID
41396574
ePublication
ePub ahead of print
Volume
94
Issue
2
First Page
485
Last Page
494
