Abstract
Importance: Basal Cell Carcinoma (BCC) represents a majority of skin cancers (80%), and can be locally invasive and difficult to treat in patients with Nevoid Basal Cell Carcinoma syndrome (Gorlin syndrome) or in patients with multiple BCCs in high-risk areas. Observations: Long-term use of Vismodegib was tolerated well in the 2 patients in this case series and was able to shrink large BCCs by up to 80.5%. Conclusions and Relevance: In Gorlin syndrome and high risk non-resectable BCCs, vismodegib can provide neoadjuvant treatment. In turn, allowing for Mohs surgery to be a viable option with smaller surgical defects with good cosmetic outcomes.
Highlights
Gorlin syndrome is an autosomal dominant disorder that usually presents with patients developing Basal Cell Carcinoma (BCC) in adolescence or early adulthood
If the gene is mutated the cell cycle is not suppressed in turn leading to the high occurrence of BCCs
Patient was referred to our Mohs surgery clinic for evaluation and resection of cancers after being on vismodegib treatment for 13 months shrinking the tumor from 2.0 cm by 1.8 cm to 1.0 cm by 0.7 cm (Figures 1a and 1b). tumor was resected using traditional Mohs surgery technique in 4 stages leaving a defect of 2 cm by 1.6 cm (Figure 1c)
Summary
Gorlin syndrome is an autosomal dominant disorder that usually presents with patients developing BCCs in adolescence or early adulthood. If the gene is mutated the cell cycle is not suppressed in turn leading to the high occurrence of BCCs. Vismodegib acts as a competitive antagonist of the smoothened receptor part of the Sonic hedgehog-signaling pathway. Patient was started on 150 mg/day vismodegib for his extensive BCCs by oncology.
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