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Therapeutic Approach

Basal cell nevus syndrome (BCNS, a.k.a. basal cell carcinoma nevus syndrome, BCCNS, Gorlin syndrome, Gorlin-Goltz syndrome) is an autosomal dominant genetic cancer syndrome caused by germline mutations in PTCH1, PTCH2, or SUFU. The most significant manifestation is dozens to hundreds of basal cell carcinomas, and treatments are directed at prevention or management of these cancers. Other manifestations include odontogenic keratocysts of the jaw (typically managed by oral surgery), palmar and plantar pits, frontal bossing, medulloblastoma, cardiac or ovarian fibroma, cleft lip/palate, and radiographic abnormalities including bilamellar calcification of the falx cerebri and bifid ribs. Therapeutic approach starts with rigorous sun protection and close dermatologic follow-up to detect BCCs at early stages. Small BCCs can be managed with minimally invasive surgical procedures such as ED+C, excision or Mohs surgery, or with topical therapy or PDT for superficial lesions. Patients with locally advanced BCC not amenable to surgery, metastatic BCC, or too many lesions to be managed surgically can be treated with vismodegib or sonidegib, but most patients have difficulty tolerating these medications for longer than 1 year. BCNS patients should not be treated with radiation therapy for any indication as this leads to innumerable BCCs. See Table 102-1.

Table 102-1BCNS Treatment Table

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