Details

Autor(en) / Beteiligte

• Cazorla, Arnault
• Viennet, Gabriel
• Uro-Coste, Emmanuelle
• Valmary-Degano, Séverine

Titel

Mucoepidermoid carcinoma: A yet unreported cancer associated with familial adenomatous polyposis

Ist Teil von

• Journal of cranio-maxillo-facial surgery, 2014-04, Vol.42 (3), p.262-264

Ort / Verlag

Scotland: Elsevier Ltd

Erscheinungsjahr

2014

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.