Patients with Gorlin syndrome who develop multiple basal cell carcinomas represent a distinct clinical population. The inherited, syndromic context fundamentally changes how each lesion is evaluated and managed.

Clinical Scenario

Gorlin syndrome is a rare, autosomal dominant familial cancer syndrome with a high degree of penetrance and variable expression. Multiple BCCs arising in this setting should be considered as locally advanced basal cell carcinomas and treated accordingly — a designation that carries specific implications for which interventions are appropriate and which are not.

Treatment Approach

Certain approaches used for sporadic BCC may be applicable, selected and adapted based on lesion-specific factors — but with meaningful constraints on the role of each modality and at least one category of treatment that must be avoided in this population entirely.

Full criteria, modality selection, and contraindications are in the complete protocol.

References

DOI: 10.1016/j.ejca.2023.113254

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.
Depending on the number, size, location, and subtype of BCCs, treatment approaches used for sporadic BCC, besides radiotherapy, can be considered.
The benefit of surgery should be weighed against the high risk of recurrent or newly developing BCCs and should be limited to solitary high-risk BCCs.
Vice versa, small or superficial variants in low-risk areas outside the face may be managed by topical treatments and/or by careful watchful waiting during follow-up.
Radiotherapy must be avoided because of the carcinogenic effect of X-rays resulting in the formation of new BCCs.

View source ↗

Treatment of Basal Cell Carcinoma of Skin in Gorlin Syndrome with Multiple BCCs

Clinical Scenario

Treatment Approach

References