title: Mucoepidermoid carcinoma: A yet unreported cancer associated with familial adenomatous polyposis.
abstract: Turcot's syndrome is a rare clinical syndrome, characterized by the association between familial adenomatous polyposis (FAP) and a primary central nervous system tumour. Gardner's syndrome is characterized by the association between FAP and several tumour types such as multiple osteomas, fibromas, epidermoid cysts and desmoid tumours. We report here the case of a twenty-six year-old woman with a history of both Turcot's and Gardner syndromes. She had a family history of adenomatous polyposis with a mutation in the APC (Adenomatous Polyposis Coli) gene. At the age of 26, she presented a mucoepidermoid carcinoma of the right parotid gland in which the MECT1-MAML2 fusion was showed. We discuss the possible addition of this latter cancer type in the definition of Gardner's syndrome.
authors.affiliation: Department of Pathology, CHU Besançon, F-25000, France.
authors.forename: Arnault
authors.initials: A
authors.lastname: Cazorla
authors.affiliation: Department of Pathology, CHU Besançon, F-25000, France; Univ Franche-Comte, F-25000 Besançon, France.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Viennet
authors.affiliation: Service d'Anatomie Pathologique et Histologie-Cytologie, CHU Rangueil, 1 Avenue Jean Poulhès, TSA 50032, 31059 Toulouse Cedex 9, France; INSERM UMR 1037, F-31000 Toulouse, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Uro-Coste
authors.affiliation: Department of Pathology, CHU Besançon, F-25000, France; Univ Franche-Comte, F-25000 Besançon, France. Electronic address: svalmary@univ-fcomte.fr.
authors.forename: Séverine
authors.initials: S
authors.lastname: Valmary-Degano
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000473:pathology; D018277:Carcinoma, Mucoepidermoid / Q000473:pathology*; D015179:Colorectal Neoplasms / Q000473:pathology; D005260:Female; D005736:Gardner Syndrome / Q000473:pathology*; D017491:Genes, APC; D006801:Humans; D009154:Mutation / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009386:Neoplastic Syndromes, Hereditary / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000032:analysis; D010307:Parotid Neoplasms / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; C495418:MECT1-MAML2 fusion protein, human; D015514:Oncogene Proteins, Fusion
keywords: Gardner's syndrome; Mucoepidermoid carcinoma; Turcot's syndrome