Pancreatic cancer as the fourth cancer in a patient with Peutz-Jeghers syndrome and a history of familial pancreatic cancer.

摘要

Peutz-Jeghers syndrome (PJS) is an au-tosomal, dominantly inherited disease characterized by hamartomatous polyps of the gastrointestinal tract and pigmented maculae of the lips and buccal mucosa. The risk of cancer associated with PJS has recently become widely acknowledged.1 Herein, a patient with PJS and a history of familial pancreatic cancer who developed pancreatic cancer as a fourth cancer is reported. The patient was a 57-year-old woman. Her mother had PJS who died of volvulus at 33 years of age. She had 2 brothers, also with PJS. The elder brother died of lung cancer (adenocarcinoma) at 48 years of age. The second brother developed a 7-cm-sized, locally advanced cancer of the head of the pancreas (poorly differentiated tubular adenocarcinoma) at 46 years of age. Despite chemoradiotherapy, he died of the cancer 1 year later.