Basal Cell Nevus Syndrome in a 9-Year-Old Boy

Adam Taintor, MD


November 02, 2011

Management of Basal Cell Nevus Syndrome

Treatment of basal cell nevus syndrome requires a multifaceted approach. Patients are advised to avoid ionizing and ultraviolet radiation by following sun precautions, including avoiding sun during peak hours, regularly using sunscreen, and wearing sun protective clothing. Because odontogenic cysts have a tendency to invade surrounding structures, they may need to be surgically excised and tooth extraction and bone grafting may be required. Basal cell carcinomas that are symptomatic or located in high-risk areas should be managed surgically, with electrodessication and curretage, wide excision, or Mohs micrographic surgery. Nonsurgical options include cryotherapy, photodynamic therapy with aminolevulenic acid, or topical treatments with 5-fluorouracil or imiquimod. Another promising treatment on the therapeutic horizon is vismodegib,[4] an investigational antagonist of the smoothened receptor and downregulator of the SHH pathway.

Case Outcome

The 9-year-old boy with basal cell nevus syndrome is being followed regularly for new lesions, with plans to surgically remove any larger lesions, lesions in high-risk areas, and symptomatic or recurrent tumors. The family has been advised that the boy should follow sun precautions, including avoiding the sun, applying sunscreen regularly, and wearing sun protective clothing.


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