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

引言：壶腹神经内分泌肿瘤（Neuroendocrine Tumors, NETs）通常于50~60岁年龄段确诊，目前尚无20岁以下患者的相关报道。本研究报道一例罕见的极低龄发病病例，为累及Vater壶腹（ampulla of Vater）的高分化神经内分泌肿瘤，伴淋巴结转移。

病例报告：一名18岁男性患者，因上腹痛及黄疸病史3个月就诊。腹部超声检查显示胆总管扩张，内镜逆行胰胆管造影（endoscopic retrograde cholangiopancreatography, ERCP）可见两处十二指肠息肉样病变，其中一处覆盖于Vater壶腹表面，黏膜呈红斑样且伴溃疡形成。组织病理学检查确诊为1级神经内分泌肿瘤。奥曲肽（octreotide）显像可见2枚主动脉旁淋巴结存在显著的放射性摄取增高。患者接受了Whipple手术并行主动脉旁淋巴结清扫。手术标本的组织病理学检查证实为2级神经内分泌肿瘤，且部分清扫的淋巴结可见副神经节瘤。术后患者接受每月一次的肌内注射奥曲肽治疗。随访期间的镓-68 dotatate（gallium-68 dotatate）显像结果无明显异常，仅见左侧主动脉旁淋巴结摄取增高，该病灶在12个月的随访中保持稳定。

结论：本病例表明，Vater壶腹神经内分泌肿瘤可于极低龄阶段发病。采用扩大淋巴结清扫的根治性手术切除联合术后奥曲肽治疗，可改善患者的预后与生存情况。

关键词：Vater壶腹、淋巴结转移、神经内分泌肿瘤、胰十二指肠切除术、小肠