Aman Mohammad S, Das Bidhan C, Islam Aminul, Arefin Murshidul, Gupta Satya N
Department of Hepatobiliary, Pancreatic and Liver Transplant Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, BGD.
Cureus. 2023 May 5;15(5):e38588. doi: 10.7759/cureus.38588. eCollection 2023 May.
Neuroendocrine tumors (NETs) of the ampulla of Vater are extremely rare. Here, we discuss the clinical presentation, diagnostic challenges, and treatment options of a recently experienced case of NET of the ampulla of Vater in light of the literature. A 56-year-old woman presented with recurrent upper abdominal pain. Ultrasonography (USG) of the whole abdomen showed multiple gallstones along with a dilated common bile duct (CBD). For evaluating the dilated CBD, a magnetic resonance cholangiopancreatography was performed, which revealed the double-duct sign. Subsequently, an upper gastrointestinal endoscopy showed a bulged-out ampulla of the Vater. Biopsy and histopathological examination of the growth yielded the diagnosis of adenocarcinoma. A Whipple procedure was performed. Macroscopically, a 2 cm growth was noted involving the ampulla of Vater, and microscopic findings were consistent with a well-differentiated NET, grade 1 (low grade). The diagnosis was further confirmed by immunohistochemical staining (pan-cytokeratin positive, synaptophysin positive, and focally chromogranin positive). Her postoperative course was uneventful except for delayed gastric emptying. A detailed evaluation and a high index of suspicion are required for the diagnosis of this rare tumor. Treatment is relatively easier after a proper diagnosis.
壶腹神经内分泌肿瘤（NETs）极为罕见。在此，我们根据文献探讨一例近期遇到的壶腹NET病例的临床表现、诊断挑战及治疗方案。一名56岁女性因反复上腹部疼痛就诊。全腹超声检查（USG）显示多发胆结石伴胆总管（CBD）扩张。为评估扩张的CBD，进行了磁共振胰胆管造影，显示双管征。随后，上消化道内镜检查显示壶腹膨出。对肿物进行活检及组织病理学检查，诊断为腺癌。遂行Whipple手术。肉眼可见一个2 cm肿物累及壶腹，显微镜检查结果符合高分化NET，1级（低级别）。免疫组化染色（全细胞角蛋白阳性、突触素阳性、局灶嗜铬粒蛋白阳性）进一步确诊。除胃排空延迟外，她术后恢复顺利。对于这种罕见肿瘤的诊断，需要进行详细评估并保持高度怀疑。正确诊断后治疗相对容易。
