Peutz-Jeghers syndrome and cancer

A 27-year-old white woman with Peutz-Jeghers syndrome (PJS) associated with a rare but distinctive ovarian tumor, the sex cord tumor with annular tubules (SCTAT), is discussed. Pertinent oral manifestations of PJS and chemotherapy complications are illustrated by the case report. Traditionally, patients exhibiting PJS have not been considered at increased risk of cancer development. This case, coupled with a review of the recent literature, suggests that these patients are, indeed, at increased risk of malignant lesions of several organ systems.