Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study

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Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study

1 Department of Stomatology, São Paulo University, São Paulo, SP, Brazil 2 Department of Stomatology, A. C. Camargo Hospital, São Paulo, SP, Brazil 3 Department of Oncogenetics, A. C. Camargo Hospital, São Paulo, SP, Brazil 4 Department of Anatomical Pathology, A. C. Camargo Hospital, São Paulo, SP, Brazil 5 Department of Stomatology, University of São Paulo, Department of Stomatology, A. C. Ca...

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Nevoid Basal Cell Carcinoma Syndrome: Report from the Zurich Nevoid Basal Cell Carcinoma Syndrome Cohort.

BACKGROUND Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin-Goltz syndrome) presents various symptoms and can disfigure patients. The estimated prevalence is around 1:100,000. OBJECTIVE To systematically investigate the clinical manifestations of NBCCS patients of the Zurich register and compare them with those described in 4 epidemiological studies performed in other countries. METHODS ...

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[Gorlin syndrome (nevoid basal cell carcinoma syndrome)].

CLINICAL CASE A 77 year-old male patient with Parkinson's disease and senile dementia had many facial basal cell carcinomas and an ectropion of the left eye. When he experienced respiratory difficulty he was diagnosed to have an ameloblastoma in left nostril requiring surgery. DISCUSSION Gorlin syndrome is an autosomal dominant condition characterized by basal cell carcinomas, and skeletal an...

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Nevoid basal cell carcinoma syndrome (Gorlin syndrome)

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, with a male-to-female ratio of 1:1. Main clinical manifestations include multiple basal cell carcinomas (BCCs), odontogenic keratocysts of...

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Novel PTCH1 Mutations in Patients with Keratocystic Odontogenic Tumors Screened for Nevoid Basal Cell Carcinoma (NBCC) Syndrome

Keratocystic odontogenic tumors (KCOTs) are cystic tumors that arise sporadically or associated with nevoid basal cell carcinoma syndrome (NBCCS). NBCCS is a rare autosomal dominantly inherited disease mainly characterized by multiple basal cell carcinomas, KCOTs of the jaws and a variety of other tumors. PTCH1 mutation can be found both in sporadic or NBCCS associated KCOTs. The aim of the cur...

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ژورنال

عنوان ژورنال: Brazilian Journal of Oral Sciences

سال: 2013

ISSN: 1677-3225

DOI: 10.1590/s1677-32252013000100006