Nevoid basal cell carcinoma syndrome: A case report
Keywords:
nevoid basal cell carcinoma syndrome, Gorlin syndrome, basal cell carcinoma, odontogenic keratocystAbstract
Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare autosomal dominant inherited condition mainly caused by mutations in the patched tumor suppressor gene (PTCH1). Prevalence rates vary by region. This syndrome is characterized by multiple basal cell carcinomas, as well as odontogenic keratocysts (OKC) in the jaw and other abnormalities involving the skeletal, central nervous system, ophthalmic, endocrine, and genital systems. This syndrome may be diagnosed by a plastic surgeon or dermatologist in patients who present with multiple basal cell carcinomas or by a dentist in patients who present with OKC. Early diagnosis and a multidisciplinary approach to treatment are important for decreasing the progression and severity of this syndrome, as well as improving the patient’s quality of life. Here, we report a case of nevoid basal cell carcinoma syndrome with a suspected high-grade glioma in a 38-year-old Thai man.
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