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Article in English | MEDLINE | ID: mdl-18417377

ABSTRACT

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder characterized by a wide range of clinical signs and symptoms. The major criteria for the diagnosis are multiple cutaneous basal cell carcinomas, multiple odontogenic keratocysts of the jaw, palmar and plantar pits, and skeletal abnormalities. Here, we report an unusual case of NBCCS in a 68-year-old woman with late onset of clinical signs and symptoms and with an associated ameloblastoma. Only 4 other cases of this unusual association have been reported.


Subject(s)
Ameloblastoma/etiology , Basal Cell Nevus Syndrome/complications , Jaw Neoplasms/complications , Maxillary Neoplasms/etiology , Aged , Basal Cell Nevus Syndrome/genetics , Chromosomes, Human, Pair 9 , Female , Humans , Jaw Neoplasms/genetics , Patched Receptors , Patched-1 Receptor , Receptors, Cell Surface/genetics
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