Tamaki Ayano, Tani Yuma, Sato Hiroki, Yoshida Ryuichi, Yasui Kazuya, Horiguchi Shigeru, Kuise Takashi, Umeda Yuzo, Yoshida Kazuhiro, Fuji Tomokazu, Kumano Kenjiro, Takagi Kosei, Yagi Takahito, Fujiwara Toshiyoshi
Center for Graduate Medical Education, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
Department of Gastroenterological Surgery, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
Surg Case Rep. 2022 Mar 2;8(1):38. doi: 10.1186/s40792-022-01386-w.
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.
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.
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.
混合性神经内分泌-非神经内分泌肿瘤（MINEN）是一种罕见疾病，关于其诊断、治疗和预后的文献很少。我们遇到了两例不寻常的胆道MINEN病例，一例位于 Vater 壶腹，另一例位于胆总管远端。在本报告中，我们详细描述了这两例病例的临床过程。
病例1：一名69岁女性，主要症状为上腹部疼痛。因胆结石性胰腺炎行内镜括约肌切开术和逆行胆道引流时，在 Vater 壶腹发现一个溃疡病变。根据活检结果，该病变被诊断为 Vater 壶腹癌，并进行了保留部分胃的胰十二指肠切除术（SSPPD）。术后组织病理学检查显示腺癌和神经内分泌癌成分并存，符合 MINEN 的诊断。此外，在胰腺背侧发现淋巴结转移，转移成分是腺癌。给予依托泊苷和顺铂辅助化疗6个月，目前患者术后64个月存活且无复发。病例2：一名79岁男性，主要症状为厌食。胆管造影显示胆总管远端严重狭窄。对狭窄病变进行活检，结果显示为腺癌。诊断为胆总管远端癌，并进行了 SSPPD。组织病理学检查显示腺癌和神经内分泌癌成分并存，肿瘤被确诊为胆总管远端 MINEN。由于患者身体状况较差，未给予辅助化疗。7个月后，发现患者发生肝转移。
我们遇到了两例有价值的胆道 MINEN 病例。为了确定更好的治疗方法，重要的是要考虑个体病例的多样性，并继续分享各种不同表现的病例。
