Metachronous triple cancer associated with Peutz-Jeghers syndrome treated with curative surgery: a case report

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. It is well known that individuals with PJS are at an increased risk of cancer in a variety of organs.

Here, we present a patient with PJS who achieved long-term survival by undergoing repeat curative surgery for metachronous triple cancer. Her medical history included hilar cholangiocarcinoma and cervical carcinoma; curative surgery was performed for both conditions. On annual follow-up, the level of carcinoembryonic antigen was elevated at 6.9 ng/ml. Enhanced computed tomography revealed a cystic tumor consisting of mural nodules at the pancreatic head; the maximal diameter was 15 mm. Magnetic resonance imaging clearly demonstrated the tumor with low intensity on T1-weighted images and high intensity on T2-weighted images. Endoscopic ultrasound sonography showed a high echoic tumor at the pancreatic head, which was confirmed as adenocarcinoma by fine-needle aspiration biopsy. The preoperative diagnosis was intraductal papillary mucinous carcinoma (IPMC; T1N0M0, stage IA). Subtotal stomach-preserving pancreaticoduodenectomy was performed and the final diagnosis was IPMC, stage 0 (TisN0M0).

Aggressive surgery for metachronous triple cancer resulted in good long-term prognosis. Continuous and systematic follow-up would allow the detection of malignancy at an early stage and make treatment with curative surgery possible.