Treatment of Basal Cell Carcinoma of Skin in Gorlin Syndrome with Multiple BCCs
This protocol addresses the specific clinical scenario of basal cell carcinoma of skin arising in the setting of Gorlin syndrome, where patients present with multiple basal cell carcinomas requiring systematic management.
Clinical Scenario
Gorlin syndrome is a rare, autosomal dominant familial cancer syndrome with a high degree of penetrance and variable expression. Multiple BCCs in patients with Gorlin syndrome should be considered as locally advanced BCCs and treated accordingly — this population requires a distinct treatment approach from sporadic BCC.
Treatment Approach
In selected patients with Gorlin syndrome and multiple BCCs, a Hedgehog pathway inhibitor may be considered as part of the therapeutic strategy — delivered orally.
Full regimen details, dosing, sequencing, and selection criteria are in the complete protocol →
References
Gorlin syndrome is a rare, autosomal dominant familial cancer syndrome with a high degree of penetrance and variable expression.
Multiple BCCs in patients with Gorlin syndrome should be considered as laBCCs and treated accordingly.
In selected patients, treatment with Hedgehog inhibitors can be considered.
The oral dose is 150 mg/day for vismodegib and 200 mg/day for sonidegib.
DOI: 10.1016/j.ejca.2023.113254
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