Acute pancreatitis associated with periampullary hamartomatous polyp in Peutz-Jeghers syndrome

We report the case of an 11-year-old girl with Peutz-Jeghers syndrome (PJS) and a history of bilateral oophorectomy for a sex cord-stromal tumor, who presented with acute abdominal pain, nausea, and vomiting. Laboratory findings indicated acute pancreatitis, and imaging revealed pancreatic enlargement with a large lobulated polypoid lesion (6.2 × 3.8 cm) in the periampullary duodenum, causing biliary and pancreatic ductal dilatation without gallstones. Upper endoscopy confirmed the lesion, and surgical duodenotomy with polypectomy and cholecystectomy was performed. Histopathological analysis identified a hamartomatous polyp consistent with PJS. No other etiologies for pancreatitis were found. This case highlights a rare but clinically significant complication of PJS, where a periampullary hamartomatous polyp caused obstructive pancreatitis. Accurate diagnosis through imaging and endoscopy, followed by surgical intervention, led to complete resolution of symptoms and stable follow-up.