Kevin Yauy, Marion Imbert-Bouteille, Virginie Bubien, Clothilde Lindet-Bourgeois, Gauthier Rathat, Helene Perrochia, Gaëtan MacGrogan, Michel Longy, Didier Bessis, Julie Tinat, Stéphanie Baert-Desurmont, Maud Blanluet, Pierre Vande Perre, Karen Baudry, Pascal Pujol, Carole Corsini
Cowden syndrome (CS) is an autosomal dominant mendelian disease related to germline pathogenic variants affecting the PTEN -gene. CS is characterized by macrocephaly, mucocutaneous lesions, and an increased risk of breast and thyroid cancers. Rare ovarian cancer cases (mostly embryonic tumors) associated with PTEN have been described in the literature, but no current CS guidelines are available for ovarian cancer risk management. We report on a woman diagnosed with ovarian clear cell carcinoma (OCCC) at 28 years of age...
January 2019: Journal of the National Comprehensive Cancer Network: JNCCN