INTRODUCTION Keratocystic odontogenic tumours raise particular interest, because of their high recurrence rate and association with nevoid basal cell carcinoma syndrome. OBJECTIVE To analyze the clinical and histopathological features of all cases diagnosed as keratocystic odontogenic tumour in a Brazilian population. METHODS A total of 64 keratocystic odontogenic tumours, arising in forty-...

We employed polymerase chain reaction and DNA sequencing analysis to characterize the PTCH gene in a Japanese nevoid basal cell carcinoma syndrome (NBCCS) patient suffering from meningioma, multiple basal cell carcinoma and epidermal cysts. Direct sequence analyses revealed a novel single base deletion at nucleotide 2613 in exon 16 (2613delC) in one PTCH allele, resulting in the frame shift and...

Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogeni...

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare condition, in which the skin of the nipple and/or areola becomes diffusely thickened, hyperpigmented, and covered with filiform or papular warty excrescences. Although it affects both men and woman, approximately 80% of cases ocur in women. Pathogenic mechanisms have been proposed but mainly unsubstantiated. Moreover, specific trea...