Department of Surgery, Bucheon St. Mary's Hospital, College of Medicine, Catholic University of Korea, Seoul, Republic of Korea.
Department of Surgery, Division of Hepatobiliary-Pancreas Surgery and Liver Transplantation, Seoul St. Mary's Hospital, College of Medicine, Catholic University of Korea, Seoul, Republic of Korea.
Medicine (Baltimore). 2024 Sep 27;103(39):e39848. doi: 10.1097/MD.0000000000039848.
Neuroendocrine carcinoma originating from extrahepatic bile duct is very rare, and only a few cases have been reported. Because of its scarcity of incidence, not much is known about the disease but for its aggressiveness and poor prognosis.
In this report, we present 2 cases of large cell neuroendocrine carcinoma (LCNEC) originating from extrahepatic bile duct. Case 1: a 60-year-old woman presented with jaundice but no abdominal pain. Case 2: a 67-year-old man also presented with jaundice, along with abdominal discomfort and appetite loss.
Case 1: LCNEC with a focal adenocarcinoma component (pT2aN1M0, pStage IIIB). Case 2: LCNEC with a focal adenocarcinoma component (pT1N1M0, pStage IIB).
Case 1: the patient underwent left hepatectomy and caudatectomy with hepaticojejunostomy, followed by 6 cycles of adjuvant chemotherapy (etoposide and cisplatin). Case 2: the patient underwent laparoscopic pylorus-preserving pancreatoduodenectomy, followed by 6 cycles of adjuvant chemotherapy (etoposide and cisplatin).
Case 1: liver metastasis was detected 6 months postoperatively, and despite multiple chemotherapy regimens, the patient died 24 months post-surgery. Case 2: liver metastasis was detected 23 months postoperatively. The patient is still alive 36 months post-surgery after receiving multiple chemotherapy regimens and radiotherapy.
Given the rarity of LCNEC, it is essential to continue collecting and reporting additional case studies to build a more comprehensive understanding of the disease. Although the prognosis for LCNEC is generally poor, the use of a multidisciplinary approach and further research will be critical in developing more effective treatment strategies in the future.
起源于肝外胆管的神经内分泌癌非常罕见，仅有少数病例报道。由于其发病率低，对该疾病的了解甚少，仅知其侵袭性强，预后差。
本报告介绍了 2 例起源于肝外胆管的大细胞神经内分泌癌（LCNEC）患者。病例 1：60 岁女性，因黄疸就诊，无腹痛。病例 2：67 岁男性，因黄疸就诊，伴腹部不适和食欲减退。
病例 1：局灶性腺癌成分的 LCNEC（pT2aN1M0，p ⅢB 期）。病例 2：局灶性腺癌成分的 LCNEC（pT1N1M0，p ⅡB 期）。
病例 1：行左半肝和尾状叶切除术联合胆肠吻合术，随后接受 6 个周期的辅助化疗（依托泊苷和顺铂）。病例 2：行腹腔镜保留幽门胰十二指肠切除术，随后接受 6 个周期的辅助化疗（依托泊苷和顺铂）。
病例 1：术后 6 个月发现肝转移，尽管接受了多种化疗方案，但患者在术后 24 个月死亡。病例 2：术后 23 个月发现肝转移。患者在接受多种化疗方案和放疗后，仍存活 36 个月。
鉴于 LCNEC 的罕见性，需要继续收集和报告更多病例研究，以更全面地了解该疾病。尽管 LCNEC 的预后通常较差，但采用多学科方法和进一步研究对于未来制定更有效的治疗策略至关重要。
