Case Reports
Gorlin syndrome
Jennifer Ann Geel, Kate Gwynneth Bennett, Jonathan Mark Rigby, Janet Elizabeth Poole
Abstract
NNaevoid 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.
Authors' affiliations
Jennifer Ann Geel, Charlotte Maxeke Johannesburg Academic Hospital
Kate Gwynneth Bennett, Wits Donald Gordon Medical Centre, Charlotte Maxeke Johannesburg Academic Hospital
Jonathan Mark Rigby, National Health Laboratory Service
Janet Elizabeth Poole, Charlotte Maxeke Johannesburg Academic Hospital,Wits Donald Gordon Medical Centre
Cite this article
South African Journal of Child Health 2011;5(1):21.
Article History
Date submitted: 2011-01-28
Date published: 2011-04-13
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