S3205 When Youth Offers No Shield: A Case of Pancreatic Cancer in a Young Adult With Neurofibromatosis Type 1

Introduction: Pancreatic neuroendocrine tumors (PNETs) are rare neoplasm, accounting for less than 2% of all pancreatic tumors. While pancreatic cancer is generally uncommon in younger individuals, its occurrence in this age group is not unheard of. PNETs can present in different ways. It can be functional or nonfunctional. This case report highlights a young patient who is a 26-year-old man diagnosed with nonfunctional PNET with a history of Neurofibromatosis type 1(NF1). Case Description/Methods: A 26-year-old man with neurofibromatosis type 1(NF1), a history of right below-knee amputation, and childhood Kawasaki disease presented with constant epigastric pain following Endoscopic ultrasound (EUS) and fine needle aspiration (FNA) of a pancreatic mass seen on computed tomography, prompted by a palpable abdominal mass. The initial computed tomography revealed a 3.9 × 4.6 × 3.2 cm soft tissue mass inseparable from the pancreatic head and the second portion of the duodenum. EUS demonstrated a hypoechoic pancreatic head mass with a 6 mm dilated main pancreatic duct with vascular involvement as superior mesenteric artery (SMA) encasement, and portal vein abutment. Labs showed total bilirubin 1.8 mg/dL, alkaline phosphatase 153 U/L, AST 25 U/L, ALT 13 U/L, and lipase 99 U/L. EUS findings raised concern for a main duct intraductal papillary mucinous neoplasm (IPMN) (T2N1Mx), but cytology confirmed a well-differentiated pancreatic neuroendocrine tumor (PNET). DOTATATE PET/computed tomography showed a 4.5 cm largely calcified mass with low uptake (SUV max 4.3) and no metastasis. He underwent Whipple procedure with cholecystectomy and biliary and pancreatic stent placements. At his 2-month follow-up, he remained asymptomatic and without complications. Discussion: Neurofibromatosis type 1 (NF1) is a genetic neurocutaneous disorder associated with various tumors, especially of the nervous system. While tumors like neurofibromas and optic gliomas are common in NF1, pancreatic tumors are rare and pose a diagnostic challenge. Pancreatic involvement in NF1 is diverse. Though uncommon, pancreatic neuroendocrine tumors (PNETs) have been reported in NF1, sometimes with atypical presentations, like cystic appearances. The pathogenesis of pancreatic tumors in NF1 remains insufficiently understood. This case report adds to the growing body of literature on the association between NF1 and pancreatic tumors, emphasizing the importance of incorporating pancreatic evaluation studies in the surveillance and management protocols for patients with NF1.