Abstract: Peutz-Jeghers syndrome: a study of long-term surgical morbidity and causes of mortality

"Patients with Peutz-Jeghers syndrome (PJS) require lifelong multidisciplinary care for gastrointestinal polyposis and increased risk of cancer.

...The cause of death was unknown in 4 patients, but was due exclusively to malignancies in all other patients, most commonly due to metastatic gynecologic cancer (5)...."