title: Nevoid basal cell carcinoma syndrome. A 15-year follow-up of cases in Ottawa and the Ottawa Valley.
abstract: The nevoid basal cell carcinoma syndrome is an autosomal dominant condition that consists of multiple basal cell carcinomas of the skin, odontogenic keratocysts, pits of the hands and feet (especially the palms and soles), various ectopic calcifications, notably lamellar calcification of the falx cerebri and skeletal anomalies. As well as these major findings, there are many other associated abnormalities. This paper is a 15-year follow-up study on twelve cases from the Ottawa Valley with details on nine new cases discovered during that time. Sixteen were males; 5, females. The number of basal cell cancers requiring removal varied greatly from patient to patient. There was one patient with a horror lesion; none had metastatic disease.
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D002280:Basal Cell Carcinoma / Q000175:diagnosis*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009864:Ontario; D010375:Pedigree; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology