Abstract
BackgroundPeutz–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.Case presentationHere, 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).ConclusionsAggressive 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.
Highlights
Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis
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
Summary
Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder with variable inheritance Characteristics of this disorder are hamartomatous polyps in the gastrointestinal tract and pigmented mucocutaneous lesions [1]. Case presentation A 62-year-old woman was admitted to Onomichi General Hospital in 2017 with a suspected pancreatic tumor. Her medical history included hilar cholangiocarcinoma (poorly differentiated adenocarcinoma, intermediate type, INFß, pat Bp, ly, v0, pn0, hinf, hm0, dm0, em2, 3 cm × 2 cm × 2 cm, T3N0M0 stage IIA, UICC version 6) at the age of 53 years and cervical cancer at the age of 55 years.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
