Introduction

Peutz–Jeghers syndrome (PJS) is traditionally managed with structured endoscopic surveillance targeting hamartomatous polyposis and the prevention of small-bowel obstruction. Conversely, despite a markedly elevated lifetime risk of pancreatic ductal adenocarcinoma (PDAC), pancreatic screening practices remain heterogeneous, and consensus on optimal imaging modalities or surveillance intervals is lacking. This discrepancy may lead clinicians to focus predominantly on gastrointestinal polyp management while inadvertently underrecognizing the pancreatic component of the syndrome (1).

Case Presentation

A 44-year-old patient with PJS was initially diagnosed in 1998 following segmental small-bowel resection for acute intestinal obstruction, with pathology confirming multiple hamartomatous polyps >2 cm. Serial colonoscopies, upper endoscopies, and double-balloon enteroscopies over subsequent years consistently demonstrated recurrent hamartomatous polyps in the jejunum, duodenum, and colon without dysplasia. Entero-MRI examinations in 2023 and February 2025 showed jejunal polyps <2 cm with no suspicious features. In March 2025, the patient presented with mild acute pancreatitis. Laboratory workup ruled out biliary, alcoholic, metabolic, autoimmune, and viral causes. CT confirmed acute edematous pancreatitis. A dedicated pancreatic MRI demonstrated dilation of the main pancreatic duct with abrupt corporeo-caudal stricture, secondary duct ectasia and focal parenchymal signal alteration. CA 19-9 raised up to 442 kU/L Endoscopic ultrasound identified a 20-mm hypoechoic lesion in the pancreatic body causing ductal narrowing with mild upstream dilatation, along with several distal microcysts. Fine-needle biopsy confirmed PDAC. Retrospective review of prior abdominal imaging (entero-MRI) revealed 2 cystic lesions in the pancreatic body suggestive of branch-duct IPMN. The patient underwent left pancreatectomy with splenectomy. Histopathologic staging showed pT2N1M0 PDAC, BRCA wild-type and KRAS-mutated. Postoperative management included adjuvant FOLFIRINOX chemotherapy

Conclusion

This case illustrates how the dominant focus on hamartomatous polyposis in PJS may mask the substantial risk of PDAC, leading to delayed recognition of pancreatic malignancy. The absence of harmonized, evidence-based pancreatic surveillance protocols persists despite the well-established high-risk profile of these patients. Our case underlines the need to re-evaluate existing surveillance frameworks and supports the integration of structured MRI- and EUS-based pancreatic screening to enable earlier detection and ultimately improve outcomes in individuals with PJS.