Supplementary Material for: Obstructive Jaundice caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life and no cases were reported in < 20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasis.
Case presentation: An 18-year-old man presented with a 3-month history of upper abdominal pain and jaundice. Abdominal ultrasound showed a dilated common bile duct and endoscopic retrograde cholangiopancreatography revealed two duodenal polypoid lesions; one of them overlying the ampulla of Vater, with erythematous and ulcerated surface. Histopathological examination confirmed the diagnosis of NET grade 1. Octreotide scan revealed 2 para-aortic lymph nodes with intense radiotracer uptake. The patient had undergone Whipple surgery with para-aortic lymph node dissection. Histopathological examination of the surgical specimens was confirmatory of NET grade 2, and paraganglioma in a few of the dissected lymph nodes. Postoperatively, the patient was kept on monthly intramuscular octreotide. Follow-up gallium-68 dotatate being unremarkable apart from an avid left para-aortic lymph node which is showing stability over 12 months of follow-up.
Conclusion: This case demonstrates that NETs of the ampulla of Vater can present at a very young age. Radical surgical excision with extended lymph node dissection and postoperative octreotide is associated with better patient outcomes and survival.
Keywords: Ampulla of Vater, Lymph node metastases, Neuroendocrine tumor, Pancreaticoduodenectomy, Small intestinal