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South African Journal of Child Health

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Gorlin syndrome

JA Geel, KG Bennett, JM Rigby, JE Poole

Abstract


Naevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is an autosomal dominant syndrome of developmental anomalies associated with an increased risk of malignancies. Patients have multiple lesions, which may be subtle, and the diagnosis can easily be missed, leading to sub-optimal follow-up. Despite its infrequency clinicians may benefit from familiarity with the syndrome, as these patients are hypersensitive to radiation and prone to develop multiple malignancies. Patients can present to paediatricians, oncologists, maxillofacial surgeons, radiation oncologists and dermatologists, and it will be to the benefit of the patient with this syndrome for these specialists to have a working knowledge of this rare but fascinating disorder.



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