Abstract
Naevoid basal cell carcinoma syndrome (NBCCS), also known as basal cell naevus syndrome or Gorlin syndrome, is an autosomal dominant tumour-predisposing disorder characterised by two hallmark features: jaw cysts (odontogenic keratocysts) and multiple cutaneous basal cell carcinomas. Other features include skeletal anomalies, large head size and an increased risk of developing childhood-onset medulloblastoma and other types of cancer. Awareness among ophthalmic specialists is important as periocular basal cell carcinomas may be the presenting feature in a minority of cases. Familiarity with the diagnostic criteria and maintaining a high index of suspicion are key to optimising the management of affected individuals. Regular dermatologic assessment is recommended and although life expectancy is not significantly altered, morbidity from complications can be substantial.
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