Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report.

Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report.
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DOI:
10.1186/s12876-021-02019-6
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发表时间:
2022-01-08
影响因子:
2.4
通讯作者:
Shang D
Shang D
中科院分区:
医学4区
文献类型:
--
作者:
Zhang B;Li S;Sun Z;Chen X;Qi B;Zhang Q;Zhang G;Shang D

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神经内分泌肿瘤(NETs)起源于神经内分泌细胞,在胆道中极为罕见。目前,还没有诊断和治疗胆道NET的指南。我们提出了一个与NETs G1的肝门胆管和她的治疗挑战。一位24岁女性因无痛性黄疸及皮肤瘙痒来我科就诊,术前诊断为胆管癌II型。她接受了Roux-en-Y肝管空肠吻合术,切除了肝外胆管树和根治性淋巴结切除术。出乎意料的是,术后病理和免疫组化检查显示门周胆管NETs G1,并在显微镜下侵犯切除的右肝管。随后患者接受了3个周期的辅助化疗(吉西他滨和替加氟-吉美拉西-奥替拉西钾胶囊)。目前,该患者已随访24个月,无复发或疾病进展。由于其罕见性,我们对胆道NET知之甚少。目前还没有诊断和治疗胆道NETs的指南。我们报告了一例肝门周胆管G1伴R1切除的NETs,据我们所知,这是第一次报告。应登记更多关于胆道NET的信息。
Neuroendocrine tumors (NETs) arise from neuroendocrine cells and are extremely rare in the biliary tract. Currently, there are no guidelines for the diagnosis and treatment of biliary NETs. We presented a case with NETs G1 of the hilar bile duct and the challenges for her treatment. A 24-year-old woman was presented to our department with painless jaundice and pruritus, and the preoperative diagnosis was Bismuth type II hilar cholangiocarcinoma. She underwent Roux-en-Y hepaticojejunostomy with excision of the extrahepatic biliary tree and radical lymphadenectomy. Unexpectedly, postoperative pathological and immunohistochemical examination indicated a perihilar bile duct NETs G1 with the microscopic invasion of the resected right hepatic duct. Then the patient received 3 cycles of adjuvant chemotherapy (Gemcitabine and tegafur-gimeracil-oteracil potassium capsule). At present, this patient has been following up for 24 months without recurrence or disease progression. We know little of biliary NETs because of its rarity. There are currently no guidelines for the diagnosis and treatment of biliary NETs. We reported a case of perihilar bile duct NETs G1 with R1 resection, as far as we know this is the first report. More information about biliary NETs should be registered.
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