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。本文报告1例罕见的极年轻发病病例，为累及壶腹（ampulla of Vater）的高分化神经内分泌肿瘤伴淋巴结转移（lymph node metastasis）。
病例介绍：患者为18岁男性，有3个月上腹痛及黄疸病史。腹部超声显示胆总管扩张，内镜逆行胰胆管造影发现2处十二指肠息肉样病变，其中一处覆盖于壶腹（ampulla of Vater）表面，黏膜呈红斑样伴溃疡形成。组织病理学检查确诊为NET 1级。奥曲肽扫描可见2处主动脉旁淋巴结存在放射性示踪剂高摄取。患者接受了胰十二指肠切除术（Whipple手术）联合主动脉旁淋巴结清扫术。术后标本的组织病理学检查证实为NET 2级，且部分清扫的淋巴结可见副神经节瘤。术后患者每月接受肌内注射奥曲肽治疗。随访期间的镓-68 DOTATATE显像无明显异常，仅左侧主动脉旁淋巴结呈高摄取状态，经12个月随访未见进展。
结论：本病例表明，壶腹（ampulla of Vater）神经内分泌肿瘤可于极年轻年龄段发病。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，可改善患者预后与生存期。
关键词：壶腹（ampulla of Vater）、淋巴结转移、神经内分泌肿瘤、胰十二指肠切除术、小肠