Abstract：
BACKGROUND: Mixed neuroendocrine-non-neuroendocrine neoplasm (MINEN) is a rare disease and there is scarce literature on its diagnosis, treatment, and prognosis. We encountered two unusual cases of MINEN in the biliary tract, one in the ampulla of Vater and the other in the distal bile duct. In this report, we describe the clinical course of these two cases in detail.
METHODS: Case 1: A 69-year-old woman presented with a chief complaint of epigastric pain. When endoscopic sphincterotomy and retrograde biliary drainage were performed for gallstone pancreatitis, an ulcerated lesion was found in the ampulla of the Vater. Based on the biopsy results, the lesion was diagnosed as the ampulla of Vater carcinoma and subtotal stomach-preserving pancreatoduodenectomy (SSPPD) was performed. Postoperative histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, consistent with the diagnosis of MINEN. In addition, lymph node metastasis was found on the dorsal side of the pancreas and the metastatic component was adenocarcinoma. Adjuvant chemotherapy with etoposide and cisplatin was administered for 6 months, and presently the patient is alive without recurrence 64 months after surgery. Case 2: A 79-year-old man presented with a chief complaint of anorexia. Cholangiography showed severe stenosis of the distal bile duct. A biopsy was conducted from the stenotic lesion and it revealed the lesion to be adenocarcinoma. A diagnosis of distal bile duct carcinoma was made, and SSPPD was performed. Histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, and the tumor was confirmed as MINEN of the distal bile duct. No adjuvant chemotherapy was administered due to the poor performance status. 7 months later, the patient was found to have a liver metastasis.
CONCLUSIONS: We experienced two valuable cases of biliary MINEN. To identify better treatments, it is important to consider the diversity of individual cases and to continue sharing a variety of cases with different presentations.
METHODS: Case 1: A 69-year-old woman presented with a chief complaint of epigastric pain. When endoscopic sphincterotomy and retrograde biliary drainage were performed for gallstone pancreatitis, an ulcerated lesion was found in the ampulla of the Vater. Based on the biopsy results, the lesion was diagnosed as the ampulla of Vater carcinoma and subtotal stomach-preserving pancreatoduodenectomy (SSPPD) was performed. Postoperative histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, consistent with the diagnosis of MINEN. In addition, lymph node metastasis was found on the dorsal side of the pancreas and the metastatic component was adenocarcinoma. Adjuvant chemotherapy with etoposide and cisplatin was administered for 6 months, and presently the patient is alive without recurrence 64 months after surgery. Case 2: A 79-year-old man presented with a chief complaint of anorexia. Cholangiography showed severe stenosis of the distal bile duct. A biopsy was conducted from the stenotic lesion and it revealed the lesion to be adenocarcinoma. A diagnosis of distal bile duct carcinoma was made, and SSPPD was performed. Histopathological examination revealed the coexistence of adenocarcinoma and neuroendocrine carcinoma components, and the tumor was confirmed as MINEN of the distal bile duct. No adjuvant chemotherapy was administered due to the poor performance status. 7 months later, the patient was found to have a liver metastasis.
CONCLUSIONS: We experienced two valuable cases of biliary MINEN. To identify better treatments, it is important to consider the diversity of individual cases and to continue sharing a variety of cases with different presentations.
摘要：
背景：混合性神经内分泌-非神经内分泌肿瘤（MINEN）是一种罕见的疾病，关于其诊断的文献很少，治疗,和预后。我们在胆道遇到了两例罕见的MINEN病例，一个在Vater壶腹，另一个在远端胆管。在这份报告中,我们详细描述了这两个病例的临床过程。
方法：案例1：一名69岁女性主诉上腹痛。当内镜下括约肌切开术和逆行胆道引流治疗胆结石性胰腺炎时，在Vater壶腹发现了一个溃疡病变。根据活检结果,该病变被诊断为Vater癌的壶腹，并进行了胃部分保留胰十二指肠切除术（SSPD）。术后组织病理学检查发现腺癌和神经内分泌癌成分共存，与MINEN的诊断一致。此外,在胰腺背侧发现淋巴结转移，转移成分为腺癌。依托泊苷和顺铂辅助化疗6个月，目前患者在手术后64个月没有复发。案例2：一名79岁的男子主诉厌食症。胆管造影显示远端胆管严重狭窄。对狭窄病变进行活检，发现病变为腺癌。诊断为远端胆管癌，并进行SSPD。组织病理学检查显示腺癌和神经内分泌癌成分共存，证实肿瘤为远端胆管MINEN。由于性能状态不佳，未进行辅助化疗。7个月后,病人被发现有肝转移。
结论：我们经历了两个有价值的胆道MINEN病例。为了找到更好的治疗方法，重要的是要考虑个案的多样性，并继续以不同的陈述分享各种个案。
方法：案例1：一名69岁女性主诉上腹痛。当内镜下括约肌切开术和逆行胆道引流治疗胆结石性胰腺炎时，在Vater壶腹发现了一个溃疡病变。根据活检结果,该病变被诊断为Vater癌的壶腹，并进行了胃部分保留胰十二指肠切除术（SSPD）。术后组织病理学检查发现腺癌和神经内分泌癌成分共存，与MINEN的诊断一致。此外,在胰腺背侧发现淋巴结转移，转移成分为腺癌。依托泊苷和顺铂辅助化疗6个月，目前患者在手术后64个月没有复发。案例2：一名79岁的男子主诉厌食症。胆管造影显示远端胆管严重狭窄。对狭窄病变进行活检，发现病变为腺癌。诊断为远端胆管癌，并进行SSPD。组织病理学检查显示腺癌和神经内分泌癌成分共存，证实肿瘤为远端胆管MINEN。由于性能状态不佳，未进行辅助化疗。7个月后,病人被发现有肝转移。
结论：我们经历了两个有价值的胆道MINEN病例。为了找到更好的治疗方法，重要的是要考虑个案的多样性，并继续以不同的陈述分享各种个案。
